Color Atlas of Congenital Heart Surgery Second Edition
Color Atlas of Congenital Heart Surgery Second Edition
S. Bert Litwin, MD Director Emeritus, Cardiothoracic Surgery, Herma Heart Center, Children's Hospital of Wisconsin; Clinical Professor of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
Springer
S. Bert Litwin, MD Director Emeritus Cardiothoracic Surgery Herma Heart Center Children's Hospital of Wisconsin and Clinical Professor of Surgery Medical College of Wisconsin Milwaukee, WI USA
Library of Congress Control Number: 2006930105 ISBN-10: 0-387-35415-8 ISBN-13: 978-0-387-35415-6
e-ISBN-10: 0-387-49925-3 e-ISBN-13: 978-0-387-49925-3
Printed on acid-free paper. © 2007 Springer Science+Business Media, LLC. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. 987654321 spnnger.com
To Judy my wife and best friend without whose love and companionship even my career in congenital heart surgery would be almost meaningless and to my daughters Amy Jessica and Jill Andrea whom I love and respect for their intelligence, talent, inner and outer beauty, and commitment to making a contribution in their respective fields.
Foreword
The last three decades have witnessed enormous progress in the care of patients with congenital heart disease. Treatment of congenital heart disease is highly dependent on technology and much of the progress we have witnessed is attributable to technological advances we take almost for granted today. It would be difficult to overestimate the impact of these advances; noteworthy examples include the development of 2-D Doppler echocardiography resulting in increased diagnostic accuracy, improvements in preoperative management including the use of prostaglandins for maintaining ductal patency, better intraoperative support such as the development of cardiopulmonary bypass circuits specifically designed for neonates and infants and improvements in postoperative care too numerous to delineate. As the spectrum of congenital heart disease we can treat successfully has broadened and the results have improved much of the focus has shifted, properly, to long-term issues such as neurodevelopmental outcome and quality of life. Despite the current focus on long-term outcomes we must not forget that surgery is central to our treatment strategy. The word technology is derived from the Greek word "techne" meaning craft and before any late outcomes can be measured the "craft" of surgery must be performed with excellence. Dr. Litwin's career has spanned these last three decades and he has witnessed and participated in the evolution of congenital heart surgery. The second edition of a "Color Atlas of Congenital Heart Surgery" is an outstanding contribution to the field by a master of the craft of congenital heart surgery. Most textbooks on congenital heart surgery rely on drawings to illustrate the operations. These drawings are the imperfect recollections of the author's experience; smoothed and cleaned of imperfections they sometimes oversimplify the operative technique and may bear only a passing resemblance to reality. A cliche has thus been coined "it never looks like it does in the book". In stark contrast, Dr. Litwin's atlas is a true-to-life depiction of the anatomy and steps required for each of the procedures. The photographs truly speak for themselves; they require little description as they demonstrate each step of the procedure. The images are testimony to the meticulous technique that Dr. Litwin is known for and which I have had the good fortune to witness and learn from first hand. In the present era when video sessions are an increasing part of surgical meetings, the "Color Atlas of Congenital Heart Surgery" is both avant-garde and historic. Operations that are part of the day to day activities of congenital heart surgery programs today such as the arterial switch and stage I palliation of hypoplastic left heart syndrome are illustrated but in addition the Senning procedure is also included. The current students of congenital heart surgery who must train and begin their careers in a complex environment marked by two opposing forces, one of increasing technical difficulty of the cases and the other a decreasing tolerance for any sort of learning curve, will find this book invaluable as it provides both detailed photographs of
vn
real operations and a breadth of procedures that can be used either in isolation or in combination to manage virtually every anatomic variance encountered. I recommend this book highly to anyone interested in congenital heart disease. It is the culmination of over thirty years of surgical experience from a dedicated surgical clinician and master technician. To those in nonsurgical specialties this atlas will provide insight into the procedures that drawings simply cannot match. For the surgeon the photographs provide not only outstanding illustrations of the procedures but also the inspiration to perform with the precision and care that would make each operation worthy of a photograph. Excellence in the craft of congenital heart surgery can only be achieved through careful study, practice and dedication. As surgeons we must remember that this skill is a necessary precursor to the pursuit of better long-term outcomes for our patients. James S. Tweddell, MD Director of Cardiothoracic Surgery Children's Hospital of Wisconsin Professor of Surgery (Cardiothoracic) and Pediatrics Medical College of Wisconsin Professor and Chief Division of Cardiothoracic Surgery Department of Surgery Medical College of Wisconsin
Preface
Because of the wide variety of anomalies encountered in congenital heart surgery, a broad understanding of the pathologic anatomy of defects is vitally important to the surgeon. More than in many other fields of surgery, a feel for three-dimensional spatial relationships of anomalies is helpful in allowing the operating surgeon to improvise technical details of a procedure. Precisely shaping and sizing an intraventricular baffle or patch, or correctly placing a long intraatrial suture can make the difference between a successful and an unsuccessful surgical outcome. The congenital heart surgeon is a student during his or her entire career because he or she encounters so many different anomalies. Learning from the experience of others should always be part of the clinician's education; this is best done by personally observing an operation performed by another. Otherwise, the best record of a procedure is a good operative photograph. For over 35 years it has been my practice to photograph most operations. These illustrations comprise a valuable part of each patient's record and are informative as a review of previous surgery and observed anatomy if future surgery is planned. The illustrations also serve to inform the referring doctor of details of surgery and this may also improve patient care. The photographs have been an invaluable teaching aid for lectures, journal publications, and this book. I hope this atlas will be of interest to fulltime or part-time congenital heart surgeons, pediatric and adult cardiologists, intensivists, pediatricians, internists, and all other students of congenital heart disease. In this second edition, I have added many new sections, deleted a few obsolete sections, and in some areas changed the format. For example, the atrial switch operation has been moved to Chapter 16, 1-Transposition of the Great Arteries (1-TGA). It is no longer used in the repair of d-transposition of the great arteries (d-TGA), but is applicable for the double-switch operation for 1-TGA. Photographs were taken with a Nikon F camera, usually using a Nikon 55-mm macro lens (Figure P-l). For some close-up pictures of the right and left ventricular outflow tracts, a Nikon 100-mm medical lens was used (Figure P-2). Pictures were taken at f8 to fll at a distance of 9 to 12 inches from the field. Lighting for most photographs was augmented with a side-mounted Honeywell Strobonar flash with a wide-angle neutral density filter (Figures P-l, P-2). Using a side-mounted flash, rather than a more traditional ring light (surrounding the lens), has resulted in some shadows in each picture, which improves the perception of depth of field. More recently, I have used a ring light mounted on the front of the lens. No special effort has been made to use the operating room lights or to move them out of the field.
IX
x Preface
P-l. Nikon camera viewer from above. A 5-mm macro lens is attached, and the light source is positioned to the side of the lens. A waist-level viewer is attached for taking pictures from the head of the operating table. FIGURE
FIGURE P-2. Nikon camera with 100-mm macro lens attached for close-up views. A sports viewer is used, and the position of the light source is at the side of the lens.
Photographs were taken from behind the patient when surgery was performed through a lateral thoracotomy and from the head of the operating room table for a median sternotomy. For orientation, pictures in this atlas are marked with arrows to indicate R, right side of patient; L, left side of patient; Cep, cephalad; Caud, caudad; Ant, anterior; and Post, posterior. 5. Bert Litwiny MD
Acknowledgments
It is an honor and a privilege to work in the field of congenital heart surgery. The workdays are long and the cases are complex and trying, but the rewards and satisfaction are enormous. I am indebted to Linda Hamilton, RN, Nancy Stover, RN, Maryanne Kessel, RN, MBA, and Kathleen Mussatto, BSN, who have worked for me during successive years as cardiovascular surgical nurse clinicians. They catalogued photographs as one of many invaluable responsibilities that they tirelessly performed, in addition to carrying out various tasks in the care of many sick infants and children. Linda went on to be a senior administrator at various healthcare facilities in Vancouver, Canada. Nancy, sadly, is deceased. Maryanne is now Director of the Herma Heart Center and Kathy is Manager of Research of the Herma Heart Center, Children's Hospital of Wisconsin. I and all of my patients are forever in their debt. Many surgeons had an influence on my career and, thus, the clinical experience that allowed this book to become a reality. Dr. Oliver Cope (deceased) taught me research techniques and an investigative approach to surgery, as well as humility and compassion for patients. Dr. W.G. Austen taught me the benefit of organizational skills and introduced me to cardiac surgery. Dr. Robert E. Gross (deceased) showed me the excitement of children's surgery and taught me the principles of congenital heart surgery. Dr. William F. Bernhard showed me the techniques of infant heart surgery, many of which have remained valid and can be applied to patient care today. Mr. R.H.R. Belsey showed me surgical technique par excellence and the value of excellent clinical judgment. Dr. Aldo Castaneda, with whom I had the opportunity to work for a short time, was the guiding light in the field of congenital heart surgery for many years and an example of the surgeon who can achieve the highest degree of success and innovation in this complex field of surgery. Dr. Willis Williams, my long-time close friend, helped me to develop the photographic techniques that I have used for many years. Tireless efforts of my cardiologists, anesthesiologists, intensivists, physician assistants, perfusionists, nurse clinicians, and many others are responsible more than I can say in helping to achieve any clinical success I have enjoyed. I thank my associate, Dr. James Tweddell, for allowing me to photograph his patients undergoing repair of intramural coronary artery and stage I palliation for HLHS (Chapter 18, Sections 2 and 6, respectively). My secretary Lisa Armitage helped enormously by typing the manuscript and cataloging photographic slides.
XI
I am extremely grateful to the editors and previous and current staff of Springer Science+Business Media for encouragement in the preparation of this atlas and recognition of the value of operative photographs. This includes Laura Gillan, Beth Campbell, and Paula Callaghan. Springer Science+Business Media has done an outstanding job in reproducing so much material in an artistic, scholarly fashion. S. Bert Litwin, MD
Contents
Foreword by James S. Tweddell Preface Acknowledgments Introduction and Techniques 1 Anomalous Systemic Venous Return 2 3 4 5 6
vii ix xi xv 1
Secundum Atrial Septal Defect Tricuspid Valve Anomalies Endocardial Cushion Defects Ventricular Septal Defects Fontan Operation
9 29 37 74 101
7 8 9 10 11 12 13 14 15 16
Pulmonary Stenosis Pulmonary Atresia and Intact Ventricular Septum Tetralogy of Fallot Double Outlet Ventricles Pulmonary Atresia with Ventricular Septal Defect Pulmonary Venous Anomalies Left Atrial Obstructive Lesions Valvular Stenosis d-Transposition of the Great Arteries 1-Transposition of the Great Arteries
129 135 138 188 198 214 237 245 285 319
17 18 19 20
Truncus Arteriosus Aortic Root Anomalies Interrupted Aortic Arch Coarctation of the Aorta
343 369 415 425
xiii
xiv Contents
21 Patent Ductus Arteriosus 22 Vascular Ring and Vascular Sling 23 Miscellaneous
444 451 463
Index
473
Introduction and Techniques
Some techniques and principles of operative exposure and cardiopulmonary bypass that I currently prefer are worth mentioning. No doubt different techniques used by other surgeons are just as effective in their hands. Most open heart operations are performed through a standard median sternotomy with a longitudinal skin incision. For female patients of all ages who undergo simpler operations and in whom I do not anticipate a repeat sternotomy or the need to leave the sternum open, my preference is a transverse skin incision to approach the sternum for the sternotomy. The cosmetic advantage is obvious and I have encountered few complications with this incision. In order to avoid damage to the lateral and anterior cutaneous branches of the intercostal nerves that may result in loss of nipple sensation, the surgeon must restrict the subcutaneous flap dissection to a triangular area with the tip at the manubrium and the triangle base at the site of the transverse skin incision below the nipples. For intra-atrial procedures, cardiopulmonary bypass can also be instituted by working through a right lateral thoracotomy in the fourth interspace. The cavae are readily exposed and the ascending aorta can be cannulated. Cardiopulmonary bypass through a left thoracotomy is more difficult. Aortic cannulation of the arch or descending thoracic aorta is simple, but a cannula for venous return has to be placed in the main pulmonary artery and, in older patients, through a femoral vein into the right atrium. When partial bypass through the left chest is used, the arterial cannula is placed in the descending thoracic aorta and a single venous cannula in the left atrial appendage. The head and neck are perfused by the beating heart, and the lower body is perfused by the partial cardiopulmonary bypass circuit. Most closed heart surgery is performed through a lateral thoracotomy in the third or fourth intercostal space without rib resection. This includes isolated Blalock shunt and pulmonary artery banding operations. In this day of multiple mediastinal operations for staged repairs, it is not wise to add to mediastinal scarring with an initial simple palliative operation that can effectively be performed through a thoracotomy. For cardiopulmonary bypass, a standard roller pump and hollow fiber membrane oxygenator are used. The arterial perfusion cannula I most often use is a plastic straight-tipped wire bound DLP cannula (Medtronic Inc., Minneapolis, MN) for small aortae and a right-angle metal-tipped Sarns Cannula (Terumo Cardiovascular Systems Corporation, Ann Arbor, MI) for older children. In most cases, bicaval cannulation is performed because this allows the surgeon to work within the cardiac chambers during cooling and rewarming periods, even without aortic cross-clamping. Right-angle, metal-tipped DLP cannulae (Hospira, Inc., Lake Forest, IL) are usually placed directly in the superior vena cava and through the right atrial wall near the inferior vena cava. A single venous
xv
cannula in the right atrial appendage is advantageous in small infants, but does not allow the surgeon to work within the atrium during cooling and rewarming. A ventricular vent is placed, usually at the interatrial groove along the right heart border or through the left atrial appendage. I prefer large vents, because this is a major component of my de-airing technique that follows repair. A small, needle is placed in the proximal ascending aorta for delivery of cardioplegia and to help in later intracardiac de-airing. Cardiopulmonary bypass indexed flow rates are at 2.2 to 3.4L/min at mild hypothermia (32°C), 1.8 to 2.2L/min at moderate hypothermia (26°C), and 0.5 to 1.5L/min for temporary low flow (under 24°C). Low-flow cerebral perfusion (0.25-0.5 L/min indexed) has supplanted the need for circulatory arrest. Placement of central OPITCATH MV0 2 catheters (Hospira, Inc.) for online monitoring as a predictor of cardiac output, and the use of near infrared spectrometry (NIRS; Somanetics Corporation, Troy, MI) for continuous measurement of cerebral oxygenation are invaluable. The inclusion of amphiphylic, biopassive polymer coating including X-COATED (Terumo Cardiovascular Systems Corporation, Ashland, MA) and SMARXT COATED (COBE Cardiovascular, Inc., Arvada, CO) coating on oxygenators and bypass tubing and the use of postbypass modified ultrafiltration have been beneficial in reducing the inflammatory response to surgery. Control of systemic vascular resistance with the beta blocker phenoxybenzanine and use of the open sternum, particularly in small neonates after complex repairs, have helped to significantly reduce mortality. The value of EMCO support as a bridge to recovery for life-threatening ventricular failure in improving survival cannot be overstated. 5. Bert Litwin, MD
1
Anomalous Systemic Venous Return
Abnormal connections between the inferior vena cava, or right or left superior venae cavae to the right or left atrium may occur. A right-side anomaly (e.g., persistent left superior vena cava to the coronary sinus or azygos continuation of the inferior vena cava) may be of no consequence and require no treatment, although when other intracardiac anomalies require repair, the right-side abnormality may require an alteration in cardiopulmonary bypass cannulation techniques. When systemic veins connect to the left atrium, there is a right-to-left shunt with cyanosis, and repair is necessary. With a persistent left superior vena cava that connects directly to the left atrial roof (also known as unroofed coronary sinus), ligation of the structure obliterates the intracardiac shunt but is dangerous unless there is a normal innominate vein or large collateral connections in the head that allow unobstructed left head and neck flow into the heart. This left cava otherwise can be anastomosed directly to the right superior vena cava in certain circumstances or requires intracardiac tunneling to the right atrium. These anomalies are usually diagnosed by echocardiography, cardiac catheterization and angiography, or cardiac magnetic resonance imaging (MRI), but occasionally are discovered as incidental findings at the time of intracardiac surgery for another anomaly. Repair is carried out using cardiopulmonary bypass with moderate hypothermia, aortic clamping, cardioplegia, and profound local cardiac cooling. The left ventricle is vented.
1
2 Color Atlas of Congenital Heart Surgery
1-1. Left Superior Vena Cava Draining to Roof of Left Atrium
atrial septal defect
cannula in anomalous left superior vena cava
ceph R<-
-•L
caud
FIGURE 1-1. The patient is on cardiopulmonary bypass and cannulae are placed in the right superior and inferior venae cavae. The right atrium is opened, and a large secundum atrial septal defect (ASD) is seen. A third caval cannula passes through the ASD and is placed in the left superior vena cava, which enters the upper posterior wall of the left atrium.
orifice of right upper pulmonary
caval cannula placed in orifice of left superior vena cava
ceph R^-
-•L
caud
1-2. The atrial septum is retracted, and the cannula is seen at the orifice of the left superior vena cava. FIGURE
1
Anomalous Systemic Venous Return 3
Dacron patch stitched to lower rim of orifice of left superior vena cava
atrial septal defect
ceph R<-
-*L
caud
1-3. A thin, knitted Dacron® patch is stitched along the lower rim of the orifice of the left superior vena cava.
FIGURE
suture around orifice of left superior vena cava
patch ceph
R<-
-•L
caud
1-4. The suture is continued around the right and left sides of the caval orifice and then runs anteriorly toward the upper rim of the atrial septum. The patch is placed caudad over the atrial septal defect and will be stitched to the atrial septum to close that defect. FIGURE
4 Color Atlas of Congenital Heart Surgery 1-5. Using a continuous suture, the patch is stitched to the atrial septum at the site of the ASD. The atrial septal defect is closed, and drainage from the left superior vena cava is now diverted to the right atrium. FIGURE
cannula In left superior vena cava
patch stitched to atrial septum
1-2. Right Superior Vena Cava Draining to Roof of Left Atrium The preoperative diagnosis in this patient was sinus venosus ASD with possible partial anomalous pulmonary venous connection.
rim of atrial septal defect
ceph R«-
->L
orifice of right superior vena cava
caud
1-6. The child has been placed on cardiopulmonary bypass and the superior vena cava is cannulated directly and remotely from the caval atrial junction. An atriotomy is made in the mid right atrium. The ASD is identified in the high lateral septum in the sinus venosus region. The right upper and middle pulmonary veins were seen draining to the left atrium immediately posterior to the atrial septal defect. The superior vena cava orifice is adjacent to the pulmonary veins in the left atrium. FIGURE
1
Anomalous Systemic Venous Return 5
probing clamp in superior vena cava
1-7. A clamp is passed through the ASD and into the caval orifice to verify its location.
FIGURE
upper segment of superior vena cava
closure of lower segment of superior vena cava
R<
1-8. The superior vena cava is divided above the region of the upper and middle lobe pulmonary veins. The lower caval segment is closed primarily.
FIGURE
Color Atlas of Congenital Heart Surgery
atrial septal defect
ceph R«-
->L
caud FIGURE 1-9. The ASD is exposed and the lower caval closure suture line identified from within to ensure there is no encroachment on the pulmonary veins.
ceph R<-
-*L
caud FIGURE
1-10. The ASD is closed with a Dacron® patch.
1
Anomalous Systemic Venous Return 7
upper segment of divided superior vena cava
atriotomy at base of right atrial appendage
ceph
R<-
-•L
caud
1-11. A generous transverse incision is made in an appropriate area at the base of the right atrial appendage for a direct cava to atrium anastomosis. A spot for the anastomosis is chosen to avoid undue tension on the new connection. FIGURE
posterior anastomosis
FIGURE 1-12. A continuous fine polypropylene suture is used for the posterior anastomosis. A similar continuous suture is used for part of the anterior anastomosis with the remainder closed with three or four interrupted sutures to ensure growth potential of the new anastomosis.
8 Color Atlas of Congenital Heart Surgery
cava to atrium anastomosis
closed atriotomy
ceph R<-
-•L
caud
1-13. The completed anastomosis is seen and there is no undue tension from the right atrium. FIGURE
2
Secundum Atrial Septal Defect
Diagnosis of secundum atrial septal defect (ASD) is made by echocardiography and cardiac catheterization is reserved for the rare case in whom evaluation of hemodynamics is needed. If the ASD measures 5 mm or more, repair is recommended. In those defects that are smaller, repair is indicated with associated right ventricular (RV) dilatation or other evidence of RV volume load. In most centers today, closure of this anomaly beyond infancy is offered by the invasive cardiologist using one of a variety of prosthetic devices. Long-term results are being evaluated for this technique. Certain anatomical features contraindicate device closure and complications, including early failure of closure or residual ASD, early or late thromboembolism, serious arrhythmias, aortic insufficiency, interference with mitral valve function, or erosion of the device with catastrophe, convince many families to use the long-tested and reliable technique of surgical repair. The operation is performed using cardiopulmonary bypass. When the defect is isolated and time on the heart-lung machine is expected to be short, core cooling to 32°C is used. In the presence of partial anomalous pulmonary venous connection or other intracardiac anomalies, when intracardiac repair requires a longer time, core cooling to 26°C to 30°C is used. In all cases, the operation is performed with aortic clamping, cardioplegia, profound local cardiac cooling, and left ventricular venting to maximize safety against air embolization during bypass.
9
10
Color Atlas of Congenital Heart Surgery
2-1. Isolated Secundum Atrial Septal Defect
FIGURE 2-1. The
atrial septal defect
child
has
been
placed on cardiopulmonary bypass, and a mid right atriotomy is made. The region of the cavoatrial junction is spared to avoid injury to the sinoatrial (SA) node. A large secundum atrial septal defect can be seen in the region of the fossa ovalis. A metal sucker is in the defect. Because the septal tissue that surrounds the ASD is substantial, it can be closed primarily. It is important to inspect the atrial chambers to rule out other anomalies and the orifices of all pulmonary veins should be identified to ensure that they enter the left atrium normally.
>L
closure stitch
tricuspid valve
coronary sinus
FIGURE 2-2. Primary closure of the ASD has been performed using a continuous suture of monofilament polypropylene. The closure is started at the caudad margin of the ASD because this is usually the area of poorest exposure. The surgeon should avoid stitching together the posterior and anterior rims of the defect remote from the lower edge of the ASD; if this is done, the surgeon can create a tunnel for inferior vena caval blood to flow directly into the left atrium, causing a right-to-left shunt postoperatively. After the closure stitch is completed, a small probe should be used to check for residual openings in the suture line, which can be closed with interrupted sutures.
2
Secundum Atrial Septal Defect
11
atrial septal defect
ceph R<-
-•L
caud
2-3. When the ASD is extremely large, primary closure with a continuous suture may cause excessive tension on and tearing of the rim of the defect, resulting in a recurrent ASD postoperatively. It is safer to close this atrial defect with a patch to avoid suture line tension. Here, the anterior cephalad septum is poorly formed. Stitches in this area should be placed in the endocardium only because the aortic valve is located beneath this area and can be injured by deeply placed sutures. FIGURE
Dacron patch
ceph R<-
-•L
caud
2-4. A Dacron® patch is stitched over the ASD with a continuous monofilament suture. The rim of the patch should be probed to check for residual openings, which can be closed with individual sutures. Endothelial growth covers the Dacron® patch within 4 to 6 weeks postoperatively, and postoperative anticoagulants are not used. I prefer Dacron® material for a patch in the atrium because it encourages tissue ingrowth; also, it will not contract as will pericardium, so there is less chance for tension on the suture line and reduced possibility of tearing the septum with recurrence of the ASD. FIGURE
12 Color Atlas of Congenital Heart Surgery
2-7-7. Coronary Sinus Atrial Septal Defect
atrial septal defect
coronary sinus orifice
ceph R^-
-•L
caud
2-5. The child has been placed on cardiopulmonary bypass and a mid right atriotomy performed. Looking through the orifice of the coronary sinus there is an atrial septal defect in the roof of this structure. If small the defect can be closed primarily; however, most will be closed with a patch to avoid undue tension on closure sutures. FIGURE
2-7-2. Atrial Septal Defect after Failed Device Closure
Sideris occluder
residual atrial septal defect ceph
R<-
-•L
caud
2-6. The child has been placed on cardiopulmonary bypass and a mid right atriotomy made. The Sideris occluder, which was placed previously, is shown with the residual ASD. FIGURE
2
Secundum Atrial Septal Defect
13
Sideris occluder
ceph A
R«-
-•L
caud FIGURE 2-7. The occluder is attached to the septum by gelatinous material along the posterior rim of the ASD.
Sideris occluder explanted
R^
2-8. The occluder is explanted by incising and excising free-floating gelatinous material.
FIGURE
14 Color Atlas of Congenital Heart Surgery
atrial septal defect
coronary sinus
ceph R«-
tricuspid valve
-•L
caud FIGURE
2-9. The remaining atrial septal defect is in the mid part of the septum.
stitch closure of atrial septal defect
ceph R<-
-•L
caud
2-10. The atrial septal defect is closed with a continuous stitch and a few additional buttressing stitches across the original suture line. FIGURE
2
Secundum Atrial Septal Defect
15
2-2. Atrial Septal Defect with Partial Anomalous Pulmonary Venous Connection When partial anomalous pulmonary venous connection is present, abnormal pulmonary veins from the right upper and middle lobes commonly drain to the superior vena cava or the right atrium near the caval atrial junction. This is usually associated with a sinus venosus ASD located opposite the anomalous pulmonary veins.
aorta
superior vena cava cava-atrial junction
anomalous pulmonary veins
ceph R<-
->L
caud
FIGURE 2-11. Anomalous pulmonary veins are seen entering the high and lateral right atrium and low superior vena cava adjacent to the cava atrial junction.
FIGURE
2-12. The
superior
vena caval cannula is passed through the right atrial appendage and into the mid superior vena cava. The superior caval snare is placed above the azygos vein to allow wide exposure of the anomalous pulmonary veins with a high cardiotomy to the mid superior vena cava. A separate tape for snaring is passed around the azygos vein for hemostasis. The atriotomy is made posterior to the sulcus terminalis and the SA node to avoid damage to the latter structure.
atrial septal defect normal pulmonary vein entering left atrium ceph
caud
16 Color Atlas of Congenital Heart Surgery
atrial septal excision
ceph R^-
-•L
caud FIGURE 2-13. In some cases the atrial septal defect is too small to freely accept all flow from the tunnel constructed around the anomalous pulmonary veins. To avoid obstruction of the tunnel at this site, the atrial septal defect should be enlarged by resecting the adjacent septum. Adequate exposure of the ASD and anomalous veins is gained by retracting the superior vena caval cannula.
Dacron patch
ceph R<-
-•L
caud
2-14. A thin Dacron® patch is cut in a pear shape, and the smaller end is stitched around the orifices of the anomalous pulmonary veins. The patch should not be too narrow in this area because minimal obstruction anywhere in the Dacron® tunnel can result in pulmonary venous obstruction. The Dacron® patch tunnel is completed by stitching it to the rim of the ASD. Flow from the anomalous pulmonary veins is now diverted behind the patch through the atrial septal defect and to the left atrium. Posteriorly, the Dacron® patch is near the rim of the posterior right atriotomy. FIGURE
2
Secundum Atrial Septal Defect
17
pericardial patch
ceph R<-
-•L
caud FIGURE 2-15.
To avoid superior vena caval obstruction by the intracardiac Dacron® patch, a gusset of pericardium is always placed over the lower superior vena cava and adjacent right atrium to widen the cava-atrial junction. When sutures are placed to attach the anterior rim of the gusset, care should be exercised to avoid damage to the SA node. Pericardium is preferable to synthetic material for the gusset because of its ease of handling and hemostatic qualities.
2-2-1. High Superior Caval Drainage of Partial Anomalous Venous Connection
Pulmonary
When repair is performed using an intracaval baffle or patch tunnel, there is a high risk of superior caval and/or pulmonary vein tunnel obstruction. Repair using a right atrial pedicle flap tunnel is indicated when anomalous veins enter the superior cava near or above the azygos vein entrance.
anomalous pulmonary
area of azygos vein ceph R<-
-•L
caud
2-16. The anomalous pulmonary veins are located high in the cava near the innominate vein. FIGURE
18 Color Atlas of Congenital Heart Surgery
pedicle flap of right atrium
ceph R««-
cannula in superior vena cava
->L
caud
2-17. The child is placed on cardiopulmonary bypass with bicaval cannulation.The purse string stitch for the superior vena caval cannula is placed in the wall of the mid right atrium, and a straight cannula is used. Alternatively, a small right-angle cannula can be placed directly in the cava above the anomalous pulmonary veins and near the innominate vein. A wide pedicle flap of right atrial wall is developed, with the base of the flap in the transverse plane, near the cava atrial junction. Care is taken to avoid injury to the SA node during development of this flap. The straight cannula is seen passing into the superior vena cava. The superior cava is divided around the cannula in the area above the insertion of the anomalous veins. The superior caval cannula is momentarily clamped, removed from its position in the superior vena cava, and passed through the large atriotomy at the site of pedicle flap and again passed into the upper superior vena cava through the caval tourniquet. FIGURE
cannula in superior vena cava
Gore-Tex patch
lower segment of superior vena cava
R^
2-18. A Gore-Tex® patch is stitched over the lower end of the divided superior vena cava, immediately above the anomalous pulmonary veins. Other patch material, such as pericardium or homograft pulmonary artery wall, can be used. FIGURE
2
Secundum Atrial Septal Defect
19
upper segment of superior vena cava
Gore-Tex patch
lower segment of superior vena cava
ceph R««-
-•L
caud
2-19. The cava above the pulmonary veins is closed with the Gore-Tex® patch, and the caval cannula is seen passing through the tourniquet in the upper superior vena cava. FIGURE
intra atrial Dacron patch ceph R«*-
->L
caud
2-20. A Dacron® patch is stitched over the high ASD, bringing the upper margin of the patch in front of the native superior caval orifice. The lower superior cava segment now functions as a conduit for anomalous pulmonary vein flow to the left atrium behind the Dacron® patch. FIGURE
20 Color Atlas of Congenital Heart Surgery harvesting pericardial patch upper superior vena cava
superior
caval cannula right atrial pedicle flap tunnel ceph R^-
right atriotomy
-•L
caud FIGURE 2-21. The atrial wall pedicle flap is wrapped around the caval cannula and anastomosed end-to-end to the upper caval segment.
pericardial patch
ceph R<-
right atrium
-•L
caud FIGURE 2-22. The deficient area in the anterior wall of the new superior vena cava is closed with a pericardial autograft. A patch of different material, that is, homograft pulmonary artery wall, can be used. I prefer a tissue patch of some variety for better hemostasis.
2-2-2. Scimitar's
Syndrome
All right pulmonary veins drain to the right atrium through a common vein that enters the atrium or cava near the inferior caval atrial junction. There is usually an associated secundum ASD and a large left-to-right shunt through both the anomalous pulmonary veins and the ASD. Anomalous systemic arteries from the abdominal aorta to the right lung are usually present and pierce the diaphragm to enter the lower lung region. These vessels must be closed at the time of surgical correction.
2
Secundum Atrial Septal Defect
21
2-2-3. Intracardiac Tunnel Repair
right atrium
anomalous right pulmonary
ceph R<-
-•L
caud FIGURE 2-23. By retracting the heart, the inferior vena cava can be seen in a normal position. The large anomalous right pulmonary vein is immediately adjacent to the cava and enters the right atrium in this area. Care must be used when the inferior vena cava is isolated with a tape for snaring because it may be adherent to the anomalous vein. The diaphragmatic surface of the pericardium is incised so that the inferior vena cava can be isolated beneath the diaphragm.
atrial septum
orifice of anomalous pulmonary vein
R<*
2-24. After the child is placed on cardiopulmonary bypass, a low posterior right atriotomy is made. The inferior vena caval cannula is retracted anteriorly, and the orifice of the anomalous pulmonary vein can be seen posteriorly.
FIGURE
22 Color Atlas of Congenital Heart Surgery
atrial septal defect
ceph R«-
-•L •
caud
2-25. An ASD is located in the region of the fossa ovalis. If it is restrictive, the surrounding atrial septum should be excised to enlarge the ASD.
FIGURE
Dacron patch
ceph R«-
-•L
caud
2-26. A thin Dacron® patch is stitched inferiorly around the orifice of the anomalous pulmonary vein and then in a cephalad direction along the posterior atrial septum and the mid atrial septum, respectively. The patch creates a tunnel to divert anomalous pulmonary vein flow to the ASD septal defect and the left atrium. The stitch around the orifice of the anomalous pulmonary vein is placed to separate the anomalous vein from the inferior vena cava without narrowing the cava. FIGURE
2
Secundum Atrial Septal Defect
23
pericardial patch
ceph R<-
-•L
caud
FIGURE 2-27. To avoid obstruction of the inferior vena cava by the intra-atrial Dacron® patch, a pericardial gusset is stitched over the upper inferior vena cava and low right atrium.
pericardial patch
inferior vena cava
FIGURE 2-28. After completing the repair, the inferior cava-right atrial gusset will distend when the blood fills the right atrium. The effect of this gusset is to widen the upper inferior cava and low right atrium.
24 Color Atlas of Congenital Heart Surgery 2-2-4. Extracardiac
Direct
Anastomosis
This repair is performed working through a right fifth or sixth intercostal space thoracotomy without cardiopulmonary bypass when no intracardiac defects are present. In the presence of an ASD, for example, repair on cardiopulmonary bypass can be carried out through this incision.
pericardium
Scimitar's vein entering inferior vena cava
ant ceph^-
right lower lobe
-•caud
post FIGURE 2-29. Working through a right fifth or sixth intercostal space thoracotomy, the inferior portion of the right lung is retracted to expose the Scimitar's pulmonary vein, which drains the entire lung to the upper inferior vena cava. If anomalous systemic arteries are present they can easily be divided through this exposure.
phrenic nerve
pericardial opening
left atrium
Scimitar's vein
ant ceph^-
-•caud
post
2-30. The pericardium is opened over the left atrium immediately anterior to the hilum and usually posterior to the phrenic nerve. FIGURE
2 2-31. The Scimitar's vein is divided at its connection to the cava and the caval end is closed. Prior to clamping the Scimitar's vein, the right pulmonary artery is occluded by a snare to avoid right lung engorgement during the procedure. Using a partially occluding side biting clamp on the left atrium, the Scimitar's vein is shifted cephalad and anastomosed directly to the left atrium.
Secundum Atrial Septal Defect 25
FIGURE
right pulmonary artery
left atrium
anastomosis
>caud post
2-2-5. Anomalous Left Upper Lobe Pulmonary Venous Connection Intact Atrial Septum
with
2-2-5-1. Extracardiac Repair Repair is carried out by working through a left fourth or fifth intercostal space thoracotomy without cardiopulmonary bypass.
innominate vein
ascending connecting
left subclavian artery
left upper lobe pulmonary ant caud^-
-^•ceph
left lower lobe pulmonary vein
post
2-32. Working through a left thoracotomy the lung is retracted posteriorly. Here, the large ascending pulmonary vein drains all left lung flow to the innominate vein. The ascending vein is dissected as are the upper and lower pulmonary veins. FIGURE
26
Color Atlas of Congenital Heart Surgery
left atrial appendage
ascending connecting vein left upper lobe pulmonary vein
ant caud<-
->-ceph
post
left lower lobe pulmonary vein
FIGURE 2-33. The pericardium is opened immediately in front of the hilum to expose the left atrial appendage.
excluding side biting clamp
left atrial appendage
vein to atrium anastomosis
ant caud<-
-•ceph
post
FIGURE 2-34. The left pulmonary artery is occluded with a snare to stop inflow into the lung to avoid lung engorgement during the anastomosis. The two pulmonary veins are then occluded with snares. The upper end of the ascending vein is divided and the upper segment is closed primarily. The left atrial appendage is retracted and a side biting clamp is placed on the base of the appendage. The surgeon should avoid constructing the anastomosis on the tip of the appendage, which may result in partial obstruction to flow through a narrow appendage internal orifice. A large incision is made in the pulmonary vein confluence near the base of the appendage and an adjacent incision made in the appendage. A side-to-side anastomosis is constructed with fine polypropylene suture.
2
Secundum Atrial Septal Defect
27
left atrial appendage
anastomosis
pulmonary vein
closed stump of ascending ant caud^-
->ceph
post
FIGURE 2-35. The completed anastomosis is shown with no tension on the pulmonary vein. The upper end of the divided ascending vein was closed primarily.
2-2-6. Repair with Cardiopulmonary
Bypass
Repair of this anomaly can be performed working through a median sternotomy, which is necessary when associated intracardiac anomalies are repaired. This is carried out using cardiopulmonary bypass.
ascending connecting pulmonary vein
left atrial appendage
main pulmonary artery ceph
R<-
-*L
ascending aorta
caud
FIGURE 2-36. The ascending connecting pulmonary vein is exposed in the left upper mediastinum.
28 Color Atlas of Congenital Heart Surgery
opened ascending vein
posterior anastomosis
opened left atrial appendage
ceph R^-
->L
caud
2-37. After placing the child on cardiopulmonary bypass, the upper end of the ascending left pulmonary vein is divided and the end near the innominate vein closed primarily. The lower segment of the ascending vein is shifted caudad. The base of the left atrial appendage is opened and an anastomosis between the vein and atrium constructed. FIGURE
turned down ascending vein
anastomosis
left atrium
R +
2-38. The anastomosis is complete and the vein is attached to the left atrium without tension or obstruction to flow. FIGURE
3
Tricuspid Valve Anomalies
In the pediatric population, surgical repair of the tricuspid valve may be required for tricuspid insufficiency or stenosis, Ebstein's anomaly, or other rare lesions. Operations are carried out working through a right atriotomy using cardiopulmonary bypass, moderate hypothermia (26°C), aortic cross-clamping and cardioplegia, profound local cardiac cooling, and left ventricular venting. Children with tricuspid atresia require single ventricle surgery and this is covered in Chapter 6.
3-1. Tricuspid Insufficiency - DeVega Annuloplasty
3-1. In this child with an atrial septal defect (ASD) and tricuspid insufficiency, the tricuspid valve annulus is dilated, resulting in severe central insufficiency. FIGURE
29
30
Color Atlas of Congenital Heart Surgery
repair suture
anterior leaf
FIGURE 3-2. The tricuspid insufficiency is repaired with a DeVega annuloplasty. A running mattress suture of monofilament material with Teflon® felt support at the ends is placed adjacent to the tricuspid valve annulus. The first arm of the stitch runs immediately adjacent to the annulus, while the second arm is placed more remote from the annulus. The stitch begins opposite the coronary sinus and anterior to the bundle of His. The concept of this procedure is to narrow the annulus at the base of the posterior tricuspid valve leaf. After tying the stitch, the valve is tested by filling the right ventricle with saline from a syringe. The ASD is then closed primarily.
3-2. Ebstein's Anomaly 3-2-1. Mild Ebstein's Anomaly
atrial septal defect
septal leaf
coronary
posterior leaf
annulus
>L caud
FIGURE 3-3. In this child with mild Ebstein's anomaly, the tricuspid valve septal leaf is attached to the septum about 1cm remote from the native valve annulus. The posterior aspect of the posterior leaf is also attached directly to the septum.
3
Tricuspid Valve Anomalies 31
septal leaf
posterior leaf
ceph R<-
annulus
-•L
caud
3-4. The septal leaf is underdeveloped and located remote from the annulus. Anteriorly, its attachment in the region of the commissure is at the annulus. FIGURE
repaired atrial septal defect
repair suture
caud
3-5. The septal and posterior leafs do not meet and in this area with each is attached to the ventricular septum remote from the tricuspid valve annulus.
FIGURE
32 Color Atlas of Congenital Heart Surgery
repaired atrial septal defect
repair suture
3-6. The repair is performed by placing a continuous mattress suture of monofilament material with Teflon® felt support. The stitch is placed deep to the tricuspid valve annulus. With this procedure, the septal and posterior leafs will be approximated so that they meet near the annulus to form a new commissure. The ASD is closed primarily. FIGURE
3-2-2. Severe Ebstein's Anomaly
right atrium
ceph R<-
right ventricle
-•L
caud
FIGURE
3-7. The right atrium is markedly distended due to tricuspid insufficiency.
3
Tricuspid Valve Anomalies 33
"sail like" anterior leaf
ceph R<-
-•L
caud
3-8. After placing the child on cardiopulmonary bypass, the right atrium is incised and a segment of the wall removed to reduce the size of the atrium. The large "sail-like" anterior leaf comprises most of the functional tricuspid valve.
FIGURE
divided free margin of anterior leaf base
fibrous bands
caud
3-9. The base of the anterior leaf is divided from the annulus from an area near the commissure with the septal leaf and extending toward the posterior leaf. By reflecting the free margin of the anterior leaf, multiple fibrous bands are seen attaching the mid part of the leaflet to the free wall of the right ventricle. These fibrous attachments will be divided in order to mobilize the anterior leaf. FIGURE
34 Color Atlas of Congenital Heart Surgery
freed anterior leaf
R^
FIGURE 3-10. After dividing the fibrous bands, the anterior leaf is more mobile and is free floating.
continuous suture to reattach anterior leaf to annulus
reattached anterior leaf
ventricular plication stitches ceph R<-
atrial plication stitches
-•L
caud
3-11. Multiple felted mattress sutures are placed in a longitudinal fashion in the septum/post wall from the tricuspid valve annulus extending toward the apex of the ventricle in order to plicate the posterior wall. This plication is placed posterior and toward the right from the region of the coronary sinus and bundle of His. The thinned portion of the posterior wall of the ventricle is obliterated in order to improve ventricular function postoperatively. Plication sutures are extended into the right atrium posteriorly. The freefloating anterior leaf is shifted clockwise and reattached to the tricuspid valve annulus with a continuous suture. This leaf is positioned so that the end of it will meet the region of the septal leaf in order to obliterate completely the atrialized portion of the ventricle. FIGURE
3
Tricuspid Valve Anomalies 35
3-2-3. Duplication of Tricuspid Valve with Double Inlet Left Ventricle
atrial septal defect
tricuspid valve orifice to right ventricle
tricuspid valve orifice to left ventricle
ceph -•L
caud
3-12. In this child there is duplication of the tricuspid valve. One orifice enters the right ventricle, while the duplicated orifice enters the left ventricle. This can be verified by viewing the left ventricle through the ASD. FIGURE
chords
papillary muscle
ceph R<-
valve leaf
-•L
caud
3-13. The duplicated left ventricular orifice includes valve leaflets as well as rudimentary papillary muscles and chords. This orifice is regurgitant, which allowed a left-toright intracardiac shunt. FIGURE
36 Color Atlas of Congenital Heart Surgery
orifice to left ventricle
ceph R<-
chords
-•L
caud
3-14. The tricuspid orifice entering the right ventricle was adequate in size so the repair was performed by closure of the duplicated left ventricular tricuspid orifice. Multiple stitches are placed in the base of these valve leaflets. FIGURE
orifice to right ventricle
patch
caud
3-15. Repair sutures are placed in a Dacron® patch to close the left ventricular orifice of the duplicated tricuspid valve. The ASD is then closed primarily.
FIGURE
4
Endocardial Cushion Defects
Babies born with these anomalies have a deficiency of the atrial septum, ventricular septum, and/or abnormalities of the atrio-ventricular (AV) valves. In partial AV canal defect, there is absence of the septum primum and usually a cleft in the anterior mitral leaflet. This anomaly is synonymous with primum atrial septal defect. The intermediate form of AV canal encompasses a primum atrial septal defect, ventricular septal defect usually in the inlet position, and usually a cleft mitral valve. The annulus between tricuspid and mitral valves is intact. In complete AV canal, the primum atrial septal defect is in continuity with a high ventricular septal defect because there is no annular continuity of mitral and tricuspid valves. This results in a single intracardiac AV valve that functions as both mitral and tricuspid valves. All patients have a significant left-to-right intracardiac shunt and in the presence of complete AV canal there is always severe pulmonary hypertension with or without AV valve regurgitation. Early surgery is necessary because of congestive heart failure and, in the presence of pulmonary artery hypertension, to avoid early development of pulmonary vascular obstructive disease. Other forms of canal deformities not in this classification are common atrium and, some feel, isolated mitral valve cleft. A primary totally corrective operation is almost always performed. In the rare case of a very small and severely nutritionally deprived neonate with complete AV canal, placement of a temporary pulmonary artery band for a few months may allow significant nutritional improvement prior to totally corrective surgery. In the asymptomatic child with partial AV canal, corrective surgery is performed in the early years of life. For the intermediate and complete forms of AV canal, the repair is carried out in the early months of life. Regardless of the lack of symptomatology, the ideal age for total repair is 2 to 4 months. Repair of these anomalies is performed working through a right atriotomy using cardiopulmonary bypass, moderate hypothermia, aortic cross-clamping and cardioplegia, profound local cardiac cooling, and left ventricular venting.
37
38
Color Atlas of Congenital Heart Surgery
4-1. Primum Atrial Septal Defect
cephalad rim of atrial septal defect mitral chord supports cephalad part of anterior mitral leaflet tricuspid septal leaflet is absent cleft in mitral leaflet extends to annulus
fibrous thickening along margin of mitral valve cleft
ceph R«-
->L
caud
FIGURE 4-1. The patient is on cardiopulmonary bypass, and a right atriotomy has been made. The low primum atrial septal defect (ASD) allows excellent exposure of the anterior mitral leaflet, which is lifted into the field. There is a cleft that separates the leaflet into cephalad and caudad components, and it extends to the common annulus between mitral and tricuspid valves. Chordae support the rim of this leaflet at the cleft, and no evidence of mitral regurgitation is apparent. There is thickening and fibrosis of the leaflet above and below the cleft; this provides tissue of good substance into which stitches can be placed for the repair. There is a deficiency or absence of the adjacent tricuspid valve septal leaflet.
rim of primum atrial septal defect
tip of right angle clamp passes through small ventricular septal defect
ceph -•L
caud
FIGURE 4-2. The area beneath
the atrio-ventricular valves is inspected for small ventricular septal defects. The tip of a rightangle clamp passes beneath the tricuspid valve leaflet and through a small ventricular septal defect.
4
Endocardial Cushion Defects
39
stitches in mitral valve cleft
first ASD repair stitch placed on RV surface of inlet septum
thickened mitral leaflet at margin of cleft caud
FIGURE 4-3. The mitral valve cleft is repaired with multiple simple interrupted sutures. The thick fibrous margin of the cleft can be seen. Care is taken not to close the cleft excessively to avoid iatrogenic mitral stenosis, although ideally the cleft is closed to the free margin of the leaflet. The first ASD repair stitch is placed as a mattress suture with a Teflon® felt pledget opposite the cleft. This stitch is placed in the upper ventricular septum along the right ventricular surface and is used as a traction suture during repair of the cleft.
rim of ASD
interrupted mattress sutures for ASD repair passed through septal muscle or in base of tricuspid leaflet
mitral leaflet attachment beneath coronary sinus
caud
stitches beneath coronary sinus near His' bundle
FIGURE 4-4. Additional interrupted mattress sutures with felt pledgets are placed in the upper margin of the ventricular septum or passed through the base of the tricuspid leaflet to be used in closing the lower rim of the ASD. Interrupted sutures are placed in tissue beneath the coronary sinus in the region of the His' bundle placing them superficially and on the left atrial surface of the septum. The posterior annulus attachment of the anterior mitral leaflet is immediately adjacent to the coronary sinus in some cases, and stitches can be safely placed at the base of this leaflet to avoid the His' bundle.
40 Color Atlas of Congenital Heart Surgery
ASD inferior repair stitches are tied
R + caud
4-5. A knitted Dacron® patch is cut to the size and shape of the ASD and the mattress sutures are passed through the patch. Stitches along the inferior rim of the ASD are tied. The aortic clamp is removed, and rewarming started. When normal sinus rhythm is observed, the stitches beneath the coronary sinus are tied. If these stitches are too near the His' bundle, heart block will be observed immediately when the offending stitch is tied. If this occurs, the stitch is removed and replaced. The upper margin of the ASD is closed with a continuous suture while rewarming continues. FIGURE
4-1-1. Primum Atrial Septal Defect with Mitral Leaflet Tissue Deficiency
rim of ASD
leaflet deficiency at base of cleft
ceph R<-
-•L
stitches in cleft near free margin of leaflet
caud
4-6. In another child, cardiopulmonary bypass has been established and the right atrium opened. There is an area of tissue deficiency at the base of the mitral valve cleft near the annulus. Primary closure of this part of the cleft was thought inadvisable for fear this would cause distortion of the mitral leaflet and mitral regurgitation. FIGURE
4
Endocardial Cushion Defects
41
pericardial patch in area of leaflet deficiency ceph R«-
-•L
caud
4-7. The mitral valve cleft near the free margin of the leaflet is closed primarily. A triangular-shaped pericardial autograft is stitched over the area of leaflet deficiency near the annulus. The primum ASD is then closed in the usual way. FIGURE
4-2. Common Atrium
left atrial appendage
mitral valve
ceph R<-
-•L
right pulmonary veins
caud FIGURE 4-8. The child has been placed on cardiopulmonary bypass, and the view is looking through a right atriotomy. There is no atrial septum, and the orifices of the left atrial appendage and right pulmonary veins are seen in the posterior wall of the common atrial chamber. The mitral valve is anterior to the left atrial appendage.
42
Color Atlas of Congenital Heart Surgery
left atrial appendage
common AV valve annulus
tricuspid valve
R<
mitral valve
FIGURE 4-9. The mitral valve is anterior to the left atrial appendage. The mitral and tricuspid valves share a common annulus.
FIGURE 4-10. As is the case in most endocardial cushion defects, there is a cleft in the anterior mitral leaflet, and it is repaired primarily with interrupted sutures. This is important, even in the absence of mitral regurgitation, because closure of the cleft supports the anterior mitral leaflet and may prevent later development of valvular regurgitation. The repair should not be excessive, to cause iatrogenic mitral stenosis; although ideally the cleft should be closed to the free margin of the leaflet. The extent of the repair is also determined by measuring the valve orifice with sized dilators to compare these numbers to the predicted mitral orifice based on body surface area.
4
Endocardial Cushion Defects
FIGURE 4-11. A knitted Dacron®
43
Dacron patch
patch is placed in the position of the atrial septum. Interrupted sutures are placed in the AV valve common annulus and beneath the coronary sinus adjacent to the AV node and His' bundle. The interrupted sutures near the AV valves are tied with the heart arrested with cardioplegia. The aortic cross-clamp is then removed, and the heart is allowed to beat in sinus rhythm. The sutures near the His' bundle are then tied so that normal sinus rhythm can be observed during this part of the procedure. If injury to the His' bundle occurs because of these stitches, complete heart block is seen immediately as they are tied. Offending sutures are removed and replaced. A continuous suture is then placed posteriorly and cephalad to secure the patch to the atrial wall during rewarming.
interrupted sutures in AV valve common annulus
interrupted sutures beneath coronary sinus and His' bundle
>L caud
4-3. Inlet Ventricular Septal Defect The inlet ventricular septal defect is located in the endocardial cushion position and is also known as a perimembranous inlet or Type III ventricular septal defect.
anterior leaflet of tricuspid valve
FIGURE 4-12. The septal and
anterior tricuspid valve leaflets are retracted to expose the VSD. The defect is located primarily beneath the septal leaflet. This position places it caudad to the location of a typical Type II or perimembranous VSD. Exposure through the right atrium is enhanced due to the more caudad location of the VSD. A secundum atrial septal defect is also present.
VSD
atrial septal defect septal leaflet of tricuspid valve ceph -•L
caud
44 Color Atlas of Congenital Heart Surgery
stitches at base of septal leaflet of tricuspid valve stitches near His' bundle
coronary sinus atrial septal defect
R<
4-13. Interrupted mattress sutures are placed along the rim of the VSD. At the base of the tricuspid valve septal leaflet, these sutures may be passed through the leaflet near the annulus. The His' bundle is located near the posterior inferior rim of the VSD in the region anterior to the coronary sinus. Sutures here must be placed superficially along the right ventricular surface of the rim of the VSD to avoid His' bundle injury. FIGURE
Dacron patch
tricuspid valve septal leaflet
atrial septal defect
FIGURE 4-14. Stitches are placed in a knitted Dacron® patch and tied. Part of the patch is hidden from view beneath the tricuspid valve septal leaflet. The ASD will be closed with a separate Dacron® patch.
4
Endocardial Cushion Defects 45
4-4. Complete Atrioventricularis Communis or Atrio-Ventricular Canal
primum atrial septal defect
ventricular septal defect beneath AV valve leaflets
ceph R<-
secundum atrial septal defect
-•L
caud
4-15. Exposure is through a right atriotomy. A primum ASD is in the lower or caudad part of the atrial septum, and it is in continuity with a high VSD located beneath the flaccid AV valve leaflets. In this child, a secundum ASD is also present. FIGURE
common anterior leaflet of AV valve chord attaching common anterior leaflet to ventricular septum ventricular septum tricuspid orifice mitral orifice
ceph R<-
->L
common posterior leaflet of AV valve
caud
4-16, The common anterior leaflet of the AV valve or anterior bridging leaflet extends over the tricuspid and mitral valve orifices. Similarly, the common posterior leaflet or posterior bridging leaflet of the AV valve extends over both valve orifices. There is no fibrous annular continuity of the mitral and tricuspid valves. Here, there are chordae that attach the common anterior leaflet to the ventricular septum (Type A AV Canal by the Rastelli classification). FIGURE
46
Color Atlas of Congenital Heart Surgery
chordal attachments of common anterior leaflet of AV valve are only to RV papillary muscle
right ventricular papillary muscle
ceph R<-
-•L
caud
FIGURE 4-17. In this child, there are chordal attachments from the common anterior leaflet only to a papillary muscle in the right ventricle (Type B AV Canal by the Rastelli classification).
free floating common anterior leaflet of AV valve without chordal attachments to ventricular septum
ventricular septum
R +
FIGURE 4-18. The common anterior leaflet is free floating with no chordal attachments to the ventricular septum (Type C Complete AV Canal by the Rastelli classification).
4
Endocardial Cushion Defects 47
4-4-1. Complete Atrio-Ventricular Canal: Patch Repair
ceph R<-
->L
caud FIGURE 4-19. The common anterior leaflet of the AV valve is undivided. When the onepatch technique for repair is used, this leaflet is surgically divided to the annulus, leaving slightly more tissue on the mitral valve portion of the leaflet.
common anterior leaflet of AV valve
ceph R <
>L
caud FIGURE 4-20. The common anterior leaflet of the AV valve in this child is naturally divided. The cleft or division in this valve may be used in the one-patch repair. If the mitral valve is deficient, the natural cleft may be closed in favor of a surgically created division toward the tricuspid valve portion of the leaflet so as to enlarge the mitral portion of the leaflet.
48 Color Atlas of Congenital Heart Surgery
common anterior leaflet of AV valve
common posterior leaflet of AV valve ceph R«-
-•L
caud
4-21. The common posterior leaflet of the AV valve may be naturally divided (less common) or in this case surgically divided to near the posterior annulus. The common anterior leaflet was also surgically divided. This is the first step in the one-patch repair of complete AV canal.
FIGURE
mitral portion of common anterior leaflet
ceph R«-
mitral portion of common posterior leaflet
-•L
caud
FIGURE 4-22. The mitral portions of the common anterior leaflet and common posterior leaflet, respectively, have been approximated with interrupted sutures placed at the extremes of the mitral leaflet repair. Ideally the mitral valve is closed to the free margin of the new leaflet, and the proposed mitral orifice is measured with sizers after repair to prevent iatrogenic mitral stenosis.
4
Endocardial Cushion Defects 49
mitral portion of common anterior leaflet
mitral portion of common posterior leaflet
4-23. The mitral leaflet repair is completed with simple interrupted sutures. The newly constructed anterior mitral valve leaflet is now free floating. The new mitral orifice must be carefully measured using sized dilators. The opening is compared to a normal valves based on body surface area to avoid excessive closure of the valve, which may result in iatrogenic mitral stenosis. FIGURE
new anterior mitral leaflet
ventricular septum
ventricular septal defect
R^ caud
4-24. The new anterior mitral leaflet is lifted to expose the ventricular septal defect beneath. To close the VSD, felted horizontal mattress sutures will be placed on the right ventricular surface of the upper rim of the ventricular septum. FIGURE
50
Color Atlas of Congenital Heart Surgery
knitted Dacron patch felted mattress sutures in ventricular septum
region of His' bundle
sutures beneath coronary sinus
ceph R<-
-•L
caud
FIGURE 4-25. A knitted Dacron® patch is cut to conform to the size and shape of the VSD and primum ASD. Stitches previously placed in the upper rim of the ventricular septum are placed in the lower rim of the Dacron® patch and tied. The most posterior ventricular septal defect stitches are near the His' bundle and should be placed slightly remote from the rim of the ventricular septum. Additional felted mattress sutures are placed superficially in the tissue beneath the coronary sinus. These sutures are in the region of the His' bundle and are tied after the aortic cross-clamp is removed and the heart is beating in sinus rhythm. If the His' bundle is damaged by these sutures, heart block will occur when they are tied. In this event, the sutures are removed and placed again.
reconstructed anterior mitral leaflet
ventricular septal defect closed by lower part of Dacron patch
stitches beneath coronary sinus ceph -•L
caud
FIGURE 4-26. The Dacron® patch is retracted anteriorly to expose the ventricular septal defect beneath the new anterior mitral leaflet. Felted mattress sutures will be placed in the base of this leaflet near the Dacron® patch. Chordae of this leaflet are left intact. The sutures will be passed through the Dacron® patch at an appropriate level on the patch to mimic the position of the mitral leaflet in its natural position during ventricular systole. Care must be exercised to avoid positioning the leaflet too far cephalad on the patch, which might result in a tented and immobile leaflet. It is better to err on the side of attaching the leaflet nearer the ventricular septum. These valve-fixing sutures can also be passed through the adjacent tricuspid leaflets on the right ventricular surface of the patch.
4
Endocardial Cushion Defects 51
mitral leaflet attached to patch by felted mattress sutures
retracted Dacron patch
ceph R^-
-•L
stitches beneath coronary sinus
caud FIGURE 4-27. The new anterior mitral leaflet is attached to the Dacron® patch by felted mattress sutures that have been tied.
tricuspid valve leaflet attached to Dacron patch
ceph R««-
->L
stitches beneath coronary sinus
caud
4-28. The right atrial surface of the patch is seen. Mitral valve sutures passed through the patch have also passed through the tricuspid valve leaflets, and the stitches are tied. Stitches beneath the coronary sinus are now passed through the adjacent patch and the aortic clamp is removed to commence rewarming. When conducted rhythm is observed, the coronary sinus stitches are tied. If the His' bundle is damaged when these stitches are tied, heart block is seen immediately. In that event offending sutures are removed and replaced. FIGURE
52 Color Atlas of Congenital Heart Surgery
upper part of Dacron patch stitched over primum atrial septal defect
ceph R«-
-•L
t caud
4-29. While rewarming continues, the upper part of the Dacron® patch is stitched to the atrial septum with a continuous suture. The right atriotomy is closed, and a left atrial pressure monitoring line is placed in the ventricular vent site near the right upper pulmonary vein shortly before discontinuing cardiopulmonary bypass. FIGURE
Dacron patch closing atrial septal defect
reconstructed tricuspid valve leaflets
ceph R<-
-•L
caud
4-30. In this child, 2.5 months after corrective surgery, the Dacron® patch is endothelialized, depicting the fate of the Dacron® material used in this repair.
FIGURE
4 4-4-2. Complete Atrio-Ventricular
Endocardial Cushion Defects 53
Canal: Double-Patch
Repair
Currently this is my preferred repair. Iatric incisions in the bridging leaflets are avoided and there is less risk of valve repair breakdown following surgery. This is especially important because most AV repairs today are performed in young small infants with more fragile valvular tissue.
common anterior leaflet ventricular septal defect
FIGURE 4-31. The exposure is through a right atriotomy after the infant is placed on cardiopulmonary bypass. The high VSD is in continuity with the low primum ASD. There is a single intracardiac AV valve comprised of common anterior and common posterior leaflets that span across the septal defects from left heart to right heart. Stay sutures have been placed in these valve leaflets for optimum exposure.
common posterior leaflet primum atrial septal defect
caud
common anterior leaflet
chords at free margin of proposed new anterior mitral leaflet
R**
posterior leaflet
FIGURE 4-32. The leaflets are carefully inspected and in most patients a fibrous rim of jet lesion demarks the point where the leaflets naturally come together during systole. This fibrous tissue is useful during the valve repair to close the new cleft because of its substance and ability to hold sutures. Both valve leaflets are inspected in order to identify the point where chordae attach to what will be the new free margin of the new anterior mitral leaflet. Great care is taken at this point in the operation to precisely define the point where the common anterior and common posterior leaflets will meet to form the new anterior mitral leaflet to avoid asymetric apposition. The surgeon can also inject saline under pressure in the ventricular chambers to open the valve leaflets to help identify the proper alignment for repair.
54 Color Atlas of Congenital Heart Surgery
common anterior leaflet
marking stitch at free margin of new anterior mitral leaflet
common posterior leaflet
4-33. When the point on the anterior and posterior leaflets near the left atrium and near the chordal insertions has been identified, a marking suture is placed to bring these points together. This posterior marking stitch is left in place during subsequent of the repair. FIGURE
marking stitches at base of new anterior mitral leaflet cleft
ceph R«-
->L
marking stitch at free margin of new anterior mitral leaflet
•
caud FIGURE 4-34. Marking sutures are then placed at the more anterior extreme of the proposed anterior leaf mitral valve cleft and these are tied. These markers are left in place but do not impede exposure during the repair prior to cleft closure.
4
Endocardial Cushion Defects 55
Dacron patch for VSD repair
ventricular septal stitches
4-35. The felted mattress sutures are placed along the right ventricular surface of the upper margin of the ventricular septum adjacent to the ventricular septal defect. Posteriorly, these sutures are placed more superficially and somewhat remotely from the rim of the VSD in order to avoid the bundle of His' located in this region. Sutures are then passed through a knitted Dacron® patch that is cut to the size and shape of the VSD. Although the upper rim of the patch here is shown to be flat, that edge is often scalloped to allow a better fit for the new valve leaflets that will be attached here. FIGURE
stitches in upper rim of VSD patch
VSD repair patch
4-36. The VSD patch has been placed over the defect and the septal rim stitches tied. Nonfelted sutures are next passed through the upper rim of the patch. The size of the patch and the shape of the upper rim are such that after completing the repair the AV valve leaflets will rest on the patch in a position similar to that during end systole. FIGURE
56 Color Atlas of Congenital Heart Surgery
••v£—
common anterior leaflet
stitches passed through common anterior leaflet
VSD repair patch
ceph R<-
->L
caud
4-37. The stitches in the upper rim of the VSD patch are passed through the common anterior and common posterior valve leaflets, respectively. The previously placed marking sutures indicate the line of these sutures so that the previously selected valve tissue can be placed appropriately on the mitral or tricuspid side of the patch. Generally, around two thirds of the undivided bridging leaflets are placed on the mitral valve side. If there is a natural division on the bridging leaflet, the transvalvular stitches can be placed in the base of the natural division of the leaflet. FIGURE
VSD repair patch stitches repairing cleft in anterior mitral leaflet
ceph R<-
->L
stitches through common posterior leaflet
caud FIGURE 4-38. Interrupted fine monofilament sutures are now used to close the cleft in the new anterior mitral leaflet. At this point the transvalvular stitches from the upper rim of the VSD patch are left untied. The new mitral valve orifice is measured carefully and the size compared with normal values in order to avoid excessive closure of the cleft and iatrogenic mitral stenosis.
4
Endocardial Cushion Defects 57
patch to close atrial septal defect
ceph R«*-
-•L
caud FIGURE 4-39. A second Dacron® patch is cut to conform to the size and shape of the primum ASD.
AV valve separating stitches in ASD patch
FIGURE 4-40. The transvalvular stitches from the upper rim of the VSD patch are placed through the base of the ASD patch.
58
Color Atlas of Congenital Heart Surgery
ASD patch
stitches beneath coronary sinus
coronary sinus R^
FIGURE 4-41. The ASD patch has been lowered into position. Additional felted mattress sutures are placed superficially beneath the coronary sinus. If they can be placed toward the left atrium it is safer in order to avoid damage to the His' bundle which is in this region. The stitches are passed through the ASD patch. Next, the stitches along the base of the patch are tied leaving the few stitches beneath the coronary sinus untied. The aortic clamp is removed and rewarming is commenced. One must await the observation of sinus rhythm before proceeding. Only after observing sinus rhythm are the interrupted sutures near the coronary sinus tied. If they are too near the His' bundle, third-degree block will be observed immediately and the offending stitch is removed and replaced.
VSD repair patch
ASD repair patch
FIGURE
4-42. Stitches
across
the base of the ASD patch have been tied, as have the stitches beneath the coronary sinus. The upper rim of the patch is attached to the atrial septum with a continuous suture to complete the repair. A left atrial line is left through the ventricular vent site near the right upper pulmonary vein.
4 4-4-3. Complete Atrio-Ventricular
Endocardial Cushion Defects
Canal: Modified
Single-Patch
59
Repair
The concept of this complete AV canal repair includes obliteration of the VSD by attaching the AV valve leaflets directly to the top of the ventricular septum avoiding the need for placement of a patch beneath the AV valves. In theory this can be performed more rapidly than other repairs because the placement of the VSD patch is omitted. An additional advantage is avoiding a surgical incision in AV valve tissue (as in the standard one-patch technique), which is especially applicable in the small infant who may have fragile valve tissue. Of course, the same technique is also used in the classic two-patch repair. My bias is for use of the modified single-patch repair only in patients who have a very shallow VSD. Otherwise, the AV valve leaflets may be distorted by attaching them in an unnatural position to the top of the ventricular septum which may result in significant residual mitral regurgitation. This operation is performed with moderate systemic hypothermia, aortic crossclamping, cardioplegia, and profound local cardiac cooling.
common anterior leaflet
marking stitch in mitral valve cleft near free margin
marking stitch in mitral valve cleft near ventricular septum
ceph R«-
common posterior leaflet
-•L
caud
4-43. The child has been placed on cardiopulmonary bypass and cardioplegia delivered after applying the aortic cross-clamp. A right atriotomy is made. Initially the common anterior leaf and common posterior leaf are carefully evaluated to determine the appropriate position of the new cleft in the proposed new anterior mitral valve leaf. The first marking stitch is placed between the common anterior and common posterior leafs, respectively, near the free margin of each leaflet. The location of the new free margin can usually be identified by insertion of chords at this point. The proposed cleft in the new anterior mitral valve leaf is identified and appropriate marking stitches placed at the septal end of this cleft. FIGURE
60 Color Atlas of Congenital Heart Surgery
common anterior leaflet
natural division in leaflet
marking stitches in cleft near ventricular septum
common posterior leaflet
ceph R<-
-•L
primum ASD rim
caud
4-44. In this child, there is a natural division in the common anterior leaf. In order to enlarge the new mitral valve leaflet, this natural division will be closed so that the common anterior leaf can be divided by sutures nearer the tricuspid orifice. FIGURE
felted stitches in ventricular septum for VSD repair
shallow VSD
caud
4-45. Felted mattress sutures are placed across the top of the ventricular septum on the right ventricular surface for the VSD repair. In this case, the VSD is shallow so that distortion of the valve should be minimal when these stitches are tied. FIGURE
4
Endocardial Cushion Defects
61
VSD repair stitches passed through AV valve leaflets
ceph
R<-
-•L
caud
FIGURE 4-46. The VSD repair stitches have been passed through the AV valve leaflets in order to separate them into tricuspid and mitral components.
natural division in common anterior leaflet
—
marker stitches in cleft
ceph R«-
pnmum ASD
-•L
caud
SHMK
FIGURE 4-47. The natural division in the common anterior leaf and the new cleft in the new anterior mitral leaf are exposed. The original marking sutures are used to align the leaflets properly.
62 Color Atlas of Congenital Heart Surgery
stitches closing natural division in common anterior leaflet
cleft repair stitches
ceph VSD repair R«-
caud
^SCIffHVJI^^IHHIi^HIHHIHIIH^^^HHHHBk
through A-V valve
4-48. Multiple simple sutures of fine polypropylene are placed to close the natural division in the common anterior leaflet and also to close the cleft in the new anterior mitral leaflet. FIGURE
pnmum ASD
stitches beneath coronary sinus
4-49. Felted mattress sutures are placed beneath the coronary sinus for use in the ASD repair. These stitches are superficial and on the left atrial surface of atrial wall beneath the coronary sinus. Some of these stitches are placed in the annulus of the mitral valve because this is always remote from the His' bundle. FIGURE
4
Endocardial Cushion Defects 63
felted VSD repair stitches
ceph R<-
-•L
caud
4-50. A patch cut to the size of the ASD is placed in the wound and the VSD repair sutures that pass through the AV valve leaflet tissue are now placed in this patch. FIGURE
VSD repair stitches
ceph R<-
->L
caud
4-51. Stitches in the base of the Dacron® patch which pass through the AV valve leaflets are tied. With such the VSD is obliterated. FIGURE
64 Color Atlas of Congenital Heart Surgery
repaired division in common anterior leaf
repaired anterior mitral valve leaflet
ASD patch
stitches beneath coronary sinus caud
4-52. Stitches placed beneath the coronary sinus are exposed and now these stitches will be placed in the Dacron® patch. The mitral valve cleft repair sutures are seen, as are the sutures that close the natural division in the common anterior leaf.
FIGURE
felted stitches in upper rim of ventricular septum
Dacron patch on top of AV valves
stitches beneath coronary sinus
R^
4-53. The ASD patch is in position and the stitches beneath the coronary sinus are placed in the Dacron® patch. The aortic cross-clamp is now removed and rewarming is commenced. Cardiac rhythm may initially be abnormal but usually complete heart block will then convert to sinus rhythm. While observing sinus rhythm, the coronary sinus stitches are tied. If they have injured the His' bundle, heart block will be seen immediately as they are tied. If such occurs the offending stitch is removed and replaced. Tying these sutures with a beating heart is safe because atrial tissue is not dynamic in contradistinction to tying ventricular septal stitches, which is almost always done with cardiac standstill. FIGURE
4
Endocardial Cushion Defects
65
ASD patch
ceph R<-
-•L
caud FIGURE 4-54. To complete the repair, the upper rim of the Dacron® patch is attached to the atrial septum with a continuous suture. A left atrial line is left through the ventricular vent site near the right upper pulmonary vein.
4-5. Complete Atrio-Ventricular Canal and Tetralogy of Fallot The surgical challenge of this uncommon anomaly is related to aortic overriding and the resulting necessity to place the VSD patch more anteriorly and around the aortic valve annulus in order to construct an unobstructed left ventricle to aorta tunnel. I prefer using a two-patch technique for this repair. With such, the dimensions of the VSD patch of appropriate size and shape are more easily predicted. In most cases the complete repair can be performed working only through a right atriotomy while retracting AV valve leaflets. If the cephalad end of the VSD is difficult to repair through this exposure, a high right ventriculotomy should be performed for completion of the repair. When there is right ventricular outflow tract hypoplasia, a transannular outflow tract patch is used rather than the placement of a homograft valved conduit even though early after surgery ventricular performance may be somewhat compromised by pulmonary insufficiency. This disadvantage is offset by avoiding the need for homograft valve exchange due to patient growth, which would be necessary in most patients. If right ventricular dilatation and failure occur some years later, a valve can be placed at this time.
66 Color Atlas of Congenital Heart Surgery
main pulmonary artery
aorta
ceph R«-
->L
caud
4-55. In this anomaly, the aorta is located more anteriorly than normal and overrides the ventricular septum. The pulmonary valve and main pulmonary artery here are moderately hypoplastic. FIGURE
atrial septal defect
common anterior AV valve leaflet ventricular septal defect
R^ caud FIGURE 4-56. After cardiopulmonary bypass is established, and cardiac standstill achieved with aortic clamping and cardioplegia, a right atriotomy is made. The common anterior AV valve leaflet is free floating and undivided (Rastelli Type C), which is the usual case in this anomaly. A high VSD is in continuity with a low primum ASD.
4
Endocardial Cushion Defects 67
common posterior AV valve leaflet
4-57. The common posterior AV valve leaflet is well formed and undivided. It is attached to the upper margin of the ventricular septum by multiple chordae. FIGURE
common anterior AV valve leaflet aortic valve
ventricular septum
ceph R<-
-•L
caud
4-58. The common anterior AV valve leaflet is retracted. The VSD is located in the upper ventricular septum. With overriding of the aorta, the aortic valve is located, in part, over the right ventricle. FIGURE
68
Color Atlas of Congenital Heart Surgery
ventricular septal defect closure stitches
ceph R<-
-bi-
caud
FIGURE 4-59. Interrupted multifilament mattress sutures with Teflon® felt pledgets are placed along the upper margin of the ventricular septum. Posteriorly, stitches are more superficial and slightly remote from the VSD to avoid the area of conductive tissue, which is located in this region. Anteriorly, stitches are placed as far as the exposure will allow. The final VSD closure stitches at the anterior extreme of the VSD will subsequently be placed working through a high right ventriculotomy.
common anterior AV valve leaflet
patch closing ventricular septal defect
common posterior AV valve leaflet
>L
FIGURE 4-60. The VSD
repair
stitches have been placed in a Dacron® patch and tied. This patch is wider and more redundant at the anterior end beneath the common anterior AV valve leaflet, which is necessary because the anterior end of the VSD incorporates the aortic valve. Additional patch material is needed to stitch around the annulus of the valve that is located in the right ventricle.
4
stitches from upper rim of VSD patch passed through AV valve leaflets
ceph R<-
Endocardial Cushion Defects 69
-•L
caud
4-61. Sutures are then passed through the upper rim of the VSD patch adjacent to the AV valve leaflets. The stitches are passed through the valve to separate this into separate mitral and tricuspid components. FIGURE
valve separating sutures
cleft in new anterior mitral valve leaflet
R^
4-62. A cleft is now created by approximating adjacent mitral portions of the common anterior and common posterior AV valve leaflets, respectively. The VSD patch is seen beneath the valve leaflets while the valve separating sutures pass through the AV valve leaflets. FIGURE
70 Color Atlas of Congenital Heart Surgery
repaired cleft
ceph
R<-
-+L
caud FIGURE
4-63. Multiple stitches are used to close the cleft in the new anterior mitral leaflet.
stitches beneath coronary sinus
4-64. A second Dacron® patch is cut to conform to the ASD. The AV valve separating stitches are placed in the base of this patch and tied. Additional stitches are placed beneath the coronary sinus and then passed through the posterior margin of the new patch. These stitches are placed superficially and in tissue nearer the adjacent mitral valve leaflet in order to avoid the His' bundle. FIGURE
4
Endocardial Cushion Defects 71
ASD patch
stitches beneath coronary sinus
FIGURE 4-65. The aortic cross-clamp is removed and warming commenced. After sinus rhythm is observed, the stitches beneath the coronary sinus are tied. If these injure the His' bundle, tissue heart block will be observed immediately on the electrocardiogram (EKG) tracing and the stitches are replaced. The upper rim of the ASD patch is then stitched to the margin of the ASD.
main pulmonary artery
ventriculotomy
caud FIGURE 4-66. In this child, it was not possible to repair the anterior and cephalad end of the VSD working through the atriotomy. In addition, the right ventricular outflow tract was restrictive due to infundibular muscle obstruction and a small pulmonary valve annulus, so a high right ventriculotomy is made.
72 Color Atlas of Congenital Heart Surgery
VSD patch
unclosed VSD
ceph R<-
-•L
t caud
4-67. The upper end of the VSD patch is seen and additional stitches are placed to attach this end of the patch to the ventricular septum. FIGURE
main pulmonary artery
tissue outflow tract patch
ceph R<-
->L
caud
4-68. The right ventricular outflow tract is restrictive above the infundibulum and the ventriculotomy is extended across the pulmonary valve annulus and proximal main pulmonary artery. A pericardial patch is used here to suture over the right ventricular outflow tract for reconstruction. Currently, a Gore-Tex® patch or a homograft pulmonary artery wall patch is preferred in this position. FIGURE
4
Endocardial Cushion Defects 73
patch in outflow tract
R^
FIGURE 4-69. The completed outflow tract reconstruction is shown with the tissue patch placed over the upper right ventricle and proximal main pulmonary artery.
5
Ventricular Septal Defects
Repair of ventricular septal defects (VSD) is performed with cardiopulmonary bypass and moderate hypothermia, aortic cross-clamping with cardioplegia, and profound local cardiac cooling. In small infants with associated complex anomalies, maximal exposure may be gained with cardiopulmonary bypass, deep hypothermia, and low-flow cerebral perfusion (about 0.25-0.5 L/min/m2) because total circulatory arrest is almost never used. The two cavae are selectively cannulated, and this allows work within the heart to progress during cooling and rewarming. Most ventricular septal defects are repaired by working through a right atriotomy. If exposure of a subpulmonary VSD is not ideal through this approach, a small transverse right ventriculotomy placed immediately below the pulmonary valve annulus is used or the defect is closed working through the proximal main pulmonary artery or, rarely, the ascending aorta. An apical VSD may be closed through a small, low right ventriculotomy, while multiple muscular VSDs may rarely require an apical left ventriculotomy. Most defects are closed with interrupted pledgeted mattress stitches and a knitted Dacron® patch. Knitted Dacron® is preferred because it facilitates tissue ingrowth and early complete endothelialization of the patch. Only the smallest VSDs are closed primarily without a patch. A running stitch technique is used rarely, in contradistinction to infants who undergo total repair of truncus arteriosus; because the right ventriculotomy exposure allows this technique to be used with facility. It is mandatory to tie stitches in the ventricular septum with the heart arrested and relaxed. Otherwise, there is a risk of tearing of septal muscle as stitches are tied. With complex low muscular VSD's the sandwich patch technique can be used avoiding placement of stitches in septal tissue.
5-1. Perimembranous Ventricular Septal Defect I prefer the transatrial approach to repair membranous VSD. There is usually excellent exposure of the entire defect, especially along the inferior and posterior rims, where conductive tissue is located.
5 FIGURE 5-1. The child has been
placed on cardiopulmonary bypass and systemically cooled. The aorta is clamped and cardioplegia solution is infused. A right atriotomy is made, and the perimembranous VSD is exposed by retracting the tricuspid valve leaflets. To stabilize the exposure, stay sutures are placed in the atrial wall at the atriotomy and in the anterior and septal leaflets of the tricuspid valve.
Ventricular Septal Defects
75
anterior tricuspid valve leaflet
ventricular septal defect septal tricuspid valve leaflet ceph A
R <
> L
caud
anterior tricuspid
valve leaflet attached to posterior rim of VSD
ventricular septal defect septal leaflet of tricuspid valve ceph R<-
-•L
caud
FIGURE 5-2. Multiple interrupted multifilament mattress sutures with Teflon® felt pledgets are placed around the rim of the ventricular septal defect. When the septal tricuspid valve leaflet is attached to the margin of the VSD along the posterior and inferior rim of the defect, sutures are passed through the base of this leaflet to avoid conductive tissue. If there is no septal leaf attachment here, sutures are placed superficially and directly in the rim of the VSD rather than in septal tissue remote from the VSD rim. More anteriorly, along the inferior margin of the VSD, stitches are placed near the margin of the VSD in muscular septum. Generally, these stitches are placed through 25% of the thickness of the septum. Along the anterior margin of the VSD, stitches are placed in about 50% of the thickness of the septum on the right ventricular surface. Stitches along the cephalad rim of the ventricular septal defect are placed similarly, or they can be placed in the base of the anterior tricuspid valve leaflet when the latter is attached to this rim of the VSD. Care must be exercised to avoid injury to aortic valve cusps that are located in this area. The posterior margin of the VSD is usually in continuity with the base of the anterior tricuspid valve leaflet. Stitches here are passed through the base of this leaflet near the annulus.
Congenital Heart Surgery
fibrous remnant of membranous septum in posterior inferior corner
ceph R^-
-•L
caud
FIGURE 5-3. In some patients a fibrous remnant of the membranous septum is present in the posterior inferior corner of the VSD. When present, the corner stitch is placed in this remnant that is adjacent to but separate from the His' bundle.
anterior tricuspid valve leaflet attached to VSD posterior rim
ceph R<-
-•L
caud
FIGURE 5-4. The anterior tricuspid valve leaflet is attached to the posterior rim of the VSD. A small probe can identify the base of the leaflet near the annulus to identify the precise location for placement of the stitches.
5
Ventricular Septal Defects
77
aortic valve cusp adjacent to cephalad margin of VSD
R^
FIGURE 5-5. The cephalad margin of the VSD is adjacent to the aortic valve. The aortic valve annulus is identified so that stitches can be placed in this structure or in adjacent right ventricular muscle and avoid damage to the aortic valve cusps. To identify the cusps, the aortic valve is observed in the closed position while cardioplegia solution is infused in the aortic root.
anterior leaflet
septal leaflet ceph R<-
-•L
caud FIGURE
5-6. Stitches have been placed circumferentially in the rim of the VSD.
78 Color Atlas of Congenital Heart Surgery
VSD patch
ceph R<-
-•L
caud
5-7. Stitches are placed in a knitted Dacron® patch which is cut to conform to the size and shape of the VSD. FIGURE
Dacron patch
ceph R<-
-•L
caud
5-8. Repair stitches placed in a Dacron® patch are tied. The integrity of the closure is checked with a 1-mm probe that is used to gently probe beneath the patch. Residual openings can usually be found and closed at this point in the operation. FIGURE
5
Ventricular Septal Defects
79
FIGURE 5-9. In another patient
who was re-explored 7 years after VSD repair, the totally endothelialized patch is invisible beneath endocardial tissue, as seen through a right atriotomy.
endothelialized patch
5-2. Subpulmonary Ventricular Septal Defect Subpulmonary ventricular septal defects are located high in the ventricular septum and immediately below the pulmonary valve. Myocardial relaxation with cardioplegic arrest allows the upper septum to be retracted inferiorly, so that many subpulmonary defects can be closed completely through the transatrial approach. In many cases, an aortic valve cusp is intimate with the rim of this ventricular defect and care must be used to avoid damage to the valve. If exposure through the atrium is not satisfactory, this approach should be aborted and a high small transverse right ventriculotomy or proximal main pulmonary arteriotomy used for the repair. Working through the proximal ascending aorta with retraction of the aortic valve is an alternative repair exposure.
subpulmonary ventricular septal defect
FIGURE 5-10. The
supracristal
subpulmonary VSD is seen immediately below the pulmonary valve by looking through a right atriotomy while retracting the anterior tricuspid valve leaflet.
membranous ventricular septum
80 Color Atlas of Congenital Heart Surgery
ventricular septal defect
ceph A R^- - • L caud
5-11. With retraction, the upper ventricular septum is shifted caudad for satisfactory exposure of the VSD, which was closed through this approach. FIGURE
main pulmonary artery
pulmonary valve leaflet
ventricular septal defect ceph R<-
-•L
caud FIGURE 5-12. In another patient, exposure of the VSD is through a proximal transverse main pulmonary arterotomy with retraction of pulmonary valve leaflets.
5
Ventricular Septal Defects
81
pulmonary valve leaflet
aortic valve leaflet
ceph R<-
-•L
caud
5-13. With the aortic valve in the closed position during delivery of cardioplegia, an aortic cusp fills most of the VSD. Without repair, the resulting stretching and distention of the cusp may lead to aortic insufficiency. Felted mattress stitches are placed around the rim of the VSD for the repair. Because there is a common annulus between the aortic and pulmonary valves, stitches at the cephalad rim of the VSD are placed at the base of a pulmonary valve leaflet through the common semilunar valve annulus without felt pledgets. FIGURE
pulmonary valve leaflet
ceph R<-
VSD patch
-^L
caud FIGURE 5-14. Stitches are placed in a Dacron® patch and then tied for repair of the defect. Along the cephalad rim, stitches are placed in the valve annulus so there should be little distortion of the pulmonary valve leaflet.
82
Color Atlas of Congenital Heart Surgery
pulmonary valve annulus
ventricular septal defect
ceph R<-
-•L
caud
FIGURE 5-15. In another patient, the subpulmonary VSD is exposed through a high right ventriculotomy. A dilated and prolapsing aortic valve cusp is present in the upper part of the VSD; this resulted in aortic insufficiency. The VSD is adjacent to the pulmonary valve annulus.
ceph R^-
->L
caud
FIGURE 5-16. The aortic valve is in the closed position with distention of the aortic root during cardioplegia solution infusion. The distended aortic valve cusp fills much of the VSD, and one can appreciate the mechanism by which aortic insufficiency develops in children with this anomaly. This child underwent concomitant aortic valvuloplasty.
5
Ventricular Septal Defects 83
stretched and insufficient right coronary cusp
non coronary cusp
caud L<-
left coronary cusp
-•R
ceph FIGURE 5-17. The proximal ascending aorta has been opened. The aortic valve is trileafed with normal left coronary and noncoronary cusps. A blunt sucker tip is placed in the sinus of Valsalva of the right coronary cusp, demonstrating the stretched and prolapsed cusp which allows aortic insufficiency.
redundancy in right leaflet
marking stitch in corpora arantii caud L<«-
-•R
ceph
5-18. A marking stitch is passed through the corpora arantii of the left and noncoronary cusps. The free margin of the right coronary cusp is pulled toward the right-left cusp commissure to exclude redundancy in the cusp. The marking stitch is passed through the region of the right cusp adjacent to the other two corpora arantii. A second stitch is placed in the right-left cusp commissure, imbricating the redundant right cusp. The right coronary cusp is now supported and should not be insufficient. FIGURE
84
Color Atlas of Congenital Heart Surgery
two mattress stitches in redundant cusp
competent right coronary cusp
caud L«-
felted stitch on top of new commissure
-•R
ceph
FIGURE 5-19. Two pledgeted mattress sutures are passed through the redundant right cusp tissue and through the aortic wall, firmly pressing the redundant tissue against the aortic wall. A third pledgeted mattress suture is placed over the top of the new right-left commissure. One arm of this mattress stitch passes through the right and left cusps, respectively, at the commissure. The other arm of the stitch passes to the child's left, through the full thickness of the aorta in the right cusp sinus of Valsalva at the commissure. It is passed through a second felt pledget and then from outside to within the aorta in the left cusp sinus of Valsalva at the commissure. Both arms of this stitch are then passed through the end of the original felt pledget that is used in the mattress suture. When it is tied, this stitch forms a buttress or support with the pledget on top of the new commissure. This prevents blood from dissecting behind the valve repair during diastole. The right coronary cusp is again probed with a blunt sucker tip to demonstrate its competency following obliteration of the prolapse. The aorta is closed and attention is turned to repair of the VSD. The competency of the aortic valve can be observed while cardioplegia solution is injected in the aortic root and the aortic valve is viewed through the VSD. Later, during rewarming, when the aortic clamp has been removed, left ventricular vent return is again measured to determine presence or absence of significant aortic insufficiency.
marking stitch in corpora arantii
redundancy of left coronary cusp
>L
FIGURE 5-20. In another
patient,
the proximal ascending aorta has been opened. The aortic valve has three leaflets with distention and stretching of the left coronary cusp. A marking stitch is placed in the corpora arantii of the right and noncoronary cusps. The left cusp is pulled toward the patient's left and redundancy in this leaflet is seen at the right-left cusp commissure. The corpora arantii marking suture is then placed additionally in the adjacent region of the new left coronary cusp.
5
Ventricular Septal Defects 85
aortic wall
felted imbricating sutures
5-21. Two felted mattress sutures are placed in the redundant portion of the left coronary cusp. These are placed through the wall of the aorta and supported with additional pledgets before tying them outside the aorta. With such, the redundant portion of the leaflet is excluded from the valve apparatus and attached to the lateral aortic wall. The left coronary cusp is now the same size as the other two leaflets. FIGURE
felted stitch on top of commissure
caud FIGURE 5-22. A felted mattress suture is placed on top of the new left-right cusp commissure to prevent blood from dissecting behind the valve repair during diastole.
86
Color Atlas of Congenital Heart Surgery
right coronary cusp
ceph -•L
R<-
caud
"wind sock" and fenestration
FIGURE 5-23. In another patient, the aortic valve is viewed through a proximal ascending aortotomy. A 1-mm probe is passed into the dilated central portion of the right coronary cusp. There is a discrete fenestration in the end of this wind sock.
fenestration in "wind sock"
ceph R«-
-•L
caud
FIGURE 5-24. The valve cusp is lifted and the fenestration at the end of the wind sock in the right coronary cusp is seen.
5
87
repair stitch of cephalad surface of valve cusp
ceph R<-
Ventricular Septal Defects
-•L
caud
FIGURE 5-25. The cusp is repaired with a single pledgeted mattress suture placed across the fenestration on the upper surface of the right coronary cusp.
5-3. Inlet Ventricular Septal Defect Perimembranous inlet or Type 3 or AV canal type of VSD are located adjacent to the tricuspid valve annulus but posterior and inferior to the region of the membranous septum. A related anomaly is left ventricle to right atrium tunnel or communication. This defect is possible by virtue of the fact that normally the tricuspid valve annulus is located slightly inferior to the mitral valve annulus. With lack of ventricular septal formation in this spot, a direct communication between the left ventricle and right atrium can occur. As an isolated defect, the opening in the right atrium is cephalad or above the tricuspid valve annulus.
anterior leaf of tricuspid valve FIGURE 5-26. This child
has
been placed on cardiopulmonary bypass and the heart arrested with aortic clamping, cardioplegia, and profound local cooling. The right atrium has been opened. There is a direct communication between the left ventricle and right atrium located above the tricuspid valve annulus. This defect is closed with multiple stitches and a Dacron® patch.
tricuspid valve annulus
left ventricle to right atrial communication ceph 4* R<- - • L caud
88
Color Atlas of Congenital Heart Surgery
anterior leaf of tricuspid valve
membranous ventricular septum
VSD
ceph R<-
->L
caud
septal leaf of tricuspid valve
FIGURE 5-27. In another patient the right atrium is opened and the defect is located inferior and posterior to the region of the membranous ventricular septum. Exposure of these defects through the atrium is excellent.
anterior leaf of tricuspid valve
septal leaf of tricuspid valve
ceph R«-
region of His' bundle
->L
caud
FIGURE 5-28. Multiple interrupted mattress sutures with felt pledgets are placed around the rim of the VSD. Posteriorly, these stitches are passed through the base of the septal leaf near the annulus. The His' bundle is located at the posterior inferior margin of the VSD so stitches here are placed superficially. More anterior stitches are placed directly in the muscular septum.
5
Ventricular Septal Defects 89
anterior leaf of tricuspid valve
septal leaf of tricuspid valve
ceph R«-
-•L
caud FIGURE
5-29. Stitches are placed in a Dacron® patch and tied to complete the repair.
5-3-1. Inlet Ventricular Septal Defect with Straddling Tricuspid Valve
anterior leaf of tricuspid valve
septal leaf of tricuspid valve
papillary muscle ceph R<-
-•L
caud
5-30. In another patient, working through a right atriotomy, the VSD is located beneath the large tricuspid valve septal leaf. Tricuspid valve chordae arise from the left ventricle. FIGURE
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Color Atlas of Congenital Heart Surgery
ventricular septal defect
papillary muscles attached in left ventricle
ceph
R<-
-•L
caud
FIGURE 5-31. On careful inspection there are two large anomalous papillary muscles to the tricuspid valve both of which arise from the left ventricular chamber.
detached papillary muscles
ceph R<-
-•L
caud
FIGURE 5-32. Both large papillary muscles are divided at the base. Multiple stitches are placed around the rim of the VSD.
5
Ventricular Septal Defects 91
5-33. A Dacron® patch is stitched over the VSD. The two anomalous papillary muscles will be re-implanted at appropriate sites on the right ventricular surface of the ventricular septum using multiple interrupted simple stitches. FIGURE
Dacron patch
detached papillary muscles
caud
5-4. Muscular Ventricular Septal Defect These defects can occur in any part of the muscular ventricular septum. Preoperative definition of the number of VSDs and the precise location of each by echocardiography or angiography is useful to the surgeon at the time of repair. The best exposure for most muscular defects is through a right atriotomy. An unusual location may warrant a high or an apical right ventriculotomy. In the presence of multiple low VSDs an apical left ventriculotomy may be optimal.
VSD
FIGURE 5-34. Cardiopulmonary
bypass is established in conjunction with aortic cross-clamping, cardioplegia, and profound local cardiac cooling. The right atrium is opened, and the septal tricuspid valve leaflet is retracted. A large muscular VSD is seen beneath this leaflet. There is a cephalad muscular rim of VSD that separates it from the tricuspid valve.
septal leaf of tricuspid valve
>L caud
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Color Atlas of Congenital Heart Surgery
Dacron patch
FIGURE 5-35. Multiple interrupted stitches with Teflon® felt pledgets are placed around the rim of the VSD. The His' bundle is located adjacent to the tricuspid valve annulus beneath the septal leaflet. Stitches in this area are placed superficially to avoid the His' bundle. Remaining stitches are inserted on the right ventricular surface of the septum, passing through approximately 50% of the thickness of the septum. The stitches are placed in a knitted Dacron® patch that is positioned beneath tricuspid leaflet chordae to avoid entrapment of same.
septal leaf of tricuspid valve
tip of clamp
FIGURE 5-36. In this child, exposure is through a right atriotomy. The muscular VSD is located beneath the posterior aspect of the septal tricuspid valve leaflet. For identification, a right-angle clamp is passed through an atrial septal defect (ASD) and the mitral valve, with the tip presenting in the muscular VSD. There is a muscle ridge along the posterior margin of the VSD and beneath the septal leaflet and the His' bundle is located in this region. In some cases, the VSD is in continuity with the tricuspid valve annulus, and the His' bundle is located in the posterior inferior corner of the defect. This muscular VSD will be closed with felted interrupted sutures and a knitted Dacron® patch.
5
Ventricular Septal Defects
93
mid muscular VSD
septal leaf of tricuspid valve
ceph R<-
-•L
caud
FIGURE 5-37. In another child, a large muscular VSD is exposed through a right atriotomy. This defect is in the midmuscular septum and is retracted into the field with a metal clamp that is passed through the septal defect. In some cases trabeculations hide the true rim of a muscular VSD, in which case care must be taken to adequately expose all margins of the VSD. With this exposure stitches can be placed around the rim of the defect being careful to avoid incomplete closure and a residual VSD.
FIGURE 5-38. In
this child, the
precise location of a high muscular VSD was determined intraoperatively. The right ventricle was explored through a right atriotomy, but no VSD was seen initially. With the left ventricular vent clamped, excessive red blood was noted in the right ventricular outflow tract. A high right ventriculotomy was made as shown here. A septal defect was found by passing a probe amongst trabeculations in the left upper part of the infundibulum. If a high VSD cannot be located with the above approach, the ventricular septum is inspected through the proximal ascending aorta while retracting the aortic valve.
region of pulmonic valve
trabeculations covering high muscular VSD
ceph -•L caud
94 Color Atlas of Congenital Heart Surgery
high muscular VSD in left lateral area of outflow tract
FIGURE
5-39. After trabeculations are resected, a large muscular VSD is seen.
region of pulmonic valve
patch
ceph R<-
-•L
caud
5-40. Interrupted felted mattress sutures are passed through the right ventricular surface of the septum, surrounding the VSD. The defect is then closed with stitches placed in a knitted Dacron® patch. FIGURE
5
Ventricular Septal Defects
95
ventricular septal defect
R^
FIGURE 5-41. In this child, it was not possible to adequately expose an apical muscular VSD by working through a right atriotomy. A short apical right ventriculotomy allowed excellent exposure of the defect.
patch
ceph R<-
-*L
caud FIGURE
sutures.
5-42. A Dacron® patch is stitched over the VSD with interrupted felted mattress
96 Color Atlas of Congenital Heart Surgery
5-5. Pulmonary Artery Banding and Band Removal at Subsequent Total Repair In the presence of a large left-to-right shunt, pulmonary artery banding may be indicated to protect the lungs and to avoid development of pulmonary vascular obstructive disease when total repair of intracardiac anomalies is not possible or should be delayed until an older age. Banding can be performed through a left or right thoracotomy or median sternotomy, regardless of the position of the great vessels. Ideally, it is carried out through a fourth interspace lateral thoracotomy on the side ipsilateral to the main pulmonary artery. A SILASTIC®impregnated Teflon® band is passed around the mid main pulmonary artery. If the band is placed more proximally and over the commissures of the pulmonary valve, thickening of valve leaflets may occur after surgery, which may result in development of a dysplastic pulmonary valve. If the band is placed too distally on the main pulmonary artery, it may encroach on and kink one or both pulmonary artery branches. During banding, distal pulmonary arterial pressure is monitored by placing a small plastic catheter through a purse string suture. A second plastic catheter is placed in the ascending aorta for blood sampling and measurement of systemic pressure, although the same can be accomplished with a peripheral arterial catheter inserted before thoracotomy. Ideally, banding should reduce distal main pulmonary artery mean pressure to 25 to 30mmHg or 30% to 50% of mean systemic pressure. In most cases before banding, pulmonary arterial pressure will be equal to systemic pressure. When pulmonary vascular resistance is low before banding, mean pulmonary arterial pressure may be low. In this case, the pulmonary artery is constricted until there is a rise in systemic pressure. The final postband pressures should be similar to those mentioned above for children who initially have pulmonary artery hypertension. If systemic outflow tract obstruction is present, the band has to be placed loosely, and ideal pressure changes may not be accomplished; this avoids ventricular hypertension as the result of double outlet obstruction. To check for this after banding is complete, a needle is passed into the distal main pulmonary artery and then proximal to the band to measure intraventricular pressure. Peripheral arterial oxygen saturation (Fi0 2 = 0.5) should not change with banding. A drop of 5% is acceptable in patients with normally related great vessels. In the presence of d-transposition a drop of 10% is acceptable, but peripheral arterial oxygen saturation should not be reduced below 65% to 70%.
5
Ventricular Septal Defects 97
catheter in ascending aorta catheter in distal main pulmonary artery
constricting band around mid main pulmonary artery
ant caud<-
-•ceph
post
5-43. Through a left fourth intercostal space thoracotomy and a pericardiotomy at the base of the heart, a left-sided main pulmonary artery is exposed. The band around the main pulmonary artery has been progressively tightened with interrupted sutures until the desired pressure changes are seen. It is fixed to the adventitia of the proximal main pulmonary artery with interrupted sutures that are placed on one half to two thirds of the circumference of the main pulmonary artery. The monitoring catheters in the distal pulmonary artery and ascending aorta are in place. FIGURE
5-5-7. Band Removal at the Time of Total Repair
main pulmonary arterotomy
band ends separated ceph R«-
-•L
caud FIGURE 5-44. On cardiopulmonary bypass, band stitches are removed and the ends of the band separated. A longitudinal incision is made in the anterior wall of the main pulmonary artery at the band site.
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Color Atlas of Congenital Heart Surgery
intimal fibrous ridge
ceph R<-
-•L
caud
FIGURE 5-45. After all sutures are removed, the band can be removed from its bed. A posterior fibrous ridge over the band tunnel remains, and this tissue must be excised to prevent residual obstruction.
fibrous ridge incised to open bed of previous band
ceph R<-
->L
caud
FIGURE 5-46. The fibrous ridge is incised transversely on the inner surface of the pulmonary artery to expose the intact adventitia of the lateral and posterior main pulmonary artery.
5
Ventricular Septal Defects
99
fibrous ridge
ceph R<-
-•L
caud FIGURE
5-47. The fibrous ridge is excised also removing loose intimal tissue.
bed of previous band
ceph R<-
-•L
caud
5-48. The smooth adventitia of the posterior main pulmonary artery is intact. If it were not, the intima would be approximated with a continuous suture.
FIGURE
100
Color Atlas of Congenital Heart Surgery
pericardial patch
R^
FIGURE 5-49. An oval pericardial patch of generous size is sutured over the anterior main pulmonary artery.
pericardial patch
ceph R«-
-•L
caud
FIGURE 5-50. With placement of the pericardial patch, the main pulmonary artery is enlarged anteriorly to avoid stenosis of the vessel. The posterior fibrous rim has been removed to avoid obstruction to flow in this area, which can occur if only anterior pericardial patch enlargement is used.
6
Fontan Operation
A Fontan operation is used for patients who have a variety of anomalies with single ventricle including, but not limited to, tricuspid atresia or hypoplastic left heart syndrome. After the repair is complete, the single ventricle is in continuity with the systemic circulation, and the systemic venous return flows to the lungs without passing through a functional ventricle. Historically, the operation was performed by using a variety of right atrial to pulmonary arterial connections. Currently, most centers use the modified Fontan procedure as a single- or a two-stage operation. This technique incorporates division of the superior vena cava and a direct end-to-side anastomosis to the right pulmonary artery. This should provide for unobstructed flow from the superior vena cava into the right and left pulmonary circulation. The tunnel used to direct inferior vena caval flow to the pulmonary arteries as part of this procedure is constructed with an intra-atrial tunnel baffle or with an extracardiac tube graft. The two-stage repair is always used in the presence of risk factors and most centers stage the procedure in all patients. A bidirectional cavo-pulmonary shunt is constructed with or without cardiopulmonary bypass. At a later operation the intra-atrial baffle or extracardiac conduit procedure is performed.
6-1. First-Stage Fontan Procedure Before cardiopulmonary bypass, extensive dissection is performed. The ascending aorta and main pulmonary artery are freed from surrounding tissue to allow division of the proximal main pulmonary artery. The left pulmonary artery is mobilized, and the right pulmonary artery is dissected to facilitate the superior vena cava-right pulmonary arterial anastomosis. The entire superior vena cava is dissected to near the inominate vein, allowing high caval cannulation and lower caval exposure for the proposed anastomosis. The cava is marked with a suture at the site of division of this vessel, which will allow for an anastomosis without tension or redundancy. The azygos vein is usually sacrificed. The inferior vena cava is cannulated at the inferior cava-atrial junction, saving direct caval cannulation for the stage 2 procedure. Usually aortic cross-clamping is not used. Air embolization is prevented by use of a large caliber ventricular vent placed across the systemic atrio-ventricular (AV) valve and systemic hypothermia to depress ventricular function. To facilitate the stage 2 operation, the pericardium is closed primarily or a Gore-Tex® pericardial membrane is left in place.
101
102
Color Atlas of Congenital Heart Surgery
high cannulation of superior vena cava cephalad portion of divided main pulmonary artery aorta
superior vena cava being divided
proximal closed portion of main pulmonary artery
FIGURE 6-1. After cardiopulmonary bypass is established, the main pulmonary artery is divided near the pulmonary valve. The valve leaflets are sutured closed to prevent release into the circulation of any clot that might later form in this blind pouch. The proximal pulmonary artery is then closed with continuous sutures in two layers. The suture line is supported with three interrupted sutures with Teflon® felt pledgets. The superior vena cava is divided at a point determined before cardiopulmonary bypass, when the vessel is naturally distended. If an atrial septectomy is required, this can be performed working through the retracted proximal superior vena cava orifice.
superior vena cava
proximal right pulmonary artery
open distal portion of main pulmonary artery
FIGURE 6-2. After a right pulmonary arteriotomy of appropriate size along the cephalad surface of this vessel, an end-to-side caval-pulmonary artery anastomosis is constructed with a continuous suture posteriorly. Care is taken to avoid twisting the cava before the anastomosis.
6
Font an Operation
103
completed anastomosis
distal portion of main pulmonary artery R^
6-3. The anterior row of the anastomosis is completed with a continuous suture only interrupting it with three or four sutures to allow for anastomatic growth.
FIGURE
occluded anastomosis between main pulmonary artery and superior vena cava
6-4. The open distal portion of the main pulmonary artery is closed primarily. The open lower portion of the superior vena cava is anastomosed circumferentially around the adjacent intact pulmonary artery segment. This establishes tissue continuity that will be useful at the time of the second-stage or completion Fontan operation.
FIGURE
104 Color Atlas of Congenital Heart Surgery
open distal main pulmonary artery pericardial patch lower superior caval segment
ceph R<-
-•L
caud
6-5. In another patient, the open distal main pulmonary artery is anastomosed end-to-end to the open lower superior caval segment. A pericardial patch is inserted in the anastomosis between the two vessels, closing the connection to occlude blood flow. In many patients this helps to maintain patency of the two blind pouches. At the later completion Fontan operation, the blind pouches are opened and the pericardial patch is simply excised. FIGURE
open distal main pulmonary artery
pericardial patch ceph R<-
-•L
caud FIGURE 6-6. The anterior row of this anastomosis is completed with a continuous suture picking up pulmonary artery wall, pericardial patch, and caval wall with each stitch.
6
Fontan Operation
105
distal main pulmonary artery
R^
superior vena cava segment
FIGURE 6-7. The anastomosis is complete and the blind caval and main pulmonary artery pouches, respectively, are in continuity.
superior vena cava
blind pouch of main pulmonary artery
right pulmonary artery caud
FIGURE 6-8. The postoperative angiogram shows a well positioned and functioning bidirectional caval pulmonary shunt. The blind main pulmonary artery pouch is also visualized.
106 Color Atlas of Congenital Heart Surgery 6-7-7. Second-Stage
or Completion
Fontan
Procedure
In preparation for this operation, extensive dissection of cardiac structures is performed. The superior and inferior venae cavae are freed for direct cannulation. The right pulmonary artery is dissected in anticipation of placing a patch over the previous lower superior cava to blind main pulmonary artery anastomosis. It is also useful to free the proximal left and distal right pulmonary arteries for snaring during the latter patch placement. This allows for hemostasis in the field and facilitates this part of the operation. Surgery is performed using cardiopulmonary bypass with moderate hypothermia and left ventricular venting. With hypothermic depression of ventricular function and the use of a large caliber vent to avoid ventricular ejection, aortic cross-clamping is rarely used. 6-7-2. Intra-Atrial
Lateral Tunnel
Repair
6-9. With the child on bypass, a short anterior right atriotomy is made. A rightangle clamp is passed into the blind caval pouch and the tip exposes the area posterior to the sinoatrial (SA) node region.
FIGURE
6
Font an Operation
107
tip of clamp
ceph R«-
->L caud
FIGURE 6-10. A longitudinal incision is made laterally in the blind pouch.
region of SA node
incision ceph R<-
-•L
caud
FIGURE 6-11. This incision is located posterior to the SA node region.
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Color Atlas of Congenital Heart Surgery
ceph R<-
-•L
eustachian valve
caud
FIGURE 6-12. Looking through the atriotomy, an atrial septal defect (ASD) is identified. The eustachian valve is prominent and will be used in the repair.
patch
R^ caud
FIGURE 6-13. A rectangular-shaped patch is cut from a large tubular Gore-Tex® graft.
6
Font an Operation 109
eustachian valve
ceph R<-
-•L
caud
6-14. The lower rim of the patch is stitched to the eustachian valve using a continuous Gore-Tex® suture. The medial margin of the patch is stitched to the atrial septum adjacent to the ASD.
FIGURE
fenestration
FIGURE 6-15. The right margin of the patch is stitched to the lateral wall of the atrium. A 5- to 6-mm fenestration is cut at the mid portion of the patch.
110 Color Atlas of Congenital Heart Surgery
exteriorized purse string stitch
R^
6-16. A large polypropylene stitch is placed as a mattress suture around the rim of the fenestration and then is exteriorized.
FIGURE
exteriorized purse string stitch
stitches at corners of fenestration
6-17. Gore-Tex® sutures placed at the two corners of the fenestration are exteriorized and tied. FIGURE
6
R<
Fontan Operation
111
tourniquet on purse string stitch
FIGURE 6-18. A tourniquet is placed around the exteriorized purse string stitch and pulled tight.
fenestration snared closed
tourniquet
R^
FIGURE 6-19. The fenestration is checked to be sure that it is obliterated by tightening the purse string stitch.
112
Color Atlas of Congenital Heart Surgery
ceph
R<-
-•L
t caud
additional stitch used to open fenestration
FIGURE 6-20. A second smaller polypropylene stitch is placed around the original purse string stitch and also exteriorized. By pulling this second suture, the original purse string stitch can be loosened if necessary after closing all cardiotomies.
sulcus terminalis
R^
FIGURE 6-21. The intra-atrial baffle is completed by attaching the upper rim of the patch to the sulcus terminalis.
6
Fontan Operation 113
Gore-Tex baffle
R<
FIGURE 6-22. The completed intracardiac baffle is in good position and will direct all inferior caval flow to the blind superior caval pouch.
main pulmonary artery pouch
superior vena cava pouch ceph R<-
-•L
caud
6-23. To establish continuity between the heart and the pulmonary arteries, a longitudinal incision is made laterally in the blind main pulmonary artery pouch that is contiguous with the incision in the superior vena caval pouch. In this patient, the two vessels had previously been approximated by direct anastomosis. The result is a thick layer of scar tissue that separates the endothelial lining. This lining will be approximated with multiple fine interrupted polypropylene sutures. FIGURE
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Color Atlas of Congenital Heart Surgery
main pulmonary artery pouch
pericardial patch
ceph R<-
-•L
caud
FIGURE 6-24. In another patient, the blind main pulmonary artery pouch is opened and the previously placed pericardial patch is seen occluding the opening with the intracardiac structures.
area of exised pericardial patch
FIGURE 6-25. This patch is excised to establish continuity with the blind superior caval pouch.
6
Fontan Operation
115
lateral Gore-Tex patch
ceph R«-
-•L
caud FIGURE 6-26. A Gore-Tex® patch is stitched over this area to connect the intra-atrial lateral tunnel to the pulmonary artery. The superior margin of this Gore-Tex® patch extends onto the inferior rim of the right pulmonary artery. This patch is placed on the lateral aspect of the cardiac structures for optimal blood flow dynamics and to avoid placing stitches in the SA node.
LA line
RA line R<-
6-27. A snare around the exteriorized fenestration suture is positioned along the atrium. If hemodynamics allow, the fenestration stitch can be tied during this surgery but after separation from cardiopulmonary bypass. If the fenestration is to be left patent, the tourniquet is stitched to the area behind the right rectus muscle. Some months or years later, cardiac catheterization is performed and the fenestration is test occluded. If it can be closed at that procedure, a small incision is made below the xiphoid and the end of the tourniquet exposed. The tourniquet is pulled tight and fixed in order to occlude the fenestration. FIGURE
116 Color Atlas of Congenital Heart Surgery
left pulmonay artery
intra-atrial lateral tunnel
ceph R<-
-•L
caud
6-28. A postoperative angiogram shows the lateral tunnel with flow primarily to the left pulmonary artery. FIGURE
fenestration
ceph A R«- -• L
,,?-
caud FIGURE
6-29. On the lateral view of this angiogram, the patent fenestration is visualized.
6
Fontan Operation 117
6-1-3. Extracardiac Conduit Repair The completion Fontan procedure can be accomplished by placement of an extracardiac conduit. Some surgeons prefer this technique because they can safely perform it, avoiding a significant risk of air embolization without aortic cross-clamping. Utilizing techniques I have already described (moderate systemic hypothermia and the placement of a large caliber systemic ventricular vent), I accomplish the same safety without aortic cross-clamping utilizing an intra-atrial baffle as well as the alternative extracardiac conduit. There is no significant advantage of one technique over the other; although some claim fewer postoperative atrial arrhythmias with the latter technique as a result of avoidance of damage to the SA node.
right atrium
FIGURE 6-30. The heart is widely
dissected of adhesions and there is ample space between the right atrium and pericardium for placement of the conduit.
FIGURE 6-31. The heart is lifted
to expose a long inferior caval segment.
118
Color Atlas of Congenital Heart Surgery
atriotomy
inferior vena cava
R +
FIGURE 6-32. To place the patient on cardiopulmonary bypass, the inferior vena cava is cannulated directly. The cava with an attached rim of right atrial wall is excised.
right atrium closed R^
FIGURE 6-33. The atriotomy is closed primarily.
6
Fontan Operation
119
FIGURE 6-34. A large Gore-Tex® tubular conduit is stitched end-to-end to the inferior vena cava.
opening in pulmonary artery branches
ceph R<-
-•L
caud FIGURE
6-35. A generous opening is made in the pulmonary artery branches.
120 Color Atlas of Congenital Heart Surgery
posterior anastomosis
ceph A
R<-
-•L
caud
6-36. The conduit is stretched to conform with the right heart border to avoid redundancy when the heart is filled after completing the surgery. The cephalad end of the graft is stitched to the pulmonary arteries with a continuous suture. FIGURE
anastomosis
R^
FIGURE
6-37. The completed anastomosis with the pulmonary arteries is seen.
6
Fontan Operation
121
fenestration
ceph R*-
-•L
caud
6-38. A 5- to 6-mm fenestration is created in the medial aspect of the conduit using an aortic punch. FIGURE
fenestration
ceph R<-
-•L
caud FIGURE
6-39. The fenestration is adjacent to the mid part of the right atrium.
Color Atlas of Congenital Heart Surgery
right atriotomy
fenestration
ceph R<-
-•L
caud
FIGURE 6-40. A small atriotomy is made and the conduit fenestration is stitched to this opening with a continuous polypropylene suture. After completing the posterior row of this anastomosis, a fenestration snare stitch will be placed.
purse string stitch
R^
FIGURE 6-41. A large polypropylene continuous mattress purse string suture is placed posteriorly and will be continued along the anterior margin of the atriotomy.
6
Fontan Operation
123
FIGURE 6-42. The anterior anastomosis is completed with a continuous suture. The purse string snare suture is exteriorized and a tourniquet placed
fenestration
purse string stitch
6-1-4. Intra-Atrial
Conduit
In a few patients with complex and unusual anatomy, neither an intra-atrial lateral tunnel nor an extracardiac conduit repair can be safely accomplished. In these cases, an intra-atrial conduit may be useful. Working within the atrial chambers, a tubular conduit is stitched to the internal orifice of the inferior vena cava. It then passes cephalad to an atriotomy on the superior surface of the atrial chambers, through which it is anastomosed to the pulmonary arteries.
intra-atrial tubular conduit
fenestration
mattress stitch in fenestration
FIGURE 6-43. The
conduit
has
been placed and a fenestration created with a flap opening in the lateral aspect of the conduit. A mattress suture is placed in the rim of the fenestration and through the flap, after which it is exteriorized through the lateral atrial wall.
124 Color Atlas of Congenital Heart Surgery 6-44. Test closure of the fenestration is shown by pulling tight a snare around the exteriorized stitch. FIGURE
test closure of fenestration
>post
6-2. Obstruction of Bulboventricular Foramen In the presence of single ventricle with d- or 1-transposition of the great arteries and obstruction of a bulboventricular foramen that separates the ventricle and aorta, ventricular hypertension occurs that must be relieved to preserve ventricular function. Two techniques are used. In the first, the proximal-divided main pulmonary is attached to the ascending aorta (Damus procedure), creating a double outlet ventricle to bypass the obstruction. With the second technique, subaortic obstruction is resected and/or septal tissue is removed to enlarge the bulboventricular foramen.
ascending aorta
restrictive bulboventricular foramen
ceph ant<-
-•post
caud
6-45. This lateral view of a single ventricular injection shows the restrictive bulboventricular foramen that separates the ventricle from the subaortic chamber.
FIGURE
6
Fontan Operation 125
6-2-1. Damus Procedure
ascending aorta
pulmonary artery band
6-46. The great vessels are transposed with an anterior ascending aorta and a posterior main pulmonary artery. The pulmonary artery band constricts the mid main pulmonary artery. FIGURE
ascending aorta
main pulmonary artery divided
6-47. Cardiopulmonary bypass is established, and the aorta is clamped. Profound local cardiac cooling and cardioplegia are used. The main pulmonary artery and its branches are dissected; the vessel is divided at the pulmonary artery band, and the band is removed. FIGURE
126
Color Atlas of Congenital Heart Surgery
ascending aortotomy
longitudinal incision
proximal main pulmonary artery
R^
FIGURE 6-48. A longitudinal incision is made in the proximal main pulmonary artery segment to near the annulus and adjacent to the ascending aorta. The ascending aorta is opened proximally and then to a point more distal than the pulmonary artery segment.
ascending aorta Gore-Tex hood
proximal main pulmonary artery
R^ caud
FIGURE 6-49. A side-to-side anastomosis is constructed between the posterior margin of the proximal aortotomy and the proximal pulmonary arterotomy. A triangular shaped Gore-Tex® hood is stitched over the distal end of the main pulmonary artery segment and then attached to the distal aortotomy.
6
Fontan Operation
127
6-50. The anterior rim of the aortotomy is stitched to the anterior rim of the pulmonary artery to complete the repair. FIGURE
6-2-2. Enlargement Septal Tissue
of Bulboventricular
Foramen
by Resection
of
This is an alternative technique to relieve ventricular outlet obstruction in patients with a single ventricle. A complication of this procedure is complete heart block caused by injury to conduction tissue during septal resection. In patients with d-looping, the His' bundle is located in septal tissue along the right and caudal margin of the bulboventricular foramen. In patients with 1-looping, the His' bundle is normally found along the cephalad and left margin of the bulboventricular foramen. Resection is carried out in the obstructing septum on the border of the foramen remote from conductive tissue. Procedures are performed using cardiopulmonary bypass. In small infants, adequate exposure may be gained working through a small incision in the ventricular wall of the subaortic outflow chamber. The ventriculotomy is remote from the systemic ventricle and should not adversely affect ventricular performance. In older children, resection of septal tissue can be easily performed through a proximal ascending aortotomy while retracting the aortic valve or a right atriotomy while retracting the AV valve. It is useful to pass a large nerve hook or a rightangle clamp into the bulboventricular foramen for retraction, facilitating exposure of the area to be resected.
128 Color Atlas of Congenital Heart Surgery
ascending aorta
restrictive bulboventricular foramen
ventriculotomy
6-51. After placing the infant cardiopulmonary bypass with aortic cross-clamping and cardioplegic arrest, a short incision is made in the anterior wall of the subaortic chamber. In some cases it may be difficult to define the best location for the ventriculotomy. In this case, an aortotomy is made and a small clamp is passed into the subaortic chamber, pressing it against the anterior wall so the precise location for the ventriculotomy can be determined. The restrictive bulboventricular foramen is seen. Conductive tissue is near the right border of the bulboventricular foramen, and the area of resection is identified to the infant's left. FIGURE
enlarged bulboventricular foramen
caud
6-52. The enlarged bulboventricular foramen is seen after resection of leftward septal tissue. The ventriculotomy is usually closed primarily. If the chamber is restrictive, muscle can be resected anteriorly in the subaortic area and the chamber can be enlarged by closing the ventriculotomy with an augmenting patch. FIGURE
7
Pulmonary Stenosis
Pulmonary stenosis can occur at one or more sites from the proximal right ventricular outflow tract to the peripheral pulmonary arteries. Valvar obstruction and peripheral pulmonary stenosis are usually treated by closed balloon angioplasty; however, surgical repair is performed in many patients, especially those in whom operation is carried out for associated anomalies. Narrowing of the main pulmonary artery and/or the proximal right and left branches is most commonly associated with tetralogy of Fallot and will be discussed in Chapter 9.
7-1. Valvular Pulmonary Stenosis Pulmonary valvotomy is performed using cardiopulmonary bypass, including left ventricular venting and aortic cross-clamping; although in unusual circumstances, as in very small neonates, it is most expeditious to avoid the use of a left ventricular vent. In these cases, care must be taken not to allow air from the opened right heart to enter the left heart through an intracardiac communication as a patent foramen ovale. Aortic clamping may also be optional but its use helps in reducing blood flow from the coronary sinus into the right ventricle and the operative field. An alternative method to reduce blood flow into the operative field when not using aortic cross-clamping is the use of a single right atrial cannula that the drains coronary sinus as well as caval return. In this instance, the tricuspid valve must remain competent during bypass or otherwise excessive air will enter the venous return line to the pump.
129
130 Color Atlas of Congenital Heart Surgery
leaflets
ceph R<-
-•L
caud
7-1. After the cardiopulmonary bypass is established, the proximal main pulmonary artery is opened transversely or longitudinally. Three commissures of the trileafed pulmonary valve are stenotic, and there is mild thickening of the leaflets. FIGURE
incised commissures
ceph R<-
-•L
caud FIGURE
7-2. All commissures are incised to the annulus.
7
Pulmonary Stenosis 131
main pulmonary artery
valve
R^
FIGURE 7-3. In this patient, after cardiopulmonary bypass is established, a proximal main pulmonary arteriotomy is made. There are three leaflets with stenotic commissures, and the valve is dysplastic. All leaflets are fibrous with limited mobility. Incising the stenotic commissures alone will not relieve the obstruction, because the leaflets are bulky and will not adequately move out of the stream of blood during systole. Part or all of the leaflets will be resected to avoid residual obstruction.
7-2. Infundibular Stenosis and Double Chamber Right Ventricle Repair is performed with cardiopulmonary bypass, aortic clamping, cardioplegic arrest, and profound local cardiac cooling.
septal band
os infundibulum
anterior wall extension of obstructing parietal band parietal band
>L
7-4. After the child is placed on cardiopulmonary bypass, a high right ventriculotomy is made. Obstruction at the os infundibulum is identified. There are large muscle ridges in the region of the parietal and septal bands extending to the anterior wall of the right ventricle. Fibrous tissue surrounds the os. FIGURE
132 Color Atlas of Congenital Heart Surgery
os infundibulum
tricuspid valve papillary muscle
caud
7-5. Obstructing muscle and fibrous tissue has been resected, and the tricuspid apparatus below this area is seen.
FIGURE
parietal band
moderator band
7-6. Double-chambered right ventricle is a form of infundibular pulmonary stenosis caused by muscle obstruction that is primarily in the area of the moderator band, as seen on this right ventriculogram.
FIGURE
7
Pulmonary Stenosis 133
parietal band
moderator band
tricuspid valve leaflets
ceph R<-
-•L
caud
7-7. In this patient with double chamber right ventricle the exposure is through a right atriotomy while the tricuspid valve is retracted. The hypertrophied moderator band forms the major blockage, although an obstructing parietal band is also seen. FIGURE
parietal band
VSD
ceph R<«-
->L
caud
7-8. An associated membranous ventricular septal defect (VSD) is located medial to the parietal band. FIGURE
134 Color Atlas of Congenital Heart Surgery
os infundibulum
VSD patch
ceph R^-
-•L
caud
7-9. The parietal and moderator bands are resected, and the os infundibulum is now open. The VSD is closed with multiple interrupted mattress sutures and a Dacron® patch. FIGURE
o
Pulmonary Atresia and Intact Ventricular Septum
Infants born with this anomaly have inadequate pulmonary blood flow (caused by pulmonary valve atresia) and severe right ventricular hypertension, unless tricuspid insufficiency allows decompression of the ventricle. Initial resuscitation includes maintenance of ductus arteriosus patency with prostaglandin Ei. Right ventricular size may vary from severe hypoplasia to a near normal size, depending on when in fetal development the pulmonary valve became atretic. If it was late in cardiac development, the chance is greater that the right ventricle will be larger at birth. Generally, hypoplasia of the tricuspid valve is proportional to the size of the right ventricular inlet. Ideal surgical palliation is aimed at increasing pulmonary blood flow and relieving right ventricular hypertension. If the tricuspid valve is of satisfactory size and the right ventricle is at least 30% to 40% of normal in size, a valvectomy alone may result in successful palliation. If the tricuspid valve is restrictive and/or the right ventricle is severely hypoplastic, an isolated valvectomy may not provide palliation and a systemic to pulmonary artery shunt will be necessary also. In this case, a nonrestrictive atrial septal defect (ASD) must be naturally present or created by balloon septostomy. In many centers, balloon valvuloplasty is performed in the cardiac catheterization laboratory after laser perforation of the valve membrane. If successful, only a surgical shunt is needed when smaller right ventricle (RV) size or decreased compliance results in inadequate pulmonary blood flow. After initial palliation, adequate right ventricular growth may allow a later two-ventricle repair. If the right ventricle remains severely hypoplastic, patients are candidates for a one-ventricle repair (Fontan procedure). In infants with inadequate pulmonary blood flow after pulmonary valvectomy alone, long-term ductus patency may mitigate the need for a surgical shunt. This may be accomplished by Formalin infiltration of the patent ductus. Right ventricular compliance usually improves with time, and ductal patency for a period of days or weeks following valvectomy may provide satisfactory pulmonary blood flow until the right ventricle recovers. Otherwise a systemic to pulmonary artery shunt will be needed.
135
136 Color Atlas of Congenital Heart Surgery PA view
Lateral View
right ventricular inlet
FIGURE 8-1. Right ventriculogram in an infant with severe right ventricular hypoplasia. The outlet portion of the ventricle is underdeveloped, and the tricuspid valve annulus is hypoplastic. This baby is not a candidate for valvectomy alone.
right ventricular outlet
right ventricular inlet
8-2. Lateral view of right ventriculogram in an infant with a near normal-size right ventricle. The tricuspid valve annulus and the outlet portion of the ventricle are well developed. A valvectomy is appropriate for this baby. FIGURE
8
Pulmonary Atresia and Intact Ventricular Septum 137
central area of atretic valve
rudimentary pulmonary valve leaflet
ceph ->L
R<-
caud
8-3. This heart is prepared for a pulmonic valvectomy. On cardiopulmonary bypass, a side-biting clamp is placed on the proximal main pulmonary artery. An arteriotomy is made, and stay sutures are inserted. A snare is pulled tight around the distal main pulmonary artery, and the clamp is removed. There is hemostasis, and the atretic valve membrane is visualized. The rudimentary commissures and leaflets are seen encircling the atretic central portion of the membrane. The atretic pulmonary valve membrane is now excised. More commonly, the pulmonary valve annulus is restrictive and a transannular outflow tract patch is needed. FIGURE
formalin infiltrated patent ductus
ceph R««-
-•L
caud
8-4. In this infant, Formalin infiltration of the patent ductus is performed. Methylene blue is mixed with 4% Formalin so that the material is easily seen when injected in tissues. A #30 needle is used with a 1-cc tuberculin syringe for precise injection in the wall of the ductus. When they are compared, a glass syringe is better than a plastic syringe, because the glass plunger slides more easily. The ductus wall is infiltrated over two thirds of the circumference and much of the length of the ductal structure. FIGURE
9
Tetralogy of Fallot
Primary total repair is the operation of choice for patients with tetralogy of Fallot. Initial palliation with a systemic-to-pulmonary artery shunt or right ventricular outflow tract reconstruction with later corrective surgery may be indicated in the presence of hypoplastic pulmonary artery branches or a left anterior descending coronary artery that arises from the right coronary artery and traverses the right ventricular outflow tract. Shunts are also useful in the management of small children with associated cardiac anomalies, which may be more safely repaired at an older age [i.e., tetralogy of Fallot with complete atrio-ventricular (AV) canal].
9-1. Palliative Shunts and Outflow Tract Reconstruction 9-1-1. Classic Blalock-Taussig Shunt
(Subclavian Artery to Pulmonary
Artery)
This operation is rarely performed because most surgeons prefer the more predictable modified Blalock shunt. It may still be useful in cases when the shunt is the end point of treatment because the subclavian artery trunk will grow with the patient. This procedure is ideally performed by working through a lateral fourth intercostal space thoracotomy on the side ipsilateral to the innominate artery. Postoperative congestive heart failure rarely occurs and the shunt may be simply closed by ligating the subclavian artery at the time of later total repair.
138
9
Tetralogy of Fallot
139
left subclavian artery
vagus nerve
ant caud<-
-•ceph
left pulmonary artery
post
FIGURE 9-1. Working through a left thoracotomy in the presence of a right aortic arch and left innominate artery, the mediastinal pleura is incised from the apex of the chest to the hilum. The left pulmonary artery is dissected extrapericardial from its origin to the first branches. The left subclavian artery is dissected from its origin to the rib margin at the thoracic inlet. The innominate artery and proximal right carotid artery are freed from surrounding tissue to allow mobility of the subclavian trunk when it is shifted caudad for the anastomosis.
innominate artery left carotid artery
subclavian artery area of distal stump subclavian artery
ant caud^-
->ceph
vagus nerve left pulmonary artery
post
FIGURE 9-2. All branches of the subclavian artery and the distal main vessel near the rib margin are ligated and divided. The subclavian artery is shifted from the apex of the chest beneath the vagus nerve and moved caudad for an end-to-side connection with the mid left pulmonary artery. The anastomosis is performed with a 6.0 or 7.0 continuous monofilament suture posteriorly and anteriorly, interrupting the latter in a few places to allow for anastomotic growth. Hemostasis during the anastomosis is accomplished with the clamps on the base of the subclavian artery and on the proximal pulmonary artery. Distal pulmonary artery branches are snared. The vagus nerve is seen crossing the subclavian artery in the posterior medistinum.
140 Color Atlas of Congenital Heart Surgery 9-1-2. Modified Artery) Shunt
Blalock-Taussig
(Subclavian
Artery to
Pulmonary
This shunt is constructed by interposing a synthetic tubular graft between the subclavian and pulmonary arteries. It can be performed in either chest, irrespective of the arch anatomy and the location of the innominate artery. Expanded polytetrafluoroethylene (PTFE) grafts (Gore-Tex® or Impra) are used. A 4-mm graft may be used for infants up to 2 months of age, a 5-mm graft from 3 to 24 months, and a 6-mm graft for children over 2 years. In general, smaller grafts are used during Stage I palliation for hypoplastic left heart syndrome (Chapter 18, Section 18-7). Advantages of this operation over the classic Blalock-Taussig procedure include conservation of the subclavian artery. A graft larger than the subclavian artery may be used, because shunt flow is generally regulated by the size of the subclavian artery; this may result in prolonged shunt patency. Patients are heparinized (lOOU/kg) intraoperatively after hemostasis, following shunt construction, but this is not continued postoperatively.
9-3. A right thoracotomy is performed in this child with a right-sided innominate artery. A pleural incision is made from the apex of the chest across the mediastinum to the right pulmonary artery. The proximal right subclavian artery is dissected medial to the vagus nerve. The azygos vein is divided and the superior vena cava is retracted anteriorly. Alternatively, the azygos vein may be left intact and retracted. The paratracheal lymph nodes are removed, and the right pulmonary artery is dissected from its origin to the first branches. FIGURE
9
Tetralogy of Fallot 141
right subclavian artery
right pulmonary artery
graft
ant A ceplv -•caud post FIGURE 9-4. A side-biting clamp is placed on the base of the right subclavian artery, working medial to the vagus nerve. A graft to artery, end-to-side anastomosis is performed with a continuous suture. The proximal right pulmonary artery is clamped proximally, and the distal vessel is snared. An end-to-side graft to pulmonary artery anastomosis is performed with a continuous suture. Accuracy must be exercised in estimating the length of the graft; if too long, the graft can kink at lower anastomosis; if too short, the subclavian or pulmonary artery may be distorted, compromising shunt flow.
left pulmonary artery
left subclavian artery ant A caud<- ->ceph post
9-5. In this child, a left thoracotomy is performed in the presence of a right-sided innominate artery. The proximal left subclavian artery is dissected near its origin at the aortic arch opening the pleura only in this area. The left pulmonary artery is dissected from its origin in the first branch. FIGURE
142 Color Atlas of Congenital Heart Surgery
left pulmonary artery
ant caud<-
-•ceph
post
9-6. The left pulmonary artery is snared proximally and distally, and an arteriotomy is made. A continuous suture is placed between the posterior wall of the artery and the posterior part of a tubular Gore-Tex® graft. A continuous suture will be used to construct the anterior part of the anastomosis. FIGURE
left subclavian artery
left pulmonary artery ant A caud^- -^ceph post
9-7. The tubular graft is tailored and an end-to-side anastomosis is performed to the proximal left subclavian artery, using a side-biting clamp on the latter vessel. FIGURE
9
Tetralogy of Fallot 143
9-7-5. Central Shunt (Ascending Aorta to Main Pulmonary
Artery)
An advantage of this shunt is long-term patency, even in small infants with small pulmonary arteries. The anastomosis grows with the patient, providing long-term palliation and blood usually flows equally to both right and left pulmonary arteries. It is simple to close at the time of later corrective surgery by direct suture working through the main pulmonary artery. When pulmonary arteries are miniscule, the proximal main pulmonary artery can be divided for an end-to-side anastomosis between the distal cut end and the ascending aorta. The operation is usually performed by working through a median sternotomy, although it can be carried out through a lateral thoracotomy on the side ipsilateral to the main pulmonary artery. It may be useful in infants with pulmonary atresia and intact ventricular septum when a shunt is needed after pulmonic valvectomy.
main pulmonary artery
FIGURE 9-8. A side-biting clamp
is placed on the left posteriorlateral ascending aorta, and an elliptical aortotomy is made. A small opening is made in the adjacent main pulmonary artery, with snares around this vessel proximally and distally.
FIGURE 9-9. The posterior ana-
stomosis is constructed with a continuous suture of polypropylene.
ascending aorta
144
Color Atlas of Congenital Heart Surgery FIGURE 9-10. The anterior
anastomosis is constructed with interrupted sutures.
anastomosis
main pulmonary artery
ascending aorta
>L caud
9-1-4. Reconstruction of Right Ventricular Outflow Tract When pulmonary arteries are severely hypoplastic and total repair is not possible, palliation may be achieved with reconstruction of the right ventricular outflow tract without closing the ventricular septal defect. Over time, if the pulmonary arteries grow, a significant left-to-right intracardiac shunt will develop, and the ventricular septal defect should be closed.
main pulmonary artery pulmonary valve
ventriculotomy
FIGURE 9-11. The infant is placed on
cardiopulmonary bypass. Snares for hemostasis are pulled tight around the proximal right and left pulmonary arteries. The right ventricular outflow tract is incised from the os infundibulum across the hypoplastic pulmonary valve annulus and main pulmonary artery.
9
Tetralogy of Fallot
145
patch
ceph R<-
-•L
caud
9-12. An oval PTFE patch is stitched over the outflow tract, starting distally on the main pulmonary artery. Alternatively, a patch of homograft pulmonary artery wall is used. With the latter, hemostasis is improved with less bleeding from needle holes. FIGURE
patch
ceph R^-
-•L
caud
9-13. The repair is complete with stitching of the patch over the right ventriculotomy. FIGURE
146 Color Atlas of Congenital Heart Surgery
9-2. Total Repair of Simple Tetralogy of Fallot - Transventricular Surgery is carried out with cardiopulmonary bypass, moderate hypothermia, aortic clamping with cardioplegia, and profound local cardiac cooling. Repair of pulmonary stenosis and the ventricular septal defect (VSD) are performed through a short high right ventriculotomy in the infundibulum, which is positioned to avoid division of coronary branches. If the pulmonary valve annulus is restrictive and a transannular patch is anticipated, a longitudinal or oblique ventriculotomy is made that is continuous with the transannular incision.
muscular infundibular floor parietal band septal band
ceph R<-
-•L
caud
9-14. A high right ventriculotomy is made over the infundibular chamber. The os infundibulum is small, and obstruction is due to prominent parietal and septal bands and a muscular infundibular floor. FIGURE
parietal band
ceph R+-
-•L
caud
9-15. A right-angle clamp is passed among trabeculations and beneath the parietal band; the band is lifted for improved exposure. FIGURE
9
Tetralogy of Fallot 147
parietal band
R<
FIGURE
9-16. The parietal band is excised by sharp dissection.
septal band
FIGURE
9-17. The septal band is probed and will be excised.
148 Color Atlas of Congenital Heart Surgery
muscular infundibular floor
ventricular septal defect
ceph 4 R^-•L caud
9-18. Much of the muscular infundibular floor is excised, and the high VSD is now exposed. FIGURE
VSD rim near His' bundle
anterior leaf of tricuspid valve
ceph R<-
septal leaf of tricuspid valve
-•L
caud FIGURE 9-19. Retracting sutures are placed in the septal and anterior leafs of the tricuspid valve. The posterior inferior rim of the VSD adjacent to the His' bundle is exposed.
9
Tetralogy of Fallot 149
remnant of membranous septum papillary muscle of the conus
FIGURE 9-20. The posterior inferior rim of the VSD is probed with a right-angle clamp. A remnant of the membranous septum is present in this child and will be incorporated in the corner VSD closure stitch, which is adjacent to the His7 bundle. The papillary muscle of the conus is adjacent to the mid part of the inferior rim of the VSD.
corner stitch
ceph ->L caud
9-21. The first closure stitch for the VSD repair is placed in the remnant of membranous septum at the posterior inferior corner. If this tissue is not present, the corner stitch is placed superficially in the rim of the VSD. Interrupted mattress sutures with Teflon® felt pledgets are inserted along the inferior border of the VSD, placing each deeper in the septum on the right ventricular surface as one works more anteriorly and away from the His' bundle region. Stitches are placed similarly along the anterior rim of the VSD, incorporating approximately 50% of the thickness of the septum. Additional stitches are placed in the base of the anterior tricuspid valve leaflet, along the posterior rim of the VSD, and near the tricuspid annulus. FIGURE
150 Color Atlas of Congenital Heart Surgery
probe
Dacron patch
ceph R<-
-•L
caud
9-22. A knitted Dacron® patch is cut which is slightly larger than the VSD and conforms to its shape. Sutures along three sides of the VSD are placed in the patch and tied. A 1-mm probe is used to gently probe the rim of the VSD from the left ventricular surface to search for residual openings beneath the patch; if these are found, additional repair sutures are placed. FIGURE
VSD patch
R^
FIGURE 9-23. The repair is completed by passing nonfelted mattress sutures through the patch and then through the remaining muscular floor of the infundibulum near the adjacent aortic valve annulus. These stitches are then passed through small felts and tied. The patch is again probed on the right ventricular surface with a 1-mm probe to search for residual openings.
9 9-2-1,
Total Repair of Tetralogy of Fallot -
Tetralogy of Fallot 151
Transatrial
I was originally interested in this technique of tetralogy repair for use in patients with an anomalous left anterior descending coronary artery that arose from the right coronary system and traversed the infundibular surface: however it is now easy to recognize there are many advantages of this exposure for use in all tetralogy repairs. The VSD is easily seen which expedites repair. Injury to the right ventricular outflow tract is less and the surgeon can more accurately gauge the size of the infundibulum to define obstructing muscle bundles while inspecting it from above. Even when a transannular patch is required, the ventriculotomy can be shorter and avoid injury to the anomalous coronary vessel. I now prefer this approach for repair of all patients with tetralogy of Fallot regardless of patient size and it is used in over 95% of repairs.
main pulmonary artery
pulmonary valve annulus
9-24. In this patient, the external appearance of the size of the pulmonary valve area indicated the annulus is normal; however, the main pulmonary artery is hypoplastic. FIGURE
muscle roof and parietal band
muscle floor
9-25. After establishing bypass and cardioplegia arrest, the right atrium is open. The tricuspid valve is retracted and the obstructing muscle bundles in the floor and roof as well as the parietal band region are exposed. FIGURE
anterior tricuspid valve leaf
152 Color Atlas of Congenital Heart Surgery
ventricular septal defect
ceph R<-
->L
caud FIGURE
9-26. Obstructing muscle bands are grasped and excised.
ventricular septal defect
ceph R<-
->L
caud
FIGURE 9-27. The VSD is in the perimembranous position.
9
Tetralogy of Fallot 153
anterior leaf
web of septal tissue in posterior inferior corner septal leaf
ceph R<-
-•L
caud
FIGURE 9-28. The web of septal tissue in the posterior inferior corner of the VSD is exposed. This will be used in repair of the defect in order to avoid placing deep stitches in this area which might damage the His' bundle.
anterior leaf patch
R<*
9-29. Multiple stitches are placed in the rim of the VSD and these are attached to a Dacron® patch. Along the posterior rim of the VSD, stitches are passed through the base of the anterior tricuspid valve leaflet. FIGURE
154 Color Atlas of Congenital Heart Surgery
patch
ceph R+-
-•L
caud FIGURE
9-30. All stitches are tied to secure the Dacron® patch.
pulmonary valve
ceph R <
> L
caud
9-31. An incision is made in the main pulmonary artery from distally to the valve annulus proximally. Pulmonary valve leaflets are thickened and there is commissural stenosis.
FIGURE
9
Tetralogy of Fallot 155
valve leaf
annulus
ceph R<-
-•L
caud
FIGURE 9-32. A large pulmonary valve leaflet is exposed for a commissurotomy.
commissure
R<-
FIGURE 9-33. The commissurotomy is complete.
156 Color Atlas of Congenital Heart Surgery
septal band and floor muscle
ceph R<-
-•L
caud
9-34. Through this exposure, additional muscle in the infundibulum is identified and resected. FIGURE
infundibulum
ceph R<-
-•L
t caud FIGURE
9-35. A widely patent infundibulum is seen.
9
Tetralogy of Fallot 157
9-36. The outflow tract is viewed again through the tricuspid valve as afinalcheck for obstructing muscle bundles. The main pulmonary artery is then reconstructed with a patch and the atriotomy closed to complete the repair. FIGURE
9-3. Repair of Hypoplastic Right Ventricular Outflow Tract Although there are advantages and disadvantages of outflow tract patching across the pulmonary valve annulus, adequate relief of outflow tract blockage is an important component of total repair operations for tetralogy of Fallot. A competent pulmonary valve with minimal or no residual outflow tract blockage is ideal. Significant residual pulmonary stenosis results in increased morbidity and mortality, so that a transanular patch is indicated when the valve annulus is severely restrictive. Homograft valve placement is not performed initially and is reserved for placement later in the subset of patients who do not tolerate pulmonary regurgitation. A pericardial patch may be used for outflow tract augmentation if postrepair pulmonary artery pressure is expected to be low. It is easy to handle, and it provides better hemostasis as compared with synthetic material. If postrepair pulmonary artery pressure is not low, a synthetic outflow tract patch is used to avoid late development of a patch aneurysm. A homograft pulmonary artery wall patch is also used in many patients because it is hemostatic and does not result in late aneurysm formation. This patch may incorporate a segment of a homograft pulmonary valve, which may prevent postoperative pulmonary insufficiency in the early postoperative period.
158 Color Atlas of Congenital Heart Surgery
pericardial patch
ceph R<-
-•L
caud FIGURE 9-37. The high longitudinal ventriculotomy incision is extended across the hypoplastic pulmonary valve annulus and main pulmonary artery. An autologous pericardial patch is stitched to the outflow tract incision, starting the continuous suture on the distal main pulmonary artery.
pulmonary valve annulus
inner surface of pericardial patch
R^
9-38. The smooth inner surface of the patch is placed within the circulation. The hypoplastic pulmonary valve annulus is seen. FIGURE
9
Tetralogy of Fallot 159
patch
ceph R<-
-•L
caud
9-39. Patch repair augmentation of the outflow tract is complete. The size of the patch is large enough to avoid residual blockage in the outflow tract but is not excessive; this may help avoid later development of a patch aneurysm. FIGURE
Dacron patch
ceph R«-
->L
caud
9-40. In the presence of right heart hypertension, the chance of late development of a pericardial outflow tract aneurysm can be reduced further by attaching a synthetic patch to the surface of the previously placed pericardial patch. This synthetic patch is cut from a tubular Dacron® graft, which incites a fibrous tissue reaction, resulting in a thick protective outer surface over the pericardial patch. FIGURE
160 Color Atlas of Congenital Heart Surgery
patch on left pulmonary artery
ceph R<-
-•L
caud
9-41. In another patient, there is hypoplasia of the annulus and stenosis of the proximal right and left pulmonary arteries. A Gore-Tex® patch is used here for extensive outflow tract reconstruction to avoid late aneurysm formation. The upper right ventricle, the main pulmonary artery, and the right and left pulmonary arteries are reconstructed with separate patches, because the use of a single large patch can be associated with twisting of the Gore-Tex® near the pulmonary artery branches, resulting in obstruction to flow. FIGURE
pulmonary valve annulus
ceph R^-
-•L
caud FIGURE 9-42. When the pulmonary valve annulus is adequate and the infundibulum is hypoplastic after muscle is resected, the annulus can be spared and the proximal outflow tract is enlarged with a Gore-Tex® patch stitched to the ventriculotomy.
9
Tetralogy of Fallot 161
homograft patch ceph R<-
-•L
caud
9-43. In another patient, outflow tract reconstruction is performed using a homograft pulmonary wall patch. FIGURE
homograft patch
ceph R«-
-•L •
caud
9-44. In this patient, outflow tract reconstruction was performed using a segment of pulmonary artery wall homograft, including a valve cusp.
FIGURE
162
Color Atlas of Congenital Heart Surgery
homograft pulmonary valve cusp ventriculotomy ceph R<-
-•L
caud
FIGURE 9-45. The homograft is oversized so that the single pulmonary valve cusp can fill much of the new outflow tract.
main pulmonary artery
homograft pulmonary valve cusp
R^
homograft patch
FIGURE 9-46. The homograft is tailored and then sutured to the ventriculotomy.
9
Tetralogy of Fallot 163
FIGURE 9-47. The upper segment of the homograft is stitched to the native main pulmonary artery to complete the repair.
9-4. Repair with Conus Left Anterior Descending Coronary Artery A critical part of this repair is relief of infundibular pulmonary stenosis without damage to the left anterior descending coronary artery which traverses the right ventricular outflow tract after originating from the main right coronary. Division of the coronary vessel followed by aorta to coronary artery bypass grafting is a poor option in children because of the small size and the lack of growth potential of currently acceptable grafts. Depending on the location of the abnormal coronary artery relative to obstruction in the right ventricular outflow tract and the extent of development of the outflow tract, obstructing muscle and fibrous tissue can be adequately resected by the transatrial approach. This preferred method may be more difficult in small infants so that an early palliative shunt may be necessary in order to delay corrective surgery until an older age. Alternatively, one or more coronary sparing right ventriculotomies are made for exposure. When excision of obstructing tissue is not feasible, the right ventricular outflow tract can be bypassed with a valved homograft conduit, which connects the right ventricular inlet to the main pulmonary artery.
164 Color Atlas of Congenital Heart Surgery subvalvar chamber circumflex coronary artery left anterior descending coronary artery main right coronary artery
high marginal branches ceph R«-
-•L
caud
9-48. The main right coronary artery arises anteriorly from the ascending aorta and is located in the AV groove. Branches of this artery include the left anterior descending artery and two large high marginal branches. The circumflex coronary artery originates from the posterior ascending aorta and is seen lateral to the right ventricular outflow tract. The anomalous left anterior descending coronary artery is located over the os infundibulum. FIGURE
left anterior descending coronary artery os infundibulum obstructing fibrous tissue and muscle high marginal branches
R<
9-49. After establishing cardiopulmonary bypass with cardioplegic arrest and profound local cardiac cooling, a short oblique right ventriculotomy is made between the high marginal and left anterior descending branches of the right coronary artery. Obstructing fibrous and muscle tissue is seen surrounding the os infundibulum. FIGURE
9 FIGURE 9-50. After
Tetralogy of Fallot 165
extensive
resection of obstructing infundibular tissue, the outflow tract is augmented by placing a Dacron® patch in the ventriculotomy (currently, a GoreTex® or tissue patch would be used). outflow tract patch
9-5. Repair of Shunts Previously constructed systemic-to-pulmonary artery shunts must be closed at the time of total corrective surgery. 9-5-7. Classic Right Blalock-Taussig
Shunt
superior vena cava
right subclavian artery trunk area of anastomosis
ascending aorta
FIGURE 9-51. The sub-
clavian artery trunk of the right BlalockTaussig shunt is usually easy to identify medial to the superior vena cava while working within the pericardial space.
right pulmonary artery
166 Color Atlas of Congenital Heart Surgery
right subclavian artery trunk
R +
9-52. Before cardiopulmonary bypass, a large silk suture is passed around the subclavian trunk and will be used to ligate the shunt. FIGURE
9-5-2. Classic Left Blalock-Taussig Shunt
innominate artery
left subclavian artery trunk
innominate vein
ascending aorta
R^
9-53. The proximal left subclavian artery trunk is isolated at its origin from the left-sided innominate artery. The dissection is performed high in the mediastinum above the innominate vein. Dissection here is safer because one avoids scar tissue, which may develop at the site of the subclavian artery to pulmonary artery anastomosis. An alternative exposure of this shunt is by dissection of the left pulmonary artery, starting within the pericardial space, until the region of the shunt anastomosis is seen. FIGURE
9
Tetralogy of Fallot 167
9-5-3. Modified Blalock-Taussig Shunt
Gore-Tex graft
ascending aorta
superior vena cava ceph R^-
-•L
caud FIGURE 9-54. Dissection of this shunt is similar to the classic Blalock-Taussig shunt. The tubular Gore-Tex® graft is a firm structure and is palpable before full exposure. The Gore-Tex® graft is encased in a sheath of fibrous tissue which, when entered, can be easily dissected from the graft.
Gore-Tex graft divided
ceph R«-
-•L
caud
9-55. To close the shunt, the graft is divided to avoid neck artery distortion by a fixed and undivided graft with later patient growth. Stitch closure of the upper end of the graft is complete and the lower end will be closed. FIGURE
168 Color Atlas of Congenital Heart Surgery
9-5-4. Waterston Shunt (Ascending Aorta to Right Pulmonary
Artery)
ascending aorta
aorta to right pulmonary artery connection
ceph R<-
-•L
caud FIGURE 9-56. For closure of this shunt, the distal ascending aorta or proximal arch is cannulated to allow ample room for ascending aortic cross-clamping above the shunt anastomosis. Immediately after cardiopulmonary bypass is established, the shunt is compressed to avoid flooding of the lungs during initial bypass cooling before aortic clamping. Digital compression of the shunt is also required after cross-clamping and during delivery of cardioplegia solution. The proximal ascending aorta is opened anteriorly to expose the anastomosis.
ceph R<-
-•L
caud FIGURE 9-57.
Multiple interrupted sutures are placed in the rim of the anastomosis to close it from side to side. This helps to avoid iatrogenic narrowing of the right pulmonary artery at this site.
9
Tetralogy of Fallot
169
R^
FIGURE 9-58. Stitches are tied to complete the shunt closure. At this stage a small incision is made in the main pulmonary artery. The right pulmonary artery is measured with Hegar dilators or sizers to ensure that there is no narrowing at the site of shunt closure.
ascending aorta
right pulmonary artery
ceph R<-
->L
caud
FIGURE 9-59. An alternative method of Waterston shunt closure is used when there is narrowing of the right pulmonary artery at the site of the anastomosis. The right pulmonary artery is detached from the ascending aorta, taking with it a small rim of aortic wall surrounding the anastomosis.
170
Color Atlas of Congenital Heart Surgery
ascending aorta
aortotomy closure right pulmonary artery
R<
FIGURE 9-60. The aortotomy is closed primarily with a continuous suture. For hemostasis, a clamp is placed over the pulmonary arteriotomy.
ascending aorta
right pulmonary artery
FIGURE 9-61. The right pulmonary artery is inspected and then opened longitudinally over any area of stenosis proximal and distal to the arteriotomy.
9
Tetralogy of Fallot
171
pericardial patch
ceph R*-
->L
caud
FIGURE 9-62. The right pulmonary artery is reconstructed with an anterior pericardial patch.
9-5-5. Central Shunt (Ascending Aorta to Main Pulmonary Artery)
orifice of aorta to main pulmonary artery shunt
left pulmonary artery orifice
ascending aorta
ceph R<-
-•L
caud
FIGURE 9-63. Aortic cannulation and cross-clamping are high, and this allows adequate exposure of the ascending aorta to the main pulmonary artery shunt. This anastomosis is exposed by working through the main pulmonary artery.
172
Color Atlas of Congenital Heart Surgery
left pulmonary artery orifice
right pulmonary artery orifice
ceph R<-
-•L
caud
FIGURE 9-64. The shunt is closed with multiple interrupted sutures.
9-5-6. Potts Shunt (Descending Aorta to Left Pulmonary
Artery)
ascending aorta
main pulmonary artery
left pulmonary artery
shunt orifice ceph R<-
-•L
caud
FIGURE 9-65. This anastomosis is repaired by working through an anterior opening in the proximal left pulmonary artery. Hypothermic circulatory arrest with low-flow cerebral perfusion is necessary during closure of the anastomosis.
9
Tetralogy of Fallot 173
9-6. Late Pulmonary Valve Placement after Tetralogy Repair with a Transannular Patch Placement of a transannular patch is necessary in some patients during tetralogy repair but this results in pulmonary regurgitation. Although many patients tolerate this indefinitely, 30% to 40% will require later valve placement. A pulmonary homograft valved conduit is the replacement of choice. Indications for surgery include progressive right ventricular dilatation and dysfunction, paradoxical septal motion, and flattening of the septum, which may result in left ventricular dysfunction and/or symptoms such as fatigue on exertion. Rarely, patients will have ventricular arrhythmias. Surgery is performed utilizing cardiopulmonary bypass and mild hypothermia. A left ventricular vent is placed but aortic cross-clamping and cardioplegic arrest are not usually necessary. 9-66. After placing the child on bypass, an incision is made through the previously placed outflow tract patch. The caudal extreme of this incision ends at the area of the native pulmonary valve annulus. FIGURE
previously placed tissue outflow tract patch
>L
anterior muscle ledge
valve leafs
9-67. A prepared pulmonary homograft is tailored, leaving a narrow posterior muscle bar below the valve annulus. The anterior muscle segment is left long and will be utilized in the repair. FIGURE
posterior muscle ledge
174 Color Atlas of Congenital Heart Surgery
opened pulmonary artery branches
• 9-68. The distal branches of the homograft are opened and then tailored to fit the native distal outflow tract. FIGURE
9-69. First, the posterior muscle ledge of the graft is stitched to the posterior outflow tract in the region of the native valve annulus. This suture encompasses roughly 50% of the circumference of the outflow tract and 50% of the circumference of the homograft valve annulus. A small probe is used to check for residual openings in the suture line which are closed with interrupted figure of eight sutures. FIGURE
9
Tetralogy of Fallot 175
left pulmonary artery right pulmonary artery distal suture
homograft
ceph R<-
-•L
caud FIGURE 9-70. The distal end of the homograft is stitched to the posterior wall of the native main pulmonary artery in an area proximal to the orifices of the pulmonary artery branches.
distal suture
homograft
anterior muscle ledge
9-71. The anterior part of the distal suture line is always brought to the surface of the outflow tract to avoid placement of the homograft completely within the native structures. The latter can result in narrowing of the homograft in the region of the distal suture line. FIGURE
176 Color Atlas of Congenital Heart Surgery
aneurysm
ceph R<-
-•L
caud
9-72. In this patient, there is thinning and aneurysmal dilatation of the anterior ventricular wall caudad to the ventriculotomy. FIGURE
homograft
plication sutures
ceph R«-
-•L
caud
9-73. Multiple imbricating mattress sutures are placed in the aneurysm to obliterate the dilated ventricular wall.
FIGURE
9 FIGURE
9-74. The
Tetralogy of Fallot 177
anterior
muscle ledge of the homograft conduit is stitched to the remaining ventriculotomy. The muscle ledge functions as the hood of the conduit, allowing the homograft valve annulus to remain oriented in the anterior posterior plane to avoid homograft valve regurgitation. anterior muscle ledge
9-7. Palliation and Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome Infants born with this rare syndrome may develop aneurysmal dilatation of the main pulmonary artery and/or its branches; this may cause tracheal and bronchial obstruction resulting in severe respiratory distress. In the rare case when totally corrective surgery cannot be undertaken, airway obstruction may be relieved by translocating the offending right or left pulmonary artery from the posterior to the anterior mediastinum. This operation is largely of historic interest but emphasizes the effectiveness of debulking the mediastinum in successfully treating this anomaly. In symptomatic infants, the ideal operation is total repair including placement of a pulmonary valve with tailoring of the aneurysmal pulmonary arteries regardless of the size or age of the patient.
ascending aorta
FIGURE
9-75. In
this
small
infant with right bronchial obstruction, palliation is performed by working through a right thoracotomy. After the pericardium is opened, the enlarged right pulmonary artery is seen.
aneurysmal right pulmonary artery
ant ceph^-
-•caud
post
178
Color Atlas of Congenital Heart Surgery
ascending aorta
right pulmonary artery
superior vena cava ant ceph^-
-•caud
post
FIGURE 9-76. The right pulmonary artery is dissected from its origin and silk sutures for snaring are passed around the branches.
ascending aorta
proximal right pulmonary artery
superior vena cava
distal right pulmonary artery ant ceph^-
->caud
post
FIGURE 9-77. The proximal right pulmonary artery near the main pulmonary artery is divided and closed. The distal right pulmonary artery is shifted lateral to the superior vena cava.
9
Tetralogy of Fallot 179
ascending aorta
superior vena cava
distal right pulmonary artery
ant ceph^-
-•caud
post
9-78. Using a side-biting clamp on the anterior main pulmonary artery, a synthetic tubular graft is anastomosed to this vessel. Right pulmonary arterial vascular continuity is reconstructed by attaching this graft to the distal pulmonary artery. The graft passes in front of the ascending aorta and superior vena cava, removing the bulk of the aneurysmal right pulmonary artery from the posterior mediastinum; this removes the extrinsic pressure on the right bronchus and relieves the bronchial obstruction. FIGURE
ascending aorta
main pulmonary artery
right ventricle
ceph R<-
-•L
caud
9-79. In another neonate, totally corrective surgery is undertaken. The enlarged main pulmonary artery and dilated right ventricle are seen. FIGURE
180 Color Atlas of Congenital Heart Surgery
ascending aorta
main pulmonary artery right pulmonary artery
ceph R<-
-•L
caud
9-80. By retracting the ascending aorta the huge right pulmonary artery is seen. Its diameter is at least twice that of the ascending aorta. FIGURE
pulmonary valve annulus
ceph R<-
,,. , %$'
-•L
caud
9-81. The child is placed on cardiopulmonary bypass, and the proximal main pulmonary artery opened. The pulmonary valve annulus is seen; however, only rudimentary valve leaflets are present. FIGURE
9
Tetralogy of Fallot 181
removed anterior segment of right and left pulmonary arteries
ascending aorta
9-82. A large segment of the anterior wall of the right and left pulmonary arteries is excised. FIGURE
area of anterior resection of left pulmonary artery
ascending aorta
area of anterior resection of right pulmonary artery R +
9-83. After right and left pulmonary arteries are tailored, there is less bulk in the mediastinum; this relieves tracheal and bronchial obstruction. FIGURE
182 Color Atlas of Congenital Heart Surgery
anterior resection of left pulmonary artery
anterior resection of main pulmonary artery
ceph R«-
-•L
caud FIGURE 9-84. Part of the anterior main pulmonary artery is resected, as well as the anterior left pulmonary artery.
right pulmonary artery
ceph R<-
-•L
caud FIGURE
9-85. The anterior pulmonary arteries are closed with a continuous suture.
9
Tetralogy of Fallot 183
ascending aorta
left pulmonary artery
right pulmonary artery
posterior suture
ceph R<-
->L
caud
9-86. In another child, the pulmonary arteries are tailored posteriorly. The posterior aspect of the aneurysmal vessels is resected and then closed primarily within the pulmonary arteries. This may be advantageous because aneurysmal tissue that is in direct contact with the trachea and bronchi is removed and this may result in improved relief of tracheal bronchial obstruction. FIGURE
pulmonary valve annulus
ventricular septal defect
tricuspid valve leaflet
9-87. The subcristal ventricular septal defect is seen, and multiple interrupted sutures are placed around its rim.
FIGURE
184
Color Atlas of Congenital Heart Surgery
VSD patch
FIGURE 9-88. The VSD is closed with a Dacron® patch.
anterior mitral valve leaflet of homograft
aortic homograft
R-*
FIGURE 9-89. An aortic valve homograft with attached ascending aorta is used for pulmonary valve replacement. The homograft anterior mitral valve leaflet will be used in the repair. An aortic homograft is used when residual pulmonary artery pressure elevation is anticipated.
9
Tetralogy of Fallot 185
native pulmonary artery
homograft
anterior mitral valve leaflet of homograft
ceph R««-
-•L
caud FIGURE 9-90. The homograft is tailored, and the posterior graft annuliis is stitched to the native pulmonary valve annulus. The upper end of the homograft is stitched to the inner wall of the main pulmonary artery posteriorly (proximal to the native branch pulmonary artery orifices) and the rim of the native pulmonary artery anteriorly.
distal suture
anterior mitral valve leaflet of homograft
ceph R«-
-•L
caud
9-91. The homograft mitral valve leaflet is stitched to the right ventriculotomy and forms a hood inferior to the homograft valve; this allows the valve to remain in an anatomic position and avoids valve regurgitation caused by annular distortion.
FIGURE
186
Color Atlas of Congenital Heart Surgery
pulmonary valve homograft
R^
FIGURE 9-92. In another child, a homograft pulmonary valve with an attached main pulmonary artery is used for pulmonary valve replacement.
posterior muscle ledge
native pulmonary valve annulus
FIGURE 9-93. The repair is begun by attaching the posterior subannular homograft muscle bar to the region of the native valve annulus with a continuous suture.
9
Tetralogy of Fallot 187
homograft
anterior muscle edge
9-94. Insertion of the homograft is similar to insertion of an aortic valve homograft. The anterior muscle ledge of the homograft is stitched to the ventriculotomy, which forms the hood over the lower end of the conduit. FIGURE
10
Double Outlet Ventricles
Double outlet ventricle is present when all of one great vessel and more than 50% of the second great vessel arise from one ventricle. The presence of pulmonary stenosis protects the lungs from overperfusion, and in its absence the lungs are flooded and there is congestive heart failure. Total correction is undertaken at any age when symptoms dictate. Surgery is performed with cardiopulmonary bypass, moderate hypothermia, aortic cross-clamping, and cardioplegia with local cardiac cooling.
10-1. Double Outlet Right Ventricle
10-1. In this infant, the great vessels and semilunar valves are oriented side by side and at the same level.
FIGURE
188
10
Double Outlet Ventricles 189
pulmonary valve
aortic valve
ventricular septal defect
ceph R<-
-•L
caud
10-2. After placing the child on bypass with aortic clamping and cardioplegia, a high longitudinal right ventriculotomy is made and extended cephalad into the proximal main pulmonary artery. There is a common annulus between the semilunar valves and both arise from the right ventricle. The ventricular septal defect is subaortic. FIGURE
ventricular septal defect
ventricular septum
ceph R<-
-•L
caud
10-3. The ventricular septal defect (VSD) is restrictive and will be enlarged anteriorly or toward the infant's left. FIGURE
190 Color Atlas of Congenital Heart Surgery
ventricular septal incision
ceph R<-
-•L
caud
10-4. The ventricular septum is incised in a more cephalad area and just below the pulmonary valve annulus, keeping the incision remote from the His bundle. FIGURE
pulmonary valve
aortic valve
ventricular septal defect
ceph R<-
-•L
caud
10-5. Interrupted felted mattress sutures are placed along the rim of the VSD. In the posterior inferior corner of the VSD, caution is used to avoid damage to the His bundle, similar to repair of tetralogy of Fallot. Working cephalad from this point, stitches are placed to the right of the aortic valve annulus and then along the cephalad rim of that annulus. In contradistinction to repair of tetralogy, there is a greater distance from the posterior inferior corner of the VSD to the upper right aspect of the aortic valve annulus. A greater number of sutures will be required in this area. FIGURE
10
Double Outlet Ventricles 191
baffle patch
ceph R«-
-•L
caud
10-6. The Dacron® patch is cut in a somewhat rectangular shape that is needed to construct the tunnel from the VSD to the aortic valve. The longer part of the patch is positioned from left to right. A redundant patch is placed to avoid left ventricle to aortic obstruction. The aortic valve is now beneath the patch, while the pulmonary valve leaflets are on the right ventricular side of the patch. FIGURE
intra ventricular baffle
ceph R<-
->L
caud
10-7. Stitches are tied as the patch is lowered into position. The upper margin of the patch will be attached directly to the common annulus between the semilunar valves or in the base of the pulmonary valve leaflets, being careful to avoid injury to the aortic valve leaflets. FIGURE
192 Color Atlas of Congenital Heart Surgery
outflow tract patch
FIGURE 10-8. A Gore-Tex® patch is used to reconstruct the outflow tract in order to avoid right ventricular outlet obstruction caused by the bulging intraventricular baffle.
outflow tract patch
ceph R«-
-•L
caud FIGURE
10-9. The repair is complete with placement of the outflow tract patch.
10
Double Outlet Ventricles 193
10-2. Double Outlet Left Ventricle
aorta
main pulmonary artery
main left coronary artery right coronary artery ceph R<-
-•L
caud FIGURE 10-10. In this child, double outlet left ventricle is associated with an anomalous right coronary artery that arises from the main left coronary artery and courses on the surface of the upper right ventricle.
aortic valve
pulmonary valve
ventricular septum
ceph R<-
ventricular septal defect
->L
caud
10-11. After cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a high right ventriculotomy is made transversely in anticipation of placement of an extracardiac conduit. The aortic valve is posterior and rightward, while the pulmonary valve is to the child's left. Both valves are seen through the VSD and are on the left ventricular side of the septum. FIGURE
194 Color Atlas of Congenital Heart Surgery
pulmonary valve
common annulus
aortic valve fibrous tissue remnant of membranous septum ceph R«-
anterior leaf of tricuspid valve
-•L
caud
10-12. The ventricular septum is retracted anteriorly and the common annulus between the pulmonary and aortic valves is seen. A fibrous tissue remnant of underdeveloped membranous ventricular septum is seen at the posterior inferior corner of the VSD adjacent to the tricuspid valve leaflet. Repair stitches can be safely placed in this fibrous tissue and in the base of the anterior tricuspid valve leaflet to avoid damage to the His' bundle. Multiple interrupted felted mattress sutures will be used for the repair, starting at the posterior inferior rim of the ventricular septal defect. Stitches will be placed around the rim of the VSD to the left of the common annulus and between the pulmonary and aortic valve annuli toward the patient's right. FIGURE
pulmonary valve
patch
ceph R^-
-•L
caud
10-13. Stitches are placed in the rectangular-shaped knitted Dacron® patch and tied. The length of the rectangular patch is placed from side to side to cover the VSD and the aortic valve. The pulmonary valve remains on the right ventricular side of the patch. FIGURE
10
Double Outlet Ventricles 195
porcine valved Dacron conduit
ceph R^-
-•L
caud FIGURE 10-14. To avoid right ventricular outflow tract obstruction caused by the large intraventricular baffle, a porcine-valved Dacron® conduit is placed from the ventriculotomy to the main pulmonary artery. The integrity of the right coronary artery is maintained.
main pulmonary artery aorta
caud
10-15. In another patient, the great vessels are normally related, but the proximal main pulmonary artery is in a more posterior location.
FIGURE
196 Color Atlas of Congenital Heart Surgery
ventricular septum
ventricular septal defect
ceph R-«-
-•L
caud
10-16. After cardiopulmonary bypass is established and with aortic clamping, cardioplegia, and profound local cardiac cooling, a high longitudinal right ventriculotomy is made. The VSD is seen, but neither semilunar valve is related to the right ventricle. Only ventricular septum is seen in the cephalad region of the right ventricle because the pulmonary valve is not found here. FIGURE
upper infundibulum
area of pulmonic and aortic valve
10-17. The ventricular septum is retracted, and the blind end of the right ventricular infundibulum is seen. The pulmonic and aortic valves are on the left ventricular side of the septum. FIGURE
10
Double Outlet Ventricles 197
pulmonic valve
right ventriculotomy
FIGURE 10-18. A longitudinal main pulmonary arteriotomy is made and the pulmonic valve is seen. An instrument placed through the valve passes into the left ventricle to confirm the presence of the double outlet left ventricle.
area of common annulus of aortic and pulmonic valves baffle patch
ceph R<-
->L
caud
10-19. The ventriculotomy is extended to the pulmonary arteriotomy. Multiple interrupted felted mattress sutures are placed around the rim of the VSD. In the cephalad area, these stitches are placed in the common annulus of the semilunar valves, while, to the patient's right, stitches are placed in front of the aortic valve annulus. A rectangular-shaped Dacron® patch is then positioned to construct the intraventricular baffle. The right ventricular outflow tract will be reconstructed with a patch over the ventriculotomy and the proximal main pulmonary artery.
FIGURE
11
Pulmonary Atresia with Ventricular Septal Defect
11-1. Total Repair
atretic outflow tract
ventricular septal defect
11-1. Working through a median sternotomy, cardiopulmonary bypass is established. Repair is carried out with aortic clamping, cardioplegia, and profound local cardiac cooling. A high longitudinal right ventriculotomy is made extending cephalad into the outflow tract chamber which is atretic at its upper end. The ventricular septal defect (VSD) is subcristal and nonrestrictive. FIGURE
198
11
Pulmonary Atresia with Ventricular Septal Defect
199
aortic valve
anterior leaf of tricuspid valve
ceph R<-
-•L
caud FIGURE 11-2. The VSD is retracted anteriorly, and the aortic valve is seen. Typically, this valve is equally related to right and left ventricles. Multiple interrupted felted mattress sutures are placed around the rim of the VSD. Stitches in the posterior inferior corner of the defect are placed superficially in the rim to avoid damage to the His' bundle. With location of the aortic valve partly over the right ventricle, the distance between the posterior inferior corner of the VSD and the cephalad margin of the aortic valve is great and requires multiple sutures to close the VSD with the Dacron® baffle.
patch
anterior leaf of tricuspid valve
ceph R«-
-•L
caud
11-3. Stitches are placed in a rectangular-shaped patch of knitted Dacron® and tied. The length of the patch is in the transverse plane and creates a tunnel or baffle that connects the left ventricle to the aortic valve.
FIGURE
200
Color Atlas of Congenital Heart Surgery
main pulmonary artery
region of outflow tract atresia
infundibulum R<
FIGURE 11-4. There is tissue continuity between the infundibulum and the main pulmonary artery. The right ventriculotomy is extended across the area of outflow tract atresia and onto the proximal main pulmonary artery.
pericardial patch
FIGURE 11-5. Vascular continuity between the right ventricle and main pulmonary artery is established by placing a pericardial outflow tract patch over the high ventriculotomy, the area of atresia, and the proximal main pulmonary artery. Pericardium is used here because of its hemostatic qualities. A patch of Gore-Tex® or homograft pulmonary artery wall may be used and avoids the risk of late development of a pericardial patch aneurysm.
11
Pulmonary Atresia with Ventricular Septal Defect
201
ceph R«-
-•L
t caud
11-6. A Dacron® patch cover is stitched over the pericardial outflow tract patch. Dense scar tissue will eventually engulf the Dacron® and form a supporting buttress over the pericardial patch. This technique is used to avoid late patch aneurysm formation. FIGURE
ascending aorta
main pulmonary artery
11-7. In another patient, there is proximal atresia and severe hypoplasia of the main pulmonary artery. FIGURE
202
Color Atlas of Congenital Heart Surgery
ventriculotomy
R^
FIGURE 11-8. An oblique high right ventriculotomy is made in a location to avoid injury to surrounding coronary arteries. Hypertrophied myocardium at the ventriculotomy is excised.
ventricular septal defect
R^
FIGURE 11-9. A large VSD is exposed.
11
Pulmonary Atresia with Ventricular Septal Defect
203
Dacron patch
ceph R^-
-•L
caud FIGURE
11-10. Repair sutures are placed in a rectangular-shaped Dacron® patch.
patch
ceph R<-
-•L
caud
11-11. Stitches are tied orienting the length of the rectangular patch transversely to connect the left ventricular flow tract to the aortic valve. FIGURE
Color Atlas of Congenital Heart Surgery
patch
ceph R^-
-•L
caud
FIGURE 11-12. Ventricular septal defect closure is completed by passing additional stitches through the upper rim of the patch and then through the floor of the outflow tract.
homograft wall patch
left pulmonary artery
main pulmonary artery
ceph R<-
-•L
caud
FIGURE 11-13. In this patient, there is stenosis of the proximal left pulmonary artery. An incision is made across the area of stenosis and a homograft wall patch is applied to this area.
11
Pulmonary Atresia with Ventricular Septal Defect
205
homograft wall patch
ceph
•
caud FIGURE 11-14. Sutures have been placed in the triangular-shaped homograft wall patch to cover the incision in the left pulmonary artery.
homograft wall patch
left pulmonary artery
ceph R<-
-•L
caud FIGURE
11-15. There is adequate relief of the area of stenosis after placement of the patch.
206 Color Atlas of Congenital Heart Surgery
homograft conduit homograft valve
posterior suture
11-16. To establish vascular continuity from the right ventricle to the pulmonary artery, a valved aortic homograft with attached ascending aorta and mitral leaflet is used. The homograft is oriented so that the mitral leaflet of the graft is placed anteriorly. Graft placement is begun by stitching the posterior muscle bar below the homograft valve annulus to the native right ventricular outflow tract. FIGURE
anterior mitral leaf of homograft
posterior suture
ceph R<-
-•L
caud FIGURE
11-17. The posterior muscle bar suture line is completed and tied at each corner.
11
Pulmonary Atresia with Ventricular Septal Defect
207
reconstructed left pulmonary artery
distal suture
homograft conduit
ceph R«-
->L
caud
11-18. The homograft conduit is positioned with the curve of the homograft extending toward the left chest. The inner curve of the conduit hugs the lateral border of the heart. The distal conduit is cut to an appropriate length. Before this final tailoring maneuver, it is advantageous to momentarily release the aortic cross-clamp to allow the ascending aorta and the coronaries to fill. At this point, one can more accurately determine the correct length of conduit needed. The distal suture line is placed within the distal main pulmonary artery but proximal to the pulmonary artery branches. FIGURE
homograft conduit
anterior mitral leaf of homograft
11-19. The anterior part of the distal suture line is placed on the anterior surface of the pulmonary artery to avoid narrowing of the distal anastomosis. FIGURE
Color Atlas of Congenital Heart Surgery
homograft conduit
anterior mitral leaf of homograft
ceph R<-
-•L
caud
11-20. The anterior mitral leaf of the homograft conduit is stitched to the anterior rim of the ventriculotomy, creating a hood that allows the homograft valve to assume a natural position. This helps to avoid homograft valve annulus distortion and valve regurgitation postoperatively. FIGURE
homograft conduit
R^
11-21. After completing homograft conduit placement, the left pericardium may be opened to allow the conduit to rest in the left chest. This avoids compression of the conduit by the sternum when the latter is closed. FIGURE
11
Pulmonary Atresia with Ventricular Septal Defect
209
11-2. Recruitment of Multiple Anomalous Collateral Arteries In infants born with severe hypoplasia or absence of the pulmonary arteries, the lungs are usually supplied by multiple collateral arteries. Recruitment of these to construct branch pulmonary arteries can be followed by total repair in many cases. For most, staged thoracotomies offer the best chance of successful pulmonary artery reconstruction. The exposure is optimal through a lateral approach and this allows for precise anastomoses and recruitment of all anomalous vessels. A Blalock shunt is usually included in each recruitment operation. Later, the branch pulmonary arteries are connected centrally and then to a right ventricle to pulmonary artery valved conduit with immediate or delayed VSD closure. When working through a thoracotomy, a fifth interspace incision is used for maximal cephalad and caudad exposure.
11-22. During a left thoracotomy in this child, a hypoplastic left pulmonary artery is dissected from the pericardium to its branches. A bronchial collateral artery arises from the distal aortic arch. FIGURE
210 Color Atlas of Congenital Heart Surgery
left pulmonary artery
descending aorta
ant caud^-
-•ceph
collateral arteries
post FIGURE 11-23. The descending aorta is exposed and a second collateral artery is dissected. The distal arch collateral passes beneath the native pulmonary artery before it enters the left lung.
collateral arteries
ant caud<-
-•ceph
post FIGURE 11-24. The two collateral arteries are divided near their origins to preserve maximal length of each vessel.
11
Pulmonary Atresia with Ventricular Septal Defect
211
collateral arteries
ant caud<-
-•ceph
post
11-25. The two collaterals are incised lengthwise to the point of convergence prior to entering the left lung. These vessels are then anastomosed lengthwise, resulting in a larger recruited vessel. FIGURE
left pulmonary artery
collateral arteries
ant •ceph
caud^ post
11-26. The recruited vessel is anastomosed end to side to the hypoplastic left pulmonary artery. A modified left Blalock shunt will then be placed. FIGURE
212 Color Atlas of Congenital Heart Surgery right upper and middle lobes
right pulmonary artery collateral arteries ant ceph^-
-^•caud descending aorta
post
11-27. In another child, while working through a right fifth interspace thoracotomy, the major fissure is completely dissected. The small right pulmonary artery is seen, as are two collateral arteries that arise from the descending aorta. FIGURE
right pulmonary artery anastomosis
collateral arteries
ant ceph^-
-^•caud
post FIGURE 11-28. The upper lobe collateral vessel is anastomosed end to side to the native pulmonary artery. The lower lobe collateral artery is anastomosed to the side of the first collateral.
11
Pulmonary Atresia with Ventricular Septal Defect 213
right pulmonary artery
right upper lobe
ant ceph<-
-•caud
post
11-29. The native right pulmonary artery is dissected in the area cephalad to the lung. The anastomosed collaterals are inferior and out of view through this exposure. FIGURE
right pulmonary artery
azygos vein
Blalock shunt • caud
11-30. A modified right Blalock shunt is constructed to the hypoplastic right pulmonary artery. FIGURE
12
Pulmonary Venous Anomalies
12-1. Total Anomalous Pulmonary Venous Connection Total repair is carried out soon after establishing the diagnosis and medical stabilization of the patient. The procedure can be emergent shortly after birth when there is obstruction of the common pulmonary venous channel (as with a subdiaphragmatic connection), or in the early days of life when there is obstruction at the atrial septal level (supracardiac or intracardiac connection). When there is no obstruction to pulmonary venous return, surgery is required in the early weeks of life because the large left-to-right shunt causes congestive heart failure or failure to thrive with or without pulmonary artery hypertension. The operation is performed with cardiopulmonary bypass and moderate hypothermia with aortic clamping and cardioplegia. Periods of reduced flow may augment exposure in small infants, but total circulatory arrest with regional cerebral perfusion is almost never needed. 12-1-1. Supracardiac
Connection
FIGURE 12-1. The right heart border is dissected to expose the posterior wall of the left atrium. The common pulmonary vein that connects right and left veins is seen adjacent to the back of the left atrium.
214
12
Pulmonary Venous Anomalies
215
main pulmonary artery
ascending pulmonary venous channel pericardium
ceph R«-
-•L
caud
FIGURE 12-2. The ascending pulmonary venous channel is dissected along the left upper heart border. It can be approached by working outside the pericardium or through a short incision in the pericardium that is adjacent to the main pulmonary artery and anterior to the left phrenic nerve. This vein connects the transverse pulmonary venous channel to the innominate vein.
FIGURE
12-3. The
enlarged
innominate vein is seen. Its large size is caused by excess flow in this vessel, because all pulmonary venous drainage passes though it. This vein should not be compressed during the dissection in preparation for cardiopulmonary bypass because this might occlude pulmonary venous return and compromise cardiac output. A transesophageal ECHO probe is not passed for the same reason.
Color Atlas of Congenital Heart Surgery
atrial septal defect
caud
12-4. After the cardiopulmonary bypass is established, a transverse mid right atriotomy is made, directing this incision posteriorly to the mid part of the atrial septal defect (ASD).
FIGURE
posterior left atrial wall
orifice of left atrial appendage
atrial septum
12-5. The transverse atriotomy is extended along the posterior mid left atrial wall to the base of the left atrial appendage. An alternative technique is a limited longitudinal right atriotomy with a separate posterior transverse left atriotomy; the anastomosis is performed by retracting the right atrium toward the infant's left or by working intraatrially. The ASD is then closed working though the right atriotomy. FIGURE
12
Pulmonary Venous Anomalies
217
right upper pulmonary vein right lower pulmonary vein transverse pulmonary vein left lower pulmonary vein ceph R«-
-•L
caud
FIGURE 12-6. The posterior left atriotomy is positioned adjacent to the transverse common pulmonary vein.
left atrial wall
transverse pulmonary vein
R^
FIGURE 12-7. A generous transverse incision is made in the common pulmonary vein. The cephalad rim of the left atrium to pulmonary vein anastomosis is constructed with a continuous suture.
218
Color Atlas of Congenital Heart Surgery
opening into transverse pulmonary vein
ceph
R<-
-•L
caud
FIGURE 12-8. The caudad rim of the anastomosis is also constructed with a continuous suture. The right end of this anastomosis is at the level of the atrial septum, and it is completed with interrupted sutures to allow for growth of the anastomosis.
transverse pulmonary vein to left atrium anastomosis
atrial septum
ceph R««-
-•L
caud
FIGURE 12-9. The posterior anastomosis in the left atrial chamber is seen through the ASD.
12
Pulmonary Venous Anomalies 219
atrial septal defect patch
ceph R<-
-•L
left atrial line
caud
12-10. A continuous suture is used to close the ASD with a Dacron® patch. A left atrial line passes beneath the patch and will be exteriorized through the right atriotomy. If the left atrium were small, it would be enlarged by shifting the atrial septal patch more anteriorly, attaching it to the lateral atrial wall. FIGURE
repaired transverse right atriotomy
left atrial line ceph R<-
-•L
caud
12-11. The transverse right atriotomy is closed with a continuous suture, and the left atrial line is exteriorized. Alternatively, the left atrial line can be placed in the left atrial appendage, but it should not be placed in the right upper pulmonary vein that is near the anastomosis. FIGURE
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Color Atlas of Congenital Heart Surgery
12-1-2. Supracardiac Connection at the Superior Vena Cava to Right Atrial Junction
main pulmonary artery ceph R<-
-•L
ascending aorta
caud
FIGURE 12-12. The right pulmonary vein can be seen entering the posterior low superior vena cava. A tape is passed around the cava above this area in anticipation of direct superior caval cannulation for cardiopulmonary bypass.
left pulmonary
ceph R<-
-•L
caud
FIGURE 12-13. The left pulmonary vein is seen; it is adjacent to the right vein. The low superior vena cava at the atrial junction is enlarged due to additional flow from the pulmonary veins.
12
Pulmonary Venous Anomalies 221
superior vena cava orifice of anomalous pulmonary veins
atrial septal defect
ceph R«-
-•L
caud FIGURE 12-14. After cardiopulmonary bypass is established, a high lateral longitudinal right atriotomy is made. The atriotomy is anterior to the sinoatrial (SA) node to avoid damage to that structure. The orifice of both pulmonary veins is seen within the lower cava, and they are separated from the ASD by the septum formed by the upper wall of the left atrium and the adjacent wall of the common pulmonary venous channel.
pulmonary veins
posterior left atrial wall
ceph R<-
-•L
caud
12-15. The tissue partition between the pulmonary veins and left atrium is excised to establish a direct pathway for pulmonary venous return. FIGURE
222 Color Atlas of Congenital Heart Surgery
superior vena cava
repaired endocardium
FIGURE 12-16. The endocardium along the posterior wall of the heart is approximated with interrupted sutures; this obliterates the raw surface to guard against clot formation. It also ensures hemostasis in the event a full-thickness defect is inadvertently made at the time of the septal excision. Interrupted stitches are used for this closure to avoid narrowing of the new pathway, which might occur because of the purse string effect of a continuous suture.
Dacron patch
ceph R<-
-•L
caud
12-17. A Dacron® patch is stitched over the orifice of the pulmonary veins, the new pathway, and the ASD. The area behind the patch is inspected to be sure the new pathway is unobstructed. FIGURE
12
Pulmonary Venous Anomalies 223
Dacron patch
ceph R<-
-•L
caud FIGURE
12-18. Pulmonary venous return is now diverted by the patch to the left atrium.
12-1-3. Intracardiac Connection to the Right Atrium
atrial septal defect left pulmonary veins ceph R««-
right pulmonary veins
-•L
caud
12-19. After the cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a lateral longitudinal right atriotomy is made. Right upper and right lower pulmonary veins enter the right atrium separately. The left pulmonary veins form a common channel, which drains to the right atrium posterior to the ASD. FIGURE
224 Color Atlas of Congenital Heart Surgery
left pulmonary
ceph R«-
->L
caud FIGURE 12-20. The coronary sinus is a separate orifice and is located between the left pulmonary vein orifice and the tricuspid valve.
tricuspid valve
ceph R<-
-•L
caud
incised septum between left pulmonary veins and left atrium FIGURE 12-21. Tissue along the lower margin of the ASD and between the left pulmonary veins and left atrium is incised to establish an unobstructed pathway for pulmonary venous flow after the repair. The endocardium is closed with interrupted sutures.
12
Pulmonary Venous Anomalies 225
ceph R<-
-•L
caud FIGURE 12-22. A large nonobstructing Dacron® patch is stitched over the ASD and all pulmonary veins. The atrio-ventricular (AV) node is caudad to the coronary sinus, which is remote from the repair.
12-1-4. Intracardiac Connection to the Coronary Sinus
coronary sinus
left pulmonary
right pulmonary veins
ceph R«-
-•L
caud FIGURE 12-23. After cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a longitudinal right atriotomy is made. The coronary sinus is large, and the orifices of left and right pulmonary veins, respectively, are seen within the sinus.
226
Color Atlas of Congenital Heart Surgery
atrial septal defect
coronary sinus
ceph R<-
right pulmonary
-•L
caud
FIGURE 12-24. The restrictive ASD is cephalad to the coronary sinus.
right angle clamp tip in septum ceph R<-
-•L
caud
FIGURE 12-25. A right-angle clamp is passed through the ASD to expose the septum that separates the roof of the coronary sinus and the left atrium.
12
Pulmonary Venous Anomalies 227
base of excised septum
ceph R<-
-•L
caud
12-26. The septum that separates the coronary sinus from the left atrium is excised, resulting in a large communication between the coronary sinus and the left atrium. FIGURE
Dacron patch
12-27. A Dacron® patch is stitched over the coronary sinus and adjacent ASD. Along the caudad margin of the coronary sinus, stitches are placed deep in the sinus, remote from the AV node and His' bundle. These sutures are interrupted and will be tied after removal of the aortic cross-clamp when sinus rhythm is observed. If sutures are encroaching on conductive tissue, AV dissociation will occur as the offending stitch is tied. In that event it is replaced. A continuous suture is used along the posterior and cephalad margins of the patch. FIGURE
228 Color Atlas of Congenital Heart Surgery
12-1-5. Subdiaphragmatic
Connection
patent ductus arteriosus left pulmonary artery main pulmonary artery
ceph R<-
->L t caud
ascending aorta
12-28. The ductus arteriosus is dissected at the distal main pulmonary artery. The anatomy may be unclear because of the large size of the ductus, and it is identified by visualizing the proximal right and left pulmonary arteries, respectively. The ductal structure is located between those two vessels. The ductus is closed with a ligature or a metal clip. In the presence of preoperative pulmonary artery hypertension, an ECHO study may not show ductal flow when it is patent. This structure is, therefore, surgically closed in all cases of total anomalous pulmonary venous connection. FIGURE
right upper pulmonary vein
left pulmonary vein
right lower pulmonary vein common pulmonary R<-
12-29. The left atrium along the right heart border is dissected. The right and left pulmonary veins form a confluence at the common pulmonary vein, which then passes caudad to below the diaphragm. FIGURE
12
Pulmonary Venous Anomalies 229
left atrlotomy
opening in common pulmonary vein
ceph R<-
-•L
caud FIGURE 12-30. Repair is carried out with cardiopulmonary bypass and moderate hypothermia with intermittent low flow when needed. An opening is made in the common pulmonary vein. An adjacent left atriotomy is made.
left atrium
common pulmonary
ceph R
-•L
caud
12-31. A side-to-side anastomosis between the pulmonary vein and left atrium is constructed, using a continuous suture along the posterior rim of the anastomosis. FIGURE
230 Color Atlas of Congenital Heart Surgery
right atriotomy
anastomosis
ceph R<-
-•L
caud
12-32. The more anterior margin of the anastomosis is constructed with a continuous suture, in part, and with interrupted sutures that may allow for growth of the anastomosis. An adjacent right atriotomy is made through which to visualize the ASD. FIGURE
suture closure of atrial septal defect
ceph R<-
-*L
caud
12-33. Here, the ASD is closed primarily. If the left atrium were restrictive, the atrial septum would be moved to a more anterior position to enlarge the left atrium; this can be carried out by using a patch to close the septal defect. The lower common pulmonary vein below the anastomosis is ligated, and a left atrial line is placed through the left atrial appendage remote from the anastomosis. FIGURE
12
Pulmonary Venous Anomalies 231
12-2. Pulmonary Vein Stenosis This rare anomaly occurs at the entrance of pulmonary veins to the left atrium. Because of the severe consequences of pulmonary venous hypertension, including congestive heart failure and/or pulmonary vascular obstructive disease, surgery to repair this lesion is indicated immediately after discovery, regardless of the age of the patient. Direct repair of the pulmonary vein is performed in the presence of mild-to-moderate stenosis. An alternative repair is sutureless with stitches placed in adjacent pericardium and in the pulmonary vein wall remote from the venotomy.
right pulmonary
atrial septal defect
FIGURE 12-34. After cardiopulmonary bypass is established, with aortic clamping, cardioplegic arrest, and profound local cardiac cooling, a right atriotomy is made. Normal right pulmonary veins are seen through the ASD.
>L
stenotic left pulmonary vein orifice
FIGURE 12-35. The stenotic orifice of the left pulmonary veins is seen through the ASD.
232
Color Atlas of Congenital Heart Surgery
left upper pulmonary
base of left atrial appendage
ceph R<-
-•L
caud
FIGURE 12-36. The obstruction is near the confluence of the left upper and lower pulmonary veins. A Y-shaped incision is made from the base of the left atrial appendage onto the upper and lower pulmonary veins, respectively.
left lower pulmonary
FIGURE 12-37. The incision over the area of stenosis is extended onto the left lower pulmonary vein.
12
Pulmonary Venous Anomalies
233
anastomosis
R^
FIGURE 12-38. Repair is performed by closing the incision as a V. The corner of the incisions over the pulmonary veins is moved to the child's right and stitched to the end of the incision at the base of the left atrial appendage. The closure is longitudinal, while the incision was originally made in a transverse plane from the atrium into the veins.
closure of atrial septum
ceph R<-
->L
caud
FIGURE 12-39. The ASD is closed primarily.
234
Color Atlas of Congenital Heart Surgery
atrial septum
ceph R«-
-•L
stenotic orifice of right pulmonary veins
caud
FIGURE 12-40. In another patient and after the cardiopulmonary bypass is established, with aortic clamping, cardioplegic arrest, and profound local cardiac cooling, a right atriotomy is made. Stenosis of the right pulmonary veins is seen through the ASD.
right pulmonary vein orifice
caud
FIGURE 12-41. The area of vein stenosis admits a 1-mm probe.
12
Pulmonary Venous Anomalies 235
interrupted stitches in anastomosis
R<
FIGURE 12-42. By working outside the heart, a transverse incision is made from the atrial wall into the right pulmonary vein and across the area of stenosis. This incision is closed longitudinally with interrupted sutures.
right pulmonary veins
R^ caud
12-43. After the repair is completed, a 5-mm probe passes easily through the anastomosis.
FIGURE
236
Color Atlas of Congenital Heart Surgery
patch over atrial septal defect
ceph R<-
-•L
caud
FIGURE 12-44. To avoid distortion of the vein repair, the laterally positioned ASD is closed with a Dacron® patch.
13
Left Atrial Obstructive Lesions
13-1. Cor Triatriatum In the presence of this rare anomaly, a membrane separates the left atrium into two chambers. The proximal chamber communicates with the pulmonary veins, while the distal chamber communicates with the atrial appendage and mitral valve.
proximal chamber of left atrium
orifice to distal chamber of left atrium
right pulmonary
ceph R^-
-•L
caud
13-1. After the cardiopulmonary bypass is established, the dilated proximal left atrial chamber is entered along the right heart border. Pulmonary veins enter this chamber, which is separated from the distal chamber by a fibromuscular septum.
FIGURE
237
238 Color Atlas of Congenital Heart Surgery
right upper pulmonary
enlarged proximal left atrial chamber
right lower pulmonary vein
ceph R<*-
-•L
caud FIGURE
13-2. The enlarged proximal chamber above the obstruction is exposed.
orifice between proximal and distal left atrial chambers
ceph R<-
-•L
caud FIGURE
13-3. The orifice that separates the two atrial chambers is restrictive.
13
Left Atrial Obstructive Lesions
239
fibromuscular septum separating left atrial chambers
ceph -•L
R<-
caud
FIGURE 13-4. During the repair, the membrane is opened by a cephalad incision starting in the obstructive orifice.
rim of excised septum
mitral valve
ceph R^-
lower pulmonary vein
-•[_
caud
FIGURE 13-5. The membrane is excised, allowing free communication between the proximal and distal atrial chambers.
240 Color Atlas of Congenital Heart Surgery
rim of excised septum
orifice of appendage ceph R<-
-•L
caud
13-6. The membrane region is retracted cephalad to expose the orifice of the left atrial appendage, which communicates with the distal chamber.
FIGURE
remnant of excised septum orifice of appendage
mitral valve
ceph A R<- - • L caud
13-7. The proximity of the left atrial appendage to the mitral valve in the distal chamber is seen.
FIGURE
13
Left Atrial Obstructive Lesions 241
13-2. Supramitral Ring In the presence of this anomaly, a fibrous membrane located immediately above the mitral valve annulus causes symptoms similar to mitral stenosis. The pulmonary veins and left atrial appendage enter the left atrium above the membrane.
fibrous membrane
orifice to mitral valve
ceph R<-
-•L
caud
13-8. After the cardiopulmonary bypass is established, the left atrium along the right heart border is entered. A thick membrane is present immediately above the mitral valve, which is not yet visualized. FIGURE
orifice
FIGURE 13-9. The orifice through the membrane is obstructive.
242
Color Atlas of Congenital Heart Surgery
supramitral ring tissue
pulmonary vein ceph R«-
->L
caud
FIGURE 13-10. To repair the anomaly, the fibrous membrane is excised, being careful to avoid injury to the adjacent mitral valve. The thick membrane is about 5 mm above the mitral valve annulus.
mitral valve
ceph R<-
-•L
caud
FIGURE 13-11. After the membrane is excised, the mitral valve is exposed. Now there is free communication between the left atrium and ventricle.
13
Left Atrial Obstructive Lesions 243
fibrous membrane
mitral valve apparatus ceph R«-
-•L
caud
13-12. In another patient who is on cardiopulmonary bypass, the left atrium along the right heart border is opened behind the interatrial groove. A fibrous membrane is exposed in the region of the valve annulus. Although the membrane does not protrude over most of the mitral orifice, it causes significant obstruction. FIGURE
membrane
chord and papillary muscle ceph R<-
-•L
caud FIGURE 13-13. The thin discrete membrane is intimate with the valve annulus. The mitral valve chordal apparatus is seen immediately below the membrane.
244 Color Atlas of Congenital Heart Surgery
annulus
membrane
ceph R<-
-•L
caud
13-14. The membrane is carefully excised, being careful not to injure the annulus. In some cases, the membranous tissue extends onto leaflet tissue. It can usually be teased away from the valve leaflet without injury to the latter. FIGURE
anterior mitral leaflet
annulus
ceph R«-
-•L
caud
13-15. The completed repair is seen and no supravalve membrane remains. In cases in whom the membrane is removed from valve tissue, this usually results in a more pliable and mobile leaflet. FIGURE
14
Valvular Stenosis
14-1. Valvular Stenosis
unicuspid valve
ceph R<-
-•L
caud
14-1. This photograph shows severe valvular stenosis as it occurs in a newborn. There is a unicuspid, horseshoe-shaped leaflet with a single posterior commissure. Leaflet tissue is thick, fibrous, and myxomatous. Successful valvotomy can be performed by creating a second commissure. Significant aortic regurgitation is rare due to the fibrous nature of the valve that prevents redundancy. Although typically there is residual stenosis after surgery, many infants will do surprisingly well for many years, even though the valve appears severely malformed and incompatible with life. This photograph was taken of a postmortem specimen in a child who died of other causes. FIGURE
245
246 Color Atlas of Congenital Heart Surgery
ceph R<-
-•L
caud
14-2. For comparison, this postmortem specimen shows a normal aortic valve in another newborn infant. There are three cusps and each is thin and pliable.
FIGURE
right coronary cusp
stenotic commissures
non coronary cusp
caud R<-
left coronary cusp
-•L
ceph
14-3. In this older child with valvular stenosis, the exposure is through a proximal ascending aortotomy, with cardiopulmonary bypass, aortic cross-clamping, and cardioplegia. There are three leaflets that are fibrous and thickened with stenosis primarily of two commissures. FIGURE
14
Valvular Stenosis
247
incised commissures
caud
R<-
-*L
ceph
FIGURE 14-4. Valvotomy is performed by incising stenotic commissures. In some cases, the leaflets are thinned by resecting fibrous tissue.
14-2. Subaortic Stenosis: Fibromuscular Obstruction
aortic valve
caud R<-
->L
ceph
FIGURE 14-5. Repair of this anomaly is performed with cardiopulmonary bypass and aortic clamping, cardioplegia, and profound local cardiac cooling, working through a proximal ascending aortotomy. Typically, the aortic valve is normal as seen here. There are three leaflets without commissural stenosis.
248
Color Atlas of Congenital Heart Surgery
fibromuscular ledge
caud R<-
->L
ceph
FIGURE 14-6. With retraction of the valve, a fibromuscular ledge is seen in the left lateral part of the outflow tract immediately below the valve annulus.
stitch in obstructing muscle ledge
caud R<-
-•L
ceph
FIGURE 14-7. A stitch is placed in the middle of the ledge to facilitate grasping it while the resection is carried out.
14
Valvular Stenosis
249
rectangular wedge
caud
R<-
-•L
ceph
FIGURE 14-8. A rectangular wedge of fibromuscular tissue is resected. It is safe to resect tissue as far anterior as the region beneath the middle of the right coronary cusp. The bundle of His' pierces the ventricular septum beneath the noncoronary cusp, after which the bundle moves forward in the ventricular septum to the commissure between the noncoronary and right coronary cusps. Tissue is resected to the left and posteriorly as far as the base of the anterior mitral leaflet, which is located in the posterior wall of the left ventricular outflow tract.
FIGURE 14-9. The resected specimen is seen here.
250 Color Atlas of Congenital Heart Surgery
fibrous collar
R^
FIGURE 14-10. In another child, a typical fibrous collar is seen in the outflow tract immediately below the aortic valve annulus.
area of resection
R^
FIGURE
14-11. The fibrous collar has been resected along with a wedge of muscle.
14
Valvular Stenosis 251
aortic valve
FIGURE
14-12. In another patient, a normal aortic valve is seen.
obstructing muscle ledge
caud R««-
->L
ceph
14-13. The valve cusps are retracted and a fibromuscular obstruction is seen immediately below the annulus.
FIGURE
252
Color Atlas of Congenital Heart Surgery
parallel incisions
caud R«-
-•L ceph
FIGURE 14-14. A stitch is placed in the mid part of the ledge for retraction, and parallel incisions are made in the obstructing tissue. The rightward one is below the mid part of the right coronary cusp.
muscle ledge to be resected
R«-
FIGURE 14-15. The obstructing muscle ledge is pulled into the field.
14
Valvular Stenosis 253
14-16. The resected specimen is shown. The obstruction extended deep into the sinus portion of the left ventricle, and the long resected specimen depicts the length of the obstructive process. FIGURE
area of resection
FIGURE
14-17. The area of resection is wide to ensure relief of the obstruction.
254 Color Atlas of Congenital Heart Surgery
14-2-1. Anomalous Mitral Valve Papillary Muscle
anomalous papillary muscle extension into subaortic area
fibrous extension into base of mitral leaflet
aortic valve leaf
14-18. After placing another child on cardiopulmonary bypass, an opening is made in the proximal ascending aorta. A trileafed aortic valve is retracted, as is a narrow membrane located anteriorly. An obstructing muscle mass is exposed in the posterior left ventricular outflow tract. This is an anomalous extension of the mitral valve posterior medial papillary muscle with a fibrous tissue extension into the base of the anterior mitral leaflet. FIGURE
right coronary cusp
anomalous papillary muscle extension
fibrous attachment
left coronary cusp
14-19. The fibrous membrane located beneath the right coronary cusp is excised and the anomalous muscle bundle is more easily seen.
FIGURE
14
Valvular Stenosis 255
papillary muscle excision
mitral valve chords
FIGURE 14-20. The cephalad 25% of the posterior medial papillary muscle is excised. The lower portion of this papillary muscle is left intact and is attached to normal chords. The raw surface of the muscle excision is seen.
ceph -•L
caud
papillary muscle excision
mitral valve chords
FIGURE 14-21. The raw surface of the excised muscle is again seen and normal mitral valve chords are identified. The lower 75% of the papillary muscle remains intact with these chords to provide mitral valve support. After closing the aorta and removing the child from bypass, there was no residual pressure gradient.
14-3. Modified Konno Procedure In some patients, the left ventricular outflow tract is narrow and local tissue resection alone is inadequate to relieve the obstruction. A modified Konno procedure can be used in this diverse group of patients, which includes tunnel-like stenosis, stenosis in patients after total repair of complete atrio-ventricular (AV) canal, and some cases of hypertrophic cardiomyopathy. The geometry of the outflow tract is altered by full thickness resection of the ventricular septum working through a right ventriculotomy and an aortotomy. The left ventricular outflow tract is further augmented by ventricular septal defect (VSD) patch closure, placing the patch on the right ventricular surface of the septum. If that patch encroaches on the right ventricular outflow tract, an additional patch can be placed in the ventriculotomy. When the right ventricular outflow tract is not compromised by the intracardiac patch, the repair can be performed working through an aortotomy and an adjacent right atriotomy.
256 Color Atlas of Congenital Heart Surgery
right ventricle outflow tract
aortic valve ceph R«-
-•L
caud
14-22. The child has been placed on cardiopulmonary bypass and the proximal ascending aorta opened. A normal aortic valve is identified and severe long segment subaortic stenosis seen. FIGURE
ventriculotomy
ventricular septum
ceph
R<-
-•L
caud FIGURE
tract.
14-23. A ventriculotomy is made in the region of the right ventricular outflow
14
Valvular Stenosis 257
aortotomy
tip of right angle clamp inVSD
ceph R<-
-•L
caud
14-24. An oblique incision is made in the ventricular septum starting immediately below the aortic valve and extending caudad toward the patient's left side (starting at or to the left of corpora arantii of the right coronary cusp to avoid the His' bundle). To identify this region, a right-angle clamp is passed through the aortic valve to protrude in the region of the septum to be incised. FIGURE
iatrogenic VSD
ceph R^-
-•L
caud
14-25. The large ventriculotomy is seen and through this region obstructing tissue in the left ventricle can be excised. If ventricular septal tissue is to be removed, this should be toward the patient's left in order to avoid the region of the bundle of His'. FIGURE
258
Color Atlas of Congenital Heart Surgery
VSD ceph -•L
R«-
caud
FIGURE 14-26. Multiple felted mattress sutures are placed around the right ventricular surface of the VSD.
patch
ceph R^-
-•L
caud
FIGURE 14-27. A Dacron® patch is used to close the VSD positioning the patch on the right ventricular surface of the septum.
14
Valvular Stenosis 259
14-28. The right ventriculotomy is closed with a tissue patch to enlarge the right ventricular tract; the aortotomy has been closed. FIGURE
outflow tract patch
14-4. Supravalvar Stenosis In the presence of this anomaly, there is usually a severe stenosing ring, at or immediately above the aortic valve commissures. A simple incision across the area of stenosis with patch angioplasty is often inadequate to relieve the obstruction. The stenosing ring must be incised into at least two sinuses of Valsalva. Three effective repair techniques are described.
ascending aorta
supravalvar stenosis
FIGURE 14-29. An external view of the heart shows the narrow proximal ascending aorta at the site of supravalvar stenosis.
260
Color Atlas of Congenital Heart Surgery
supravalvar stenosing ring
lower ends of inverted "Y" incision
ceph R<-
-•L
caud
FIGURE 14-30. After the cardiopulmonary bypass is established, with moderate hypothermia, aortic cross-clamping, cardioplegic arrest, and profound local cooling, an inverted Y incision is made in the ascending aorta. The stenosing supravalvar ring is seen.
area of stenosing ring aortic valve
ceph R<-
->L
caud
lower ends of inverted "Y" incision
FIGURE 14-31. The lower ends of the inverted Y incision are extended into the sinuses of Valsalva of the noncoronary and right coronary cusps, respectively.
14
Valvular Stenosis 261
stenosing ring
orifice of left coronary artery
aortic valve cusp
ceph R<-
->L
caud FIGURE 14-32. The root of the aorta is exposed after completing the inverted Y incision. The stenosing supravalvar ring is immediately above the valve commissures. Normal valve leaflets are seen, and the orifice of the left coronary artery is located just beneath the ring. In some cases coronary stenosis occurs when fibrous tissue compromises the orifice.
lower points of patch
R +
14-33. A synthetic patch is tailored so that the lower points of the patch will fit in the opened sinuses of Valsalva. FIGURE
262
Color Atlas of Congenital Heart Surgery
area of commissure
lower points of patch ceph R<-
-•L
caud
FIGURE 14-34. The completed repair is seen after the patch is stitched in place with a continuous suture.
supravalvar stenosis ceph R<-
-•L
caud
FIGURE 14-35. In another baby who has been placed on cardiopulmonary bypass, the area of supravalvar stenosis is in the typical position at the sino-tubular junction.
14
Valvular Stenosis
263
distal ascending aorta
stenosing ring
ceph R<-
->L
caud
FIGURE 14-36. The ascending aorta is divided at/or immediately above the stenosing ring. Care must be taken to avoid injury to the coronary artery orifices, which are below the ring.
left coronary artery orifice
sinus of Valsalva incisions
right coronary artery orifice
R<-
FIGURE 14-37. Incisions are made in each of the three sinuses of Valsalva from the ring to near the annulus. The proximity of the coronary artery orifices is seen.
264 Color Atlas of Congenital Heart Surgery
homograft patches
aortic valve leaflets
ceph R«-
-•L
caud
14-38. Triangular-shaped tissue patches of homograft pulmonary wall are stitched over incisions in the left and right coronary cusp sinuses. The noncoronary cusp sinus remains open and a patch will be placed here to complete this part of the repair. FIGURE
homograft patch
aortic anastomosis
ceph R<-
-•L
caud
14-39. A direct end-to end anastomosis is performed between the two aortic segments. Counter incisions in the upper aorta may be required due to the disparity in diameter of the two segments caused by adding the homograft patches. FIGURE
14
Valvular Stenosis 265
supravalvar stenosis
ceph R<«-
-•L
caud FIGURE 14-40. In another infant, after establishing cardiopulmonary bypass with aortic clamping, cardioplegia, and profound local cooling, the supravalvar stenosis is seen at the sinotubular junction.
stenosing ring
14-41. The ascending aorta has been widely dissected including the arch for mobility because the anastomosis will shorten the ascending aorta. Here, it is divided immediately above the stenotic ring. Care must be taken to avoid injury to the coronary arteries, which originate below the ring. FIGURE
266
Color Atlas of Congenital Heart Surgery
aortic valve
ceph R
-•L
caud
FIGURE 14-42. The ring is retracted to expose normal aortic valve leaflets and to view the coronary artery orifices.
sinus of Valsalva incisions
ceph R<-
-•L
caud
FIGURE 14-43. Incisions are made from the ring into each sinus of Valsalva to near the aortic valve annulus.
14
Valvular Stenosis 267
incisions in distal aortic segment
commissure ceph -•L
caud
14-44. Longitudinal counter incisions are made in the upper aortic segment, each being opposite an aortic valve commissure. FIGURE
incision in distal aortic segment
commissure
ceph R«-
-•L
caud FIGURE
14-45. Each commissure will fit in an adjacent upper aortic longitudinal incision.
268
Color Atlas of Congenital Heart Surgery
primary aortic anastomosis
ceph R<-
-•L
caud
FIGURE 14-46. A direct end-to-end anastomosis is performed between the two aortic segments. With such, the area of previous stenosis has been opened widely by the incisions into each aortic segment.
14-5. Aortic Root Enlargement Procedures 14-5-1. Posterior Root Enlargement
aortic valve cusps
caud L<-
-•R
ceph
FIGURE 14-47. With cardiopulmonary bypass, aortic clamping, and cardioplegia with profound local cardiac cooling, a proximal ascending aortotomy is made. A bicuspid stenotic and dysplastic valve is seen. After it is determined that valvuloplasty is not possible, the valve leaflets are excised.
14
Valvular Stenosis 269
anterior mitral leaflet extended aortotomy
annulus caud L<-
-•R
ceph
14-48. The aortotomy is extended caudad and posteriorly through the valve annulus to the base of the anterior mitral leaflet. With this technique, the annulus can be enlarged by up to 4 to 5 mm. FIGURE
treated pericardial patch caud L«-
-•R
ceph
14-49. Glutaraldehyde-treated pericardium is stitched over the lower aortotomy extension.
FIGURE
270 Color Atlas of Congenital Heart Surgery
pericardial patch
caud L<-
-•R
ceph FIGURE
14-50. The pericardial patch is retracted anteriorly and seen from outside the
aorta.
prosthetic valve valve stitches in patch
pericardial patch caud L««-
->R
ceph
14-51. A prosthetic valve is implanted at the level of the native aortic valve annulus, and the pericardial patch now comprises part of that annulus. To complete the repair, the remaining pericardial patch is stitched over the aortotomy. FIGURE
14
Valvular Stenosis 271
14-5-2. Konno Procedure with a Prosthetic Valve
aortic valve cusps
caud L««-
-•R
ceph FIGURE 14-52. The aortotomy is made in a longitudinal direction and to the left of the right coronary artery orifice. The severely scarred valve cusps are beyond repair, and it is decided to proceed with valve replacement and anterior root enlargement.
aortotomy
right ventriculotomy
ventricular septum
caud
14-53. An adjacent incision is made in the anterior wall of the right ventricular outflow tract to expose the septum.
FIGURE
272
Color Atlas of Congenital Heart Surgery
valve leaflet
ventricular septal incision
ceph R«-
->L caud
FIGURE 14-54. The aortic valve annulus is incised by working to the left of the right coronary orifice. The incision extends into the upper part of the ventricular septum cephalad to the His' bundle. Valve leaflets are excised in preparation for valve replacement.
patch in ventricular septal defect
ceph R««-
->L
caud
FIGURE 14-55. A woven Dacron® patch is tailored to conform to the iatrogenic VSD and adjacent aortotomy. The lower part of the patch is placed over the VSD with interrupted felted mattress sutures, placing the patch on the left ventricular surface of the septum.
14
Valvular Stenosis 273
native valve annulus
ventricular septal defect patch
ceph R<-
-•L
caud
14-56. The patch is retracted anteriorly and interrupted valve sutures are placed in the native valve annulus. Additional anterior valve sutures pass through the patch. FIGURE
prosthetic valve
R«-
14-57. After the valve orifice is sized, an appropriate prosthetic valve is stitched in place in the subcoronary position. At least 60% of the valve annulus should be placed in the native annulus. Significant enlargement of the left ventricular outflow tract is accomplished, allowing for placement of a larger prosthesis. FIGURE
274
Color Atlas of Congenital Heart Surgery
annulus of valve
patch over ventricular septal defect
ceph R<-
->L t caud
FIGURE 14-58. The upper segment of the Dacron® patch is stitched to the aortotomy with a continuous suture. Felt pledgets of valve sutures are seen and the repaired VSD is caudad to the valve annulus.
pericardial patch over right ventricular outflow area
patch covering aortic wall patch
ceph
R<-
-*L
caud
FIGURE 14-59. A pericardial patch is stitched over the right ventriculotomy and extends in continuity across the surface of the Dacron® patch. Blood that leaks from the Dacron® patch is collected beneath the pericardial patch and drains to the right ventricle.
14
Valvular Stenosis 275
14-5-3. Konno Procedure with a Homograft Valve
ascending aorta
right coronary artery
R^
FIGURE
14-60. The right coronary artery is in the usual anterior aortic root location.
aortotomy to left of right coronary artery
right ventriculotomy
aortic valve cusps
ventricular septal incision
FIGURE 14-61. A longrtudinal incision in the proximal aorta is directed to the left of the right coronary artery orifice. An adjacent right ventriculotomy is made, and the aortic valve annulus is incised, extending this incision into the ventricular septum
276 Color Atlas of Congenital Heart Surgery
mitral leaflet of homograft patch
homograft to native annulus stitch
ventricular septal
ceph R<-
-•L
caud
14-62. Native aortic valve cusps are excised. An aortic valve homograft is tailored, leaving a large patch of homograft wall anteriorly. The homograft is positioned so that its mitral leaflet is located anteriorly. The homograft is stitched to the native valve annulus with a continuous monofilament suture, placing this stitch immediately below the homograft valve annulus. FIGURE
mitral leaflet of homograft
ceph R<-
-•L
ventricular septum
caud FIGURE 14-63. The homograft mitral leaflet is used to close the VSD with interrupted felted mattress sutures. These stitches are passed from the left ventricular surface of the septum and then through the adjacent homograft mitral leaflet. Pledgets are used on both surfaces of the septum.
14
Valvular Stenosis 277 aortotomy
posterior homograft to native aorta suture
anterior wall of homograft
14-64. Homograft aortic wall is removed from two posterior sinuses of Valsalva of the graft. The central homograft commissure is in the midline, and the upper rim of the homograft is stitched to the posterior wall of the native ascending aorta. FIGURE
anterior homograft wall to native aorta suture
homograft mitral leaflet stitched to ventricular septum ceph R^-
right ventriculotomy
-*L
caud FIGURE
aorta.
14-65. The anterior homograft aortic wall is stitched to the aortotomy of the native
278 Color Atlas of Congenital Heart Surgery
anterior wall of homograft stitched to native aorta
harvested homograft patch in right ventricular outflow
R< caud
14-66. A homograft aortic wall patch that was harvested in the tailoring process is used to reconstruct the right ventricular outflow tract. The homograft aortic wall patch covers the ventriculotomy and is stitched to the anterior homograft valve annulus. FIGURE
14-5-4. Ross-Konno Procedure
aortotomy
autograft explant site in right ventricular outflow ventricular septal incision
R
14-67. After establishing cardiopulmonary bypass with moderate hypothermia, aortic clamping, cardioplegic arrest, and profound local cooling, the pulmonary autograft is harvested. The main pulmonary artery has been divided proximal to the branches and the proximal vessel with the valve was harvested from the right ventricule. A circumferential 0.5-cm muscle bar was taken with the graft. Here, the ascending aorta has been opened in a longitudinal direction, extending this excision across the aortic valve annulus into the ventricular septum. This relieves the left ventricular outflow tract obstruction. FIGURE
14
Valvular Stenosis 279
muscle ridge
autograft leaflets
ceph R<-
-•L
caud FIGURE
14-68. Prior to implantation, the autograft leaflets are inspected and found to be
normal.
annulus
marking stitches
explant site
ventricular septal defect
14-69. Aortic valve leaflets are excised, leaving a 1-mm segment of leaflet attached to the native annulus. The new left ventricular outflow tract opening is marked with three trifurcating stitches. These are placed opposite the native sinuses of Valsalva and will be inserted near the middle of each autograft sinus of Valsalva.
FIGURE
280 Color Atlas of Congenital Heart Surgery
autograft
stitches for ventricular septal defect closure ceph R<-
-•L
caud
14-70. The trifurcating stitches are placed in the proximal autograft muscle bar, opposite the mid part of each autograft leaflet. Multiple interrupted felted stitches are passed through the ventricular septum from left ventricle into right ventricle in preparation for closing the VSD. FIGURE
autograft
anterior muscle bar
VSD patch
ceph R+-
->L
caud
14-71. A Dacron® patch is placed over the VSD utilizing previously inserted stitches. The autograft will be attached to the native aortic valve annulus with multiple interrupted fine polypropylene sutures. In situ the graft fits well. The VSD patch will be attached to the anterior muscle bar with additional interrupted sutures.
FIGURE
14
Valvular Stenosis
281
native aorta
autograft
VSD patch
right ventriculotomy
R^
FIGURE 14-72. Working inside the native aorta, the posterior wall of the graft sinuses of Valsalva is tailored and then the graft is stitched to the posterior native aorta below and around the coronary orifices. The posterior graft commissure is also attached to the posterior native aorta. The anterior wall of the autograft is stitched to the opening in the native aorta.
pulmonary homograft
anterior muscle bar
ventriculotomy
ceph
R<-
-•L
caud
FIGURE 14-73. A pulmonary valve homograft with attached main pulmonary artery is used for reconstruction of the right ventricular outflow tract. Its muscle bar is stitched to the posterior right ventricle and the anterior graft muscle bar is stitched to the ventriculotomy.
282 Color Atlas of Congenital Heart Surgery
14-6. Apical Left Ventricle to Ascending Aorta Conduit In small infants, when an apical-aortic conduit is required, there may be inadequate space in which to work to attach the conduit to the descending aorta when working through a median sternotomy. Here the conduit is attached to the ascending aorta.
main pulmonary artery
ascending aorta
ceph R<-
-•L
caud
14-74. Through a median sternotomy, a small ascending aorta is seen. The procedure is performed with cardiopulmonary bypass, moderate hypothermia, aortic clamping, and cardioplegic arrest plus profound local cooling. FIGURE
left apical ventriculotomy
caud
FIGURE 14-75. A left apical ventriculotomy is made, and a button of ventricular wall is removed.
14
Valvular Stenosis 283
conduit valve
R^
14-76. A porcine-valved conduit (12 mm in this case) is beveled and stitched to the ventriculotomy with a continuous monofilament suture. The conduit valve is placed near the ventriculotomy. FIGURE
ascending aorta
ceph R<-
->L
caud
14-77. A longitudinal ascending aortotomy is made to accept the distal end of the conduit.
FIGURE
284 Color Atlas of Congenital Heart Surgery
14-78. The completed aortic anastomosis is shown and the conduit lies along the left heart border. FIGURE
15
d-Transposition of the Great Arteries
15-1. Arterial Switch: Jatene Operation Arterial switch repair is the preferred operation for d-transposition of the great arteries without severe anatomic left ventricular outflow tract narrowing or when such narrowing can be treated with resection. Other surgical repairs are rarely performed. The traditional contraindication to its use is the presence of an abnormal pulmonary valve, which would become the aortic valve after repair, although today one would consider combining a form of the Ross procedure and arterial switch operations for management of such a patient. Unusual coronary artery anatomy or intramural course of a coronary are not contraindications. Arterial switch is usually performed in the first week of life because the preparation of the left ventricle may be unpredictable after 10 to 14 days of age. If the operation is delayed, staged repair may be performed with preparatory temporary pulmonary artery banding and Blalock shunt followed by arterial switch after adequate left ventricular hypertrophy has developed. In infants with d-transposition and nonrestrictive ventricular septal defect, total repair may be delayed because the left ventricle remains prepared because of systemic pressure in this chamber. Early repair is still preferable before ventricular function is compromised. Operations are performed using cardiopulmonary bypass with moderate hypothermia, aortic clamping, cardioplegia, and profound local cardiac cooling. Bicaval cannulation is used and this allows completion of the intracardiac repair during rewarming. Success of the coronary artery transfer is more predictable in the presence of a direct anterior-posterior relationship of the great vessels (0° rotational axis) as compared to side-by-side great vessels (90° rotational axis). The coronary artery anatomy that is most advantageous for successful transfer includes a left coronary artery that arises from the left-sided sinus of Valsalva and a right coronary artery that arises from the right.
285
286 Color Atlas of Congenital Heart Surgery
ascending aorta
main pulmonary artery
left coronary artery
ceph R<-
-•L
caud FIGURE 15-1. The ascending aorta is anterior and toward the right, while the main pulmonary artery is posterior and toward the left (45° rotational axis). The main left coronary artery arises from the aortic root to the left, and it divides into the left anterior descending and circumflex coronary arteries.
right coronary artery
R^
FIGURE
15-2. The right coronary artery arises from the right aspect of the aortic root.
15
d-Transposition of the Great Arteries 287 arch
left pulmonary artery
main pulmonary artery
ceph R<-
ascending aorta
-•L
caud
15-3. Extensive circumferential dissection of the great vessels is performed and the arch freed as far distal as the isthmus. The left pulmonary artery is dissected circumferentially to the left hilum. The right pulmonary artery will be dissected in a similar way. FIGURE
innominate artery
left subclavian artery
cannula purse string stitches
R^
FIGURE 15-4. The transverse aortic arch is dissected to the isthmus, and the proximal arch vessels are freed. Marking stitches are placed on the posterior great vessel at the proposed coronary implant sites. These stitches are more accurately placed at this stage of the operation with the vessels distended and the heart beating. Purse string stitches for the aortic cannula are placed laterally in the ascending aorta at the base of the innominate artery.
288 Color Atlas of Congenital Heart Surgery
aortic cannula
FIGURE
15-5. The aortic cannula is positioned high and lateral in the ascending aorta.
patent ductus arteriosus
R-*
15-6. With initiation of cardiopulmonary bypass, the ductus arteriosus is doubly clamped and divided.
FIGURE
15
d-Transposition of the Great Arteries 289
ductal stumps
FIGURE
15-7. Each end of the ductus is closed with a continuous suture.
divided aortic segments
pulmonary artery branch snares
R^
15-8. An aortic cross-clamp is applied, and cardioplegia solution is injected. The heart is packed with ice for profound local cooling. Snares are tightened around the pulmonary artery branches for hemostasis. The aorta is divided, leaving a longer segment near the heart for later use in constructing the neomain pulmonary artery; this may help to avoid branch pulmonary artery stenosis because of less tension on the proximal branches. The upper aortic segment can be moved freely because the arch has been extensively dissected. This allows construction of the neoaorta without undue tension on the anastomosis. FIGURE
290 Color Atlas of Congenital Heart Surgery
divided main pulmonary artery
R^
FIGURE 15-9. The main pulmonary artery is divided proximal to the branches. A longer proximal pulmonary artery segment allows ample space for implantation of coronary buttons, while a larger distal pulmonary artery segment facilitates the construction of a neomain pulmonary artery anastomosis without tension.
left coronary artery button
15-10. A circular button of aorta that surrounds the left coronary artery is explanted. The top of the button is cut as an inverted V to facilitate identification; this helps to avoid twisting the button during implantation. If tissue is available, the button is excised so that its anterior portion is wider; this will help to avoid kinking after implantation. FIGURE
15
d-Transposition of the Great Arteries 291
light coronary artery button
Re-
15-11. The right coronary button is excised with a V at the top, leaving more aortic wall anteriorly, as was done with the left coronary button. FIGURE
right coronary artery button
left coronary artery button R<
15-12. The two coronary buttons hang free, and the coronary arteries are dissected only a short distance to mobilize them for the transfer to the posterior great vessel. With extensive dissection their support is removed, and there is greater chance of kinking of the coronary arteries after implantation.
FIGURE
Color Atlas of Congenital Heart Surgery
left coronary button
flap at implant site R<
FIGURE 15-13. The marking stitch that was placed previously at the site of the proposed left coronary implant site is located. A semicircular or elliptical incision is made in the wall of the posterior great vessel, resulting in a flap that is lifted medially. This flap and the wider anterior segment of the button allow the button to be implanted with less chance of kinking. The elliptical incision is made with care, observing the semilunar valve to avoid injury to valve leaflets. Ideally, the button should be rotated less than 60° degrees in the transverse plane during implantation to avoid kinking of the coronary artery.
flap at right coronary implant site
ceph R<-
-•L
caud
15-14. A semicircular or elliptical incision is now made in the anterior wall of the posterior great vessel to accept the right coronary button. The base of the flap is medial, and this helps to avoid kinking of the coronary artery after the button is shifted posteriorly in the transverse plane for the anastomosis. The wider rim on the anterior surface of the button also helps to avoid kinking. FIGURE
15
d-Transposition of the Great Arteries 293
implanted right coronary artery button
1-mm probe ceph R<-
-•L
caud FIGURE 15-15. The right and left coronary buttons are stitched to respective implant sites with continuous 7/0 monofilament suture. The V at the top of the button is carefully positioned at the top of the implantation site to avoid rotation or twisting of the button on the axis of the coronary artery. Here, the patency of the right coronary artery is checked by passing a 1-mm probe into the vessel.
right coronary artery
left coronary artery coronary explant sites
R^
FIGURE 15-16. Implantation of both buttons is complete without twisting them or the coronary arteries. The implant sites in the cephalad caudad plane are chosen to result in appropriate tension (not too loose, not too tight) on the coronary arteries to help prevent kinking. The coronary buttons have been shifted less than 60° in the anterior posterior or transverse plane and this also avoids coronary artery kinking.
294 Color Atlas of Congenital Heart Surgery
left coronary artery button
right coronary artery button
R^
15-17. In another patient, an alternative method of coronary button explantation is used. The right button is taken in circular fashion, while the left button is explanted with a larger cephalad segment of aortic wall. The latter is performed by incising the aorta, starting in the transsection rim of the aorta. FIGURE
coronary buttons
implant sites
R^
15-18. The implant sites in the posterior great vessel are prepared by incising the vessel from its free margin. The coronary buttons are positioned for implantation. FIGURE
15
d-Transposition of the Great Arteries 295
left coronary artery button
ceph R<-
->L
caud FIGURE
15-19. The left coronary button is implanted with a continuous suture.
right coronary artery button
FIGURE
15-20. The right coronary button is implanted with a continuous suture.
296 Color Atlas of Congenital Heart Surgery
main pulmonary artery left coronary artery button right coronary artery button aortic valve
R^
15-21. In another infant, rectangular coronary buttons are explanted with incisions made from the transsection margin of the divided aorta.
FIGURE
neoaorta
left coronary artery
right coronary artery
ceph R«-
-•L
caud
15-22. Each button is implanted in the posterior great vessel in respective incisions made from the transsection margin of the vessel.
FIGURE
15
d-Transposition of the Great Arteries 297
distal main pulmonary artery distal ascending aorta
proximal posterior great vessel
proximal anterior great vessel
ceph -•[_ r>.##
caud
•* %
15-23. After the coronary buttons are implanted, a Le Compte maneuver is performed by shifting the distal ascending aorta posterior to the bifurcation of the main pulmonary artery. The neoaorta is constructed by end-to-end anastomosis of the upper aorta and the proximal posterior great vessel or neoaorta, which now contains the coronary arteries. FIGURE
distal ascending aorta
proximal neoaorta
15-24. The posterior neoaorta is constructed with a continuous suture while working within the great vessel. FIGURE
298 Color Atlas of Congenital Heart Surgery
distal main pulmonary artery neoaorta
left coronary artery
right coronary artery R^
15-25. Neoaorta construction is complete after a continuous suture is placed in the anterior part of the anastomosis.
FIGURE
distal ascending aorta
orifice of main right and left coronary arteries
15-26. In another child, the main right and left coronary arteries arise from a single posterior aortic sinus of Valsalva. This is confirmed after dividing the aorta. FIGURE
15
d-Transposition of the Great Arteries 299
button with right and left coronary arteries
FIGURE
15-27. A single button containing both coronary arteries is explanted.
proximal neoaorta
coronary button
FIGURE 15-28. The button is moved posteriorly and only the upper margin of the button is stitched to the anterior rim of the proximal posterior great vessel or neoaorta. The distal ascending aorta is then moved behind the pulmonary artery bifurcation and anastomosed end-to-end to the posterior great vessel, leaving a large anterior opening to be used in the construction of a tunnel to the coronary arteries.
300 Color Atlas of Congenital Heart Surgery FIGURE 15-29. A pericardial patch is placed over the remaining opening in the neoaorta and around the coronary button to construct a tunnel for coronary flow. neoaorta suture line
pericardial patch of coronary button tunnel
>L
anterior great vessel divided high counter incision near valve
pericardial coronary tunnel
R^
15-30. In another child, the main right coronary artery and the left anterior descending coronary arise anteriorly from the ascending aorta with orifices near each other in a common sinus of Valsalva. It is not possible to shift these arteries to the posterior great vessel in a traditional way. By working through a counter incision in the aorta immediately above the valve, 4-mm adjacent openings are made in the great vessels and are stitched together to create an aorto-pulmonary (A-P) window immediately above the semilunar valves. A pericardial patch is placed within the anterior great vessel to construct a tunnel for coronary flow between the aorto-pulmonary window and the anterior coronary orifices. The circumflex coronary arose to the right and was explanted as a single button and then implanted in the right base of the posterior great vessel. FIGURE
15
d-Transposition of the Great Arteries 301
area of A-P window
pericardial tunnel
area of left descending and right coronary arteries
R^
15-31. The aorto-pulmonary window is located beneath the upper end of the pericardial patch tunnel, while the coronary orifices are at the lower end of the tunnel. FIGURE
15-32. After the neoaorta has been constructed, fibrin glue is injected over the posterior suture line. FIGURE
302 Color Atlas of Congenital Heart Surgery
15-33. Fibrin glue is also injected around the anterior neoaorta and coronary anastomoses.
FIGURE
neoaorta
parietal band
ceph R<-
->L
caud
15-34. In many cases, the parietal band in right ventricle infundibulum is prominent. This is routinely resected by working through the valve of the anterior great vessel, thereby avoiding postoperative infundibular muscle obstruction that may alter hemodynamics. FIGURE
15
d-Transposition of the Great Arteries 303
bifurcation of pulmonary artery
coronary explant sites
proximal anterior great vessel
15-35. In preparation for construction of the neomain pulmonary artery, coronary button explant sites will be closed with untreated circular pericardial autografts. Alternatively, homograft pulmonary artery wall patches may be used.
FIGURE
distal main pulmonary artery
circular pericardial patch in right coronary explant site
caud
15-36. The neomain pulmonary artery is constructed with an end-to-end anastomosis. Extensive dissection of the pulmonary artery branches before repair helps to avoid tension on the branches after completion of the repair; this is important in avoiding stenosis of the proximal right and left pulmonary arteries. If there is narrowing of one or both proximal branches, a pericardial patch is placed in the anterior neomain pulmonary artery and across the proximal stenotic branch. FIGURE
304 Color Atlas of Congenital Heart Surgery
distal pulmonary artery
proximal neomain pulmonary artery
R^
FIGURE 15-37. Construction of the neomain pulmonary artery is complete and is accomplished by simple end-to-end anastomosis when the great vessels are in a direct anterior-posterior plane. When the great vessels are obliquely positioned or are side by side, it may be necessary to close the left aspect of the distal main pulmonary artery and implant the proximal anterior great vessel in the rightward aspect of the distal main pulmonary artery and/or the right pulmonary artery.
homograft patch
posterior commissure posterior sinuses of Valsalva
anterior wall of native vessel
caud
15-38. When rectangular coronary buttons are explanted from the anterior great vessels or when circular explant sights are large, deficiencies in both posterior sinuses of Valsalva are large. A single pentaloon-shaped patch of homograft pulmonary wall is used to construct the posterior wall of the neo main pulmonary artery. Here, the patch has been stitched into the sinuses and surrounds the previously free hanging posterior commissure. FIGURE
15
d-Transposition of the Great Arteries 305
neoaorta
.'0? ' *:'v' **^r
'w^life,,»i«giil>'
V -tilti
homograft patch
'WPP'
ceph R<
>L caud
15-39. The homograft patch has been attached to the neomain pulmonary artery and is retracted anteriorly to show its relationship to the neoaorta.
FIGURE
homograft patch
anterior part of native vessel
ceph
R<-
-•L
caud FIGURE 15-40. This patch is moved cephalad for anastomosis to the posterior distal main pulmonary artery and branches. The patch will be tailored prior to placing sutures.
306 Color Atlas of Congenital Heart Surgery
anterior suture line of neomain pulmonary artery
ceph R«-
-•L
caud
15-41. The anterior suture is placed between the proximal native vessel wall and the distal main pulmonary artery. FIGURE
neoaorta
right pulmonary artery
left pulmonary artery
neomain pulmonary artery R^
FIGURE 15-42. The completed arterial switch repair is seen. There is no tension on the proximal right and left pulmonary arteries.
15
d-Transposition of the Great Arteries
307
FIGURE 15-43. In most cases, the sternum is not closed and a large SILASTIC® patch is stitched to the skin as a dressing. More recently, the patch is simply glued to the skin with Dermabond®. This is a useful technique in the presence of mediastinal edema when the closed sternum may press on the cardiac structures and affect hemodynamics. The same benefit may be seen in children who require a high positive end-expiratory lung pressure, which results in undue pressure on cardiac chambers initially following surgery. Delayed sternal closure is usually carried out 1 to 4 days later after appropriate diuresis and/or stabilization of hemodynamics. ceph R<-
->L
caud
15-2. Rastelli Operation This procedure is used in the correction of d-transposition of the great arteries associated with a ventricular septal defect (VSD) and pulmonary stenosis. By working through the right ventricle, a patch is placed over the VSD and the aortic valve to create a tunnel to establish flow from the left ventricle through the VSD and into the aorta. The procedure is usually not performed in small infants, because an adequate working area is needed for the extensive tunneling procedure in the right ventricle.
right ventriculotomy
FIGURE 15-44. An oval segment of right ventricular wall is removed when the high right ventriculotomy is performed. This maneuver is always performed when an extracardiac conduit is attached to the right ventricle because it helps to avoid conduit inflow obstruction.
>L
308 Color Atlas of Congenital Heart Surgery
position of aortic valve
ventricular septal defect
FIGURE
15-45. The high VSD is exposed.
patch
ceph R<-
-•L
caud
15-46. A knitted Dacron® patch is stitched over the VSD and then cephalad over the aortic valve with interrupted felted mattress sutures. The patch is redundant to avoid left ventricular outlet obstruction in the new tunnel that connects the left ventricle to the aortic valve. FIGURE
15
d-Transposition of the Great Arteries
309
distal pulmonary artery
position of conduit valve
ceph -•L
R<-
caud
FIGURE 15-47. The proximal main pulmonary artery has been divided and the proximal end stitched closed. A porcine-valved Dacron® conduit is chosen for this operation, and the distal end is stitched distally to the pulmonary arteries. Currently, a cryopreserved homograft conduit is used if available.
ventriculotomy
ceph R<«-
-•L
caud
FIGURE 15-48. The ventriculotomy is inspected to be sure the orifice is of adequate size to accept the conduit.
310 Color Atlas of Congenital Heart Surgery
position of conduit
valve
15-49. The proximal conduit is stitched to the ventriculotomy. The valve in the conduit is distal and near the pulmonary arteries; this allows the rigid valve ring to more readily fit behind the sternum when the chest is closed. Sternal closure should cause minimal compression on the remaining conduit. FIGURE
ventricular septal defect
R^
FIGURE
15-50. In another patient, a high right ventriculotomy is made to expose the VSD.
15
d-Transposition of the Great Arteries 311
patch tunnel
ceph R<-
-•L
caud FIGURE 15-51. Using felted mattress sutures, a Dacron® patch is placed over the VSD and the aortic valve to create a tunnel for left ventricular flow into the aorta.
proximal main pulmonary arteriotomy
R^
15-52. A large arteriotomy is made in the main pulmonary artery. There is atresia of the valve and hypoplasia of the annulus.
FIGURE
312 Color Atlas of Congenital Heart Surgery
homograft to pulmonary artery anastomosis
proximal homograft R^
15-53. An aortic homograft is used in this repair. The posterior anastomosis is constructed at the ventriculotomy and the graft is tailored for appropriate length. It is then stitched distally to the pulmonary arterial opening. FIGURE
homograft
mitral leaflet of graft
ceph R<-
-•L
caud
15-54. The proximal homograft is stitched to the anterior ventriculotomy using the mitral leaflet of the graft as a hood at the proximal anastomosis. This allows a natural curve of the conduit after implantation and avoids distortion of the homograft valve annulus, which could result in valvular insufficiency. FIGURE
15
d-Transposition of the Great Arteries 313
distal main pulmonary artery ascending aorta proximal main pulmonary artery
15-55. In another patient with pulmonary stenosis, the pulmonary artery is divided and the proximal end is closed while working behind and to the right of the transposed ascending aorta.
FIGURE
ascending aorta
pulmonary artery
ceph
4 i
-•L
caud
15-56. The distal main pulmonary artery and proximal branches are mobilized and shifted to the left of the ascending aorta.
FIGURE
Color Atlas of Congenital Heart Surgery
pulmonary homograft
R^
FIGURE 15-57. A pulmonary homograft is used in this repair and the posterior rim of the proximal graft is stitched to the posterior ventriculotomy. The aortic cross-clamp is removed and the heart is allowed to fill. This allows more accurate tailoring of the graft length. The distal anastomosis is constructed with a continuous suture.
Gore-Tex patch hood
patch over ventricular septal defect
ceph R<-
-•L
caud
15-58. To create a hood for this graft at the proximal anastomosis, a Gore-Tex® patch is stitched to the anterior rim of the homograft near the annulus. FIGURE
15
d-Transposition of the Great Arteries 315
Gore-Tex hood
R^
FIGURE
15-59. The hood is then stitched to the lower margin of the ventriculotomy.
pulmonary homograft
Gore-Tex hood
ceph R<-
-•L
caud
15-60. After the repair is completed, the homograft is allowed to fill to check for redundancy of the graft. The Gore-Tex® hood lengthens the lower part of the graft to prevent distortion of the graft valve annulus. FIGURE
316 Color Atlas of Congenital Heart Surgery
15-3. Kawashima Operation In the presence of d-transposition of the great arteries, VSD, and side-by-side relationship of the great vessels, when arterial switch procedure is not chosen, the Kawashima operation may be used to achieve the correction. This places the left ventricle in the systemic circulation by the placement of a large commashaped patch in the right ventricle.
ascending aorta
main pulmonary artery
ceph
R<-
->L
caud FIGURE
15-61. The great vessels are located side by side.
ventriculotomy
conus muscle
R<
15-62. A high right ventriculotomy is made, and conus muscle is resected to establish an unobstructed route for blood flow from the VSD to the aortic valve. FIGURE
15
d-Transposition of the Great Arteries 317
position of aortic valve
ventricular septal defect
Re-
15-63. The VSD is seen. Multiple interrupted felted mattress sutures are placed around its rim and then along the tract that leads to the aortic valve.
FIGURE
posterior of pulmonary valve
patch tunnel
ceph R«-
-•L
caud FIGURE 15-64. A segment of an opened tubular Dacron® conduit is used for the tunnel patch. Care must be taken to avoid narrowing of the tunnel, which may cause left ventricular outlet obstruction.
318 Color Atlas of Congenital Heart Surgery
mam pulmonary artery
outflow tract patch
ceph A
R < \ > L caud
15-65. The right ventricular outflow tract is enlarged with a patch to avoid right heart obstruction secondary to the large intraventricular baffle.
FIGURE
16
1-Transposition of the Great Arteries
Although the circulation is normal in this anomaly, surgical repair of associated lesions may be necessary. Ventricular septal defect (VSD) and/or pulmonary stenosis is common, and technical aspects of repair of these problems are unusual because of differences in ventricular morphology. The double-switch operation is also gaining popularity in order to place the morphologic left ventricle in the systemic circulation, and the morphologic right ventricle in the pulmonary circulation.
16-1. With a Ventricular Septal Defect and Pulmonic Stenosis
ascending aorta
main pulmonary artery systemic ventricle right coronary artery
pulmonary ventricle
FIGURE 16-1. The ascending aorta is anterior and toward the left, while the main pulmonary artery is posterior and toward the right. The right coronary artery is located on the pulmonary ventricle outflow tract.
319
320
Color Atlas of Congenital Heart Surgery
pulmonary ventricular outflow tract mitral valve
ceph R<-
->L •
caud
FIGURE 16-2. Looking through a right atriotomy the right-sided mitral valve is retracted. The pulmonary ventricular outflow tract is narrow because of fibrous tissue located anteriorly and toward the left. This tissue cannot safely be resected, because the His' bundle is located anteriorly, the ventricular septum is located toward the patient's left, the mitral valve annulus is located toward the right, and the back wall of the heart is posterior.
ventricular septal defect
Bicaud FIGURE 16-3. A high VSD is exposed.
16
1-Transposition of the Great Arteries 321
ventricular septal defect outflow tract
R<*
16-4. The VSD is closed with a Dacron® patch, placing the stitches on the systemic ventricular surface of the septum near the defect and remote from the His' bundle, which is found on the pulmonary ventricular surface of the septum. The adjacent outflow tract is seen. Alternatively, I have repaired a high VSD working through a left atriotomy, because repair stitches can more easily be placed far from the His' bundle. FIGURE
FIGURE 16-5. To repair the outflow tract obstruction in this child, a valved extracardiac conduit is placed. A high ventriculotomy is made in the pulmonary ventricle.
322 Color Atlas of Congenital Heart Surgery
conduit
FIGURE 16-6. A porcine-valved Dacron® conduit is placed between the ventriculotomy and the main pulmonary artery and to the right of the ascending aorta. Today, I probably would use a homograft-valved conduit.
ascending aorta
main pulmonary artery
right coronary artery
R<-
16-7. In another patient, 1-transposition is present. The right coronary artery is located on the pulmonary ventricle outflow tract. FIGURE
16
1-Transposition of the Great Arteries 323
His' bundle
ventricular septal defect
mitral valve leaflet
caud FIGURE 16-8. The right atrium is opened, and the right-sided mitral valve is retracted. The His' bundle is seen on the pulmonary ventricle surface of the septum and courses along the cephalad and anterior rims of the VSD.
His' bundle
R+
FIGURE
16-9. Another view of the VSD shows the more caudad course of the His' bundle.
324 Color Atlas of Congenital Heart Surgery
mitral valve leaflet
ventricular septal defect
ceph R<-
-•L
caud
16-10. Stitches to repair the VSD are placed on the systemic ventricular surface of the septum, working through the septal defect.
FIGURE
patch
His' bundle
ceph R<-
-•L
caud FIGURE
16-11. Stitches are placed in a Dacron® patch and tied.
16
1-Transposition of the Great Arteries 325
pulmonary ventricle outflow tract mitral valve
ceph R<-
-•L
caud FIGURE
16-12. The narrow pulmonary ventricle outflow tract is seen.
cardiac muscle mass
ventricular septal defect patch pulmonary valve
distal main pulmonary artery
caud L««-
-•R
ceph
16-13. To relieve the outflow obstruction tract, the main pulmonary artery is opened in a spiral fashion. The incision starts in the distal pulmonary artery anteriorly and extends toward the heart, around the right side of the pulmonary artery, and then into the pulmonary valve annulus posteriorly. The posterior annular incision avoids the anteriorly positioned His' bundle.
FIGURE
326 Color Atlas of Congenital Heart Surgery
16-14. A spiral synthetic patch is stitched over the opening in the pulmonary artery to enlarge the outflow tract.
FIGURE
16-2. Double-Switch Operation When a VSD is present, the double-switch operation is becoming popular for anatomic repair of corrected transposition. With both a venous switch procedure and a ventriculoarterial connection switch, the morphologic left ventricle is placed in the systemic circulation and the morphologic right ventricle in the pulmonary circulation. I prefer the Senning operation for the venous switch over the Mustard procedure in that the former uses natural landmarks to position various suture lines. This accounts for a simpler procedure with more predictable venous pathways, especially in small patients. Living tissue is utilized, which should allow for growth of these venous pathway tissues. In 1-transposition of the great arteries (1-TGA), the semilunar valves are shifted to the right and may encroach on the new superior vena caval tunnel. The pericardial patch may be made wider here to enlarge that tunnel and avoid obstruction to venous blood return, which is an advantage of using Mustard's operation. In changing the ventriculoarterial connection, either a classic arterial switch procedure (see Chapter 15) or a Rastelli operation (see Chapter 15) is utilized, depending on intracardiac anatomy. Because the double switch is a long complex operation, it is generally reserved for patients beyond infancy. Cardiopulmonary bypass with bicaval cannulation, aortic clamping, cardioplegic arrest, and profound local cardiac cooling are utilized.
16
1-Transposition of the Great Arteries 327
16-1 -1. Senning Procedure
inferior vena cava
R^
FIGURE 16-15. A right atrial incision is made from the base of the appendage along the posterior and lateral wall about 5 mm anterior to the sulcus terminalis to near the mid part of the eustachian valve.
right pulmonary veins
left atriotomy
16-16. A left atrial incision is made from the upper part of the right upper pulmonary vein to the lower margin of the right lower pulmonary vein immediately behind the interatrial groove. No effort is made to make this incision deep in the interatrial groove; rather it is made at a convenient spot near the groove. This leaves more left atrial endocardial tissue attached to the atrial septum, which will be used as part of the atrial septal flap.
FIGURE
328 Color Atlas of Congenital Heart Surgery
endocardial incision
R^
16-17. An atrial septal flap is developed by incising the anterior septum in a direction parallel to the tricuspid valve annulus, working cephalad up to the back wall of the left atrium. The incision is then directed laterally passing beneath the superior cava to the upper part of the right upper pulmonary vein. This incision ends at the lateral aspect of the superior vena cava. The endocardium has been incised and muscle beneath this will then be incised. When the nodal artery is seen in this part of the atrial septum, an attempt is made to dissect that artery away from the incision to preserve its integrity. FIGURE
flap of atrial septum beneath caval atrial junction
ceph R<-
-•L
caud
16-18. The transverse septal incision beneath the superior vena cava is complete. A redundant flap of atrial septum remains beneath the caval atrial junction. Much of the tissue in the later area is excised in order to avoid obstruction of the new caval tunnel at this point. FIGURE
16
1-Transposition of the Great Arteries 329
atrial septal flap
ceph R<-
-•L
caud FIGURE 16-19. A free atrial septal flap now hangs from the lateral wall of the right atrium. This flap will be used to construct the floor of the new caval tunnels.
right atrial surface
left atrial surface
ceph
R<-
-•L
caud
16-20. To enlarge the flap, it is filleted by unfolding the left atrial endocardial surface; this also accomplishes thinning of the septal flap, which may avoid obstruction to left pulmonary vein flow behind the flap after completion of the repair. FIGURE
330 Color Atlas of Congenital Heart Surgery
atrial septal flap
ceph R<-
-•L
caud FIGURE
16-21. The filleted flap here is large enough for the repair.
atrial septal rim beneath superior vena cava
augmenting pericardial patch
ceph R<-
-•L
t caud
16-22. If the flap is too small after filleting, it is enlarged by stitching a pericardial patch in the deficient area. Excess muscle tissue in the posterior atrial wall beneath the superior vena cava is excised to avoid a ridge of tissue that may partially obstruct flow through the new caval tunnel. Excess endocardium may be excised for the same reason. The back wall of the left atrium is probed to identify perforations that may have been created during this resection. If perforations are found, the defects are closed with stitches placed in the epicardium working within the atrium. The atrial endocardium in this region is approximated with interrupted simple sutures. A continuous stitch is not used here because the purse string effect of such may partially constrict the new tunnel. FIGURE
16
1-Transposition of the Great Arteries 331
superior vena cava atrial septal rim
septal flap stitches
ceph R<-
-•L
t caud
16-23. The floor of the new caval tunnels is constructed by lowering the atrial septal flap in the atrium posterior to the plane of the previous atrial septum. Using a fine monofilament stitch and starting directly anterior to the left pulmonary veins, the septal flap is stitched to the back wall of the left atrium behind the rim of previous atrial septum. This stitch ends at the cephalad base of the septal flap beneath the superior vena cava. The caudad portion of the flap is stitched to the lower back wall of the left atrium in a similar fashion. FIGURE
superior or cephalad stitch
sulcus terminalis anterior rim of atrial septum posterior rim of right atrial wall inferior stitch
caud
16-24. The roof of the new caval tunnels is constructed by stitching the posterior margin of the right atriotomy to the anterior rim of atrial septum, starting this suture inferiorly. If the eustachian valve is prominent and intact, it is incorporated in the suture line. A second suture is placed at the cephalad end of the sulcus terminalis and is used to construct the upper part of the tunnel. The sulcus terminalis is used for this stitch rather than placing it in the margin of the atriotomy. This maneuver allows the margin of the upper end of the posterior rim of the right atriotomy to remain exterior to the caval tunnel suture line. This exteriorized margin will be used in a later suture line to avoid placing stitches in or across the sinoatrial (SA) node. FIGURE
332
Color Atlas of Congenital Heart Surgery
superior vena caval tunnel
sulcus terminalis
inferior vena caval tunnel R<
FIGURE 16-25. Construction of the roof of the caval tunnels is now complete. The coronary sinus remains in the system circulation.
right upper pulmonary vein
FIGURE 16-26. The opening in the left atrium is somewhat small and elliptical, which increases the likelihood of obstruction in the pulmonary venous pathway. A longitudinal incision is made in the right upper pulmonary vein.
16
1-Transposition of the Great Arteries 333
right lower pulmonary
ceph R<-
-•L
caud
16-27. The left atriotomy is enlarged further with a longitudinal incision in the right lower pulmonary vein.
FIGURE
counter incisions in right atrial wall
16-28. The anterior margin of the right atriotomy will be stitched to the lateral margin of the left atrial opening in front of the right pulmonary veins. The length of each margin is carefully measured. If the anterior atrial wall at the right atriotomy is inadequate, this margin is lengthened with longitudinal counter incisions at the base of the appendage and inferiorly near the inferior cava, respectively.
FIGURE
334 Color Atlas of Congenital Heart Surgery
SA node region rim of atriotomy
stitch across superior vena cava
16-29. A continuous monofilament suture is used to start a stitch at the upper part of the right upper pulmonary vein, attaching the anterior right atrium across the external surface of the superior vena cava. In the SA node region, the posterior rim of the original right atriotomy is free for use in this suture line and avoids placement of stitches in the node. FIGURE
completed trans superior caval stitch
R< caud
16-30. The trans-superior vena caval suture is complete and the first part of the pulmonary venous pathway has been constructed. Care has been exercised to avoid purse stringing this suture, which might result in obstruction in the superior vena caval tunnel. FIGURE
16
ceph R*<-
1-Transposition of the Great Arteries 335
trans inferior caval stitch
-•L
caud FIGURE 16-31. A second continuous suture is placed from the lower part of the right lower pulmonary vein across the surface of the inferior vena cava to attach the caudad end of the anterior margin of the right atriotomy to the epicardium in this area.
right ventricular vent
rim of left atrium at pulmonary veins
anterior rim of right atrium
16-32. Interrupted sutures are placed to attach the mid part of the anterior rim of the right atriotomy to the anterior wall of the left atrium in front of the right pulmonary veins. A right ventricular vent on suction is placed through the anatomic right atrial appendage and the right sided mitral valve. FIGURE
336 Color Atlas of Congenital Heart Surgery
right sided ventricular vent
pulmonary vein to atrium stitches ceph R<«-
-•L
caud
16-33. The interrupted sutures are tied to complete construction of the pulmonary venous channel. Augmentation of this channel or the new left atrium is rarely necessary when the technique of incising the anterior right pulmonary veins (see Figure 16-26, Figure 16-27) is used and when care is taken to ensure a generous length of the anterior rim of the right atriotomy (see Figure 16-28). FIGURE
atrial pressure monitoring catheter
16-34. A catheter for monitoring the pulmonary venous atrial pressure is later placed through the vent site. FIGURE
16
1-Transposition of the Great Arteries
337
16-1-2. Mustard Procedure
caval purse strings
ceph R«-
-•L
caud
FIGURE 16-35. For venous cannulation, purse string stitches are placed in the superior and inferior venae cavae, respectively, so that these cannulae will not encroach on the atrium and the proposed intra-atrial suture lines.
atrial septal defect
FIGURE 16-36. After the child has been placed on cardiopulmonary bypass at moderate hypothermia, cardioplegic arrest is established with profound local cardiac cooling. A right atriotomy is made. The atrial septal defect (ASD) and surrounding atrial septal tissue are exposed.
338 Color Atlas of Congenital Heart Surgery
atrial septum excision site
left pulmonary vein
ceph R<-
->L
caud
16-37. All atrial septal tissue is excised. The area beneath the superior vena caval orifice in particular should be thinned because a ridge here may cause obstruction in the new superior caval tunnel. The endocardium is closed with fine interrupted sutures, avoiding the use of a continuous suture that might create a purse string effect, resulting in narrowing of the new pulmonary venous channel. Left pulmonary veins are seen. FIGURE
right pulmonary veins
R^
FIGURE
16-38. The right pulmonary veins are exposed and are in a normal position.
16
1-Transposition of the Great Arteries 339
waist
crotch
legs
16-39. A previously harvested pericardial autograft is cut in the shape of a pair of trousers. The waist of the trousers will be attached to the atrial septum in front of the left pulmonary veins, while the crotch of the trousers will be stitched immediately anterior to the confluence of the left pulmonary veins. Each leg of the trousers will form a superior or inferior venal caval tunnel, respectively. FIGURE
superior vena caval tunnel
waist
crotch
R^
FIGURE 16-40. The repair starts with a continuous suture in the crotch of the pericardial patch at the junction between the left upper and left lower pulmonary veins. The suture is placed at the orifice of the veins, but not deep in the veins. The deep suture line is constructed along the superior caval tunnel, which is placed around the left upper pulmonary vein, then extended deep or posterior to the plane of the atrial septum pointing toward the back wall of the lateral aspect of the superior caval orifice. The anterior part of this tunnel is constructed with a separate stitch placed in the mid part of the waist of the patch, which is attached to the free margin of the atrial septal remnant anteriorly. This stitch then is placed more on the right atrial surface of the atrial septum, extending toward the medial part of the superior caval orifice. The end of the trousers leg is then stitched in front of the caval orifice. The inferior caval tunnel is constructed in a similar fashion. Here, the superior caval tunnel has been completed and work is starting on the inferior caval tunnel.
340 Color Atlas of Congenital Heart Surgery
leg of patch for inferior vena caval tunnel
ceph R«-
-•L
caud FIGURE 16-41. The trousers leg segment of the patch to be used for the inferior caval tunnel is lifted out to expose the orifice of the completed superior caval tunnel.
superior vena caval tunnel channel for pulmonary veins inferior vena caval tunnel
R^
16-42. The pericardial patch is in place. There is some redundancy of the patch over the caval tunnels, which results in unrestricted patency of these tunnels.
FIGURE
16
1-Transposition of the Great Arteries 341
left pulmonary vein channel
R<-
16-43. The new pulmonary venous channel is probed to be sure that it is not compromised by the pericardial patch.
FIGURE
inferior vena caval tunnel R^
16-44. Each caval tunnel is checked by individually releasing a caval tourniquet and partially occluding the respective cannula. This forces blood through the new caval tunnel to expand it. The caval channels are constructed so that there is no obstruction of same, however, the pericardial patch should not be so redundant so as to compromise the pulmonary venous channel.
FIGURE
342 Color Atlas of Congenital Heart Surgery
redundant tunnel tissue
ceph R<-
-•L
caud FIGURE 16-45. In this patient, the inferior caval tunnel patch is redundant and may compromise the pulmonary venous channel.
elliptical excision
R<
16-46. The redundancy is repaired by excising transversely an elliptical segment of the patch, which is then closed transversely with a continuous suture.
FIGURE
17
Truncus Arteriosus
Because of the direct aorta to pulmonary artery connection in this anomaly, pulmonary artery hypertension and excessive pulmonary blood flow are present from birth, necessitating total corrective surgery immediately after establishing the diagnosis and preferably in the first month of life.
17-1. Valved Conduit Repair Operations are carried out with cardiopulmonary bypass, moderate hypothermia, aortic clamping, cardioplegia, and profound local cardiac cooling. In most cases, repair is performed with a valved conduit placed in the right of the heart; otherwise residual pulmonary hypertension that may be present after surgery may result in significant pulmonary regurgitation and right heart failure. 17-1-1. Repair with Porcine-Valved
Conduit
These conduits are readily available in various sizes; however, the Dacron® tube is stiff and more difficult to use in anastomoses with the delicate structures present in infants. These grafts are alternatives to homograft conduits, as are other heterograft prostheses.
343
344 Color Atlas of Congenital Heart Surgery
ascending aorta
left pulmonary artery
truncal vessel ceph R«-
-•L
caud
17-1. The pulmonary arteries arise from the left lateral and posterior aspect of the truncal vessel.
FIGURE
pulmonary arteries
aortotomy
17-2. The side-by-side pulmonary arteries are detached with a rim of posterior aortic wall.
FIGURE
17
Truncus Arteriosus 345
aortic suture line
ceph R<-
-•L
caud FIGURE
17-3. A primary transverse closure of the aorta is performed.
ventricular septal defect
R<
17-4. A high right ventriculotomy is made, and the ventricular septal defect (VSD) is exposed.
FIGURE
Color Atlas of Congenital Heart Surgery
patch
17-5. The VSD is closed with a knitted Dacron® patch using a continuous monofilament suture, starting at the posterior inferior corner of the defect. The patch is stitched to the anterior rim of the aortic valve annulus, which overrides the ventricular septum. A continuous suture can be placed more rapidly as compared to interrupted sutures. FIGURE
pulmonary arteries
conduit
ceph R<-
-•L
caud
FIGURE 17-6. A porcine-valved Dacron® conduit is stitched to the orifice of the pulmonary arteries.
17
Truncus Arteriosus 347
conduit valve
ceph R<-
-•L
caud FIGURE 17-7. The conduit is tailored, cutting short the distal end so that the conduit valve will be located distally near the pulmonary arteries.
excision of muscle
R^
17-8. An ellipse of the lower margin of the ventriculotomy is excised to establish an unobstructed pathway to the conduit after it is stitched to the ventriculotomy.
FIGURE
348
Color Atlas of Congenital Heart Surgery FIGURE 17-9. The lower end of the
conduit is stitched to the right ventriculotomy. While the stent of the valve portion of the conduit is rigid, it fits comfortably behind the sternum when the valve is more distal.
conduit valve
>L caud
17-1-2. Aortic Homograft-Valved
Conduit
This is the preferred conduit for use in the repair of truncus arteriosus because of improved hemostasis at suture lines. The aortic homograft seems to have an advantage over the pulmonary homograft, because the former may more readily withstand the higher pressure that may exist in the right heart in some infants after this operation. Although aortic grafts may calcify more readily, this is of less consequence because all conduits placed in infants require early or intermediate replacement due to patient growth.
left pulmonary artery ascending aorta
truncal vessel
caud
FIGURE 17-10. In this infant the pulmonary arteries arise from the base of the truncal vessel.
17
Truncus Arteriosus 349
ascending aorta
left atrium
ceph R<-
-•L
t caud
17-11. The left atrium is distended as a result of excessive pulmonary blood flow and increased blood return to the left atrium.
FIGURE
right pulmonary artery
ceph R«-
->L
caud
17-12. The large right pulmonary artery arises from the posterior aspect of the ascending aorta.
FIGURE
350 Color Atlas of Congenital Heart Surgery
left pulmonary artery right pulmonary artery
aortotomy ceph R<-
-•L
caud FIGURE
17-13. The pulmonary arteries are explanted from the truncal vessel.
pericardial patch
ceph R<-
-•L
caud
17-14. Because of the large size of the aortic opening at the explant site, it cannot be closed primarily for fear of distorting the aortic valve. To reconstruct the aorta, a gluteraldehyde-treated pericardial autograft is used as a patch. FIGURE
17
Truncus Arteriosus 351
pericardial patch
ventricular septal defect
truncal
valve annulus ceph R<-
-•L
caud
17-15. A high right ventriculotomy is made and the VSD exposed. The overriding truncal valve annulus is located in part over the right ventricle.
FIGURE
patch
ceph R<-
-•L
T
caud
17-16. The VSD is closed with a Dacron® patch, bringing the patch in front of the truncal valve annulus to connect the left ventricle to the aorta.
FIGURE
352 Color Atlas of Congenital Heart Surgery
aortic homograft
homograft mitral leaflet
17-17. The oblique right ventriculotomy is enlarged by removing an ellipse of ventricular wall along its lower margin. The posterior annulus of the aortic homograft is stitched to the posterior rim of the ventriculotomy. FIGURE
distal homograft
caud
17-18. The distal part of the homograft is tailored and stitched to the pulmonary arteries. It is useful to temporarily remove the aortic cross-clamp and allow the heart to fill during the tailoring to accurately define the appropriate length of the conduit. FIGURE
17
Truncus Arteriosus 353
homograft
homograft mitral leaflet
ceph R<-
->L
caud
17-19. The mitral leaflet portion of the homograft is stitched to the lower rim of the ventriculotomy and functions as a hood over the lower part of the conduit. The natural curve of the aortic conduit allows it to swing gently leftward and to lie away from behind the sternum along the left heart border.
FIGURE
17-1-3. Pulmonary Homograft-Valved Conduit
pericardial patch
ventriculotomy
ceph R<-
-•L
caud FIGURE 17-20. When a pulmonary homograft is used, a transverse right ventriculotomy is made. The pulmonary arteries have been detached from the truncal vessel and the aorta repaired with a pericardial patch.
354
Color Atlas of Congenital Heart Surgery
homograft
proximal muscle rim
R<
FIGURE 17-21. The proximal muscle rim of the pulmonary homograft is inspected. The posterior part will be partially excised.
pulmonary homograft
R^
FIGURE 17-22. The posterior muscle rim is stitched to the upper border of the ventriculotomy.
17
Truncus Arteriosus 355
pulmonary homograft
Gore-Tex hood
ceph R<-
-•L
caud
FIGURE 17-23. The homograft is tailored and stitched distally to the pulmonary arteries. A Gore-Tex® hood is placed over the proximal graft and attached to the lower rim of the ventriculotomy. In some cases, the anterior muscle rim of the proximal homograft is long enough to function as the hood, and the use of Gore-Tex® is avoided.
17-2. Valveless Patch Repair
pulmonary artery
ascending aorta
FIGURE
17-24. The aorta and pulmonary arteries arise from the truncal vessel.
356 Color Atlas of Congenital Heart Surgery
excised right pulmonary artery
ceph R««-
-•L
caud FIGURE
17-25. The pulmonary arteries are excised from the posterior aspect of the truncal
vessel.
truncal valve
R^
17-26. Through the aortic opening, the truncal valve is exposed and a valvotomy can be performed when truncal valve stenosis is present. FIGURE
17
Truncus Arteriosus
357
ventricular septal defect
ceph R<-
-•L
caud
FIGURE 17-27. After the aorta is closed primarily, a high right ventriculotomy i s made.
patch
R^
FIGURE 17-28. The VSD is closed with a Dacron® patch using a continuous stitch.
358 Color Atlas of Congenital Heart Surgery
proximal pulmonary artery
FIGURE 17-29. The posterior margin of the pulmonary artery branches is stitched to the upper rim of the ventriculotomy. Wide dissection of the pulmonary artery branches is necessary for adequate mobilization. In some cases, it is necessary to divide the ascending aorta and shift the right pulmonary artery anterior to the aorta to mobilize the pulmonary arteries adequately. The aorta is then reconstructed.
pulmonary artery
pericardial patch
R<
17-30. A pericardial patch is stitched over the anterior rim of the pulmonary arteries and then along the ventriculotomy. The lower margin of the pericardial outflow tract patch is wide, and this allows unobstructed flow into the pulmonary arteries. FIGURE
17
Truncus Arteriosus 359
17-3. Late Replacement of Obstructed Conduit When a valved conduit is used in infants and young children, replacement will always be necessary due to conduit failure or growth of the patient resulting in a conduit of inadequate size. Porcine-valved Dacron® conduits are particularly susceptible to failure because of neointima ingrowth or calcification of valve leaflets. At the time of conduit replacement, a pulmonary homograft is preferable, because it is less likely to develop significant leaflet or homograft wall calcification. Pulmonary artery pressure is usually normal in these patients so that the possible resistance of the aortic homograft to pulmonary hypertension is not needed. In some cases, when the pulmonary artery pressure is normal and a second failed conduit is being replaced, valveless patch reconstruction of the right ventricular outflow tract is performed. There is resulting pulmonary insufficiency that is usually well tolerated; however, an additional later operation is less likely because recurrent narrowing of the right ventricular outflow tract is rare.
conduit
ventriculotomy
ceph R<-
-•L
caud
17-31. After dissection of the heart and Dacron® conduit and placement of the child on cardiopulmonary bypass, the right ventricle to conduit connection is opened.
FIGURE
360 Color Atlas of Congenital Heart Surgery
neointima
porcine valve
ceph R«-
-•L
caud
17-32. The Dacron® conduit is detached from the ventricle and the neointima is apparent. FIGURE
pulmonary arteries
R^ caud FIGURE
17-33. The conduit is explanted from its bed and is detached distally.
17
Truncus Arteriosus 361
pulmonary homograft
homograft valve leaflets
R^ caud
17-34. A pulmonary homograft is used to reconstruct the right ventricular outflow tract. The posterior proximal muscle ridge of the homograft is tailored and stitched to the upper rim of the right ventriculotomy. FIGURE
pulmonary artery
homograft
R^
FIGURE 17-35. With the heart beating, the distal homograft is tailored to an appropriate length, and an end-to-end anastomosis is performed to the pulmonary arteries.
362
Color Atlas of Congenital Heart Surgery
Gore-Tex hood
R^
FIGURE 17-36. A hood of Gore-Tex® is used to connect the lower rim of the right ventriculotomy to the anterior muscle ridge of the homograft.
ascending aorta
homograft
R^
FIGURE 17-37. After completion of the repair, the new homograft conduit fits well and sternal compression should not occur.
17
Truncus Arteriosus 363
ascending aorta
conduit
neointima
R^
FIGURE 17-38. In another child, cardiopulmonary bypass is established, and the proximal part of the Dacron® conduit is opened. The thick neointima is exposed.
valve leaflets
neointima
17-39. The conduit is explanted, and the obstructing neointima is seen. Two valve leaflets are pliable, while the third is scarred and contracted.
FIGURE
364
Color Atlas of Congenital Heart Surgery FIGURE 17-40. The
pulmonary arteries
posterior
fibrous tract of the conduit will form the posterior wall of the reconstructed right ventricular outflow tract without inserting a valve. An anterior pericardial or Gore-Tex® patch will b e stitched over the tract to complete the reconstruction.
conduit tract
ventriculotomy ceph
17-4. Palliation with Plication In rare cases when total repair is contraindicated because of associated medical problems, palliation can be accomplished by creating osteal stenosis of the pulmonary arteries; this is performed without cardiopulmonary bypass. Small indwelling catheters are placed in the ascending aorta and in each branch pulmonary artery to monitor pressures; this is similar to the procedure that is used in pulmonary artery banding for complex VSD (see Chapter 5, Section 5-5).
ascending aorta
left pulmonary artery
right pulmonary artery
>ceph
FIGURE 17-41. Working through a left fourth interspace thoracotomy, the pericardium is opened. The pulmonary arteries arise from the base of the truncal vessel.
17
Truncus Arteriosus 365
area of osteal stenosis felted mattress sutures
17-42. Four felted mattress plicating sutures are placed full thickness through anterior and posterior aortic walls cephalad and caudad, respectively, to create osteal stenosis of the pulmonary arteries.
FIGURE
distal ascending aorta
pulmonary artery
area of osteal stenosis
caud
truncal vessel FIGURE 17-43. In the same patient 6 years later, an angiogram in preparation for total repair shows the area of osteal stenosis. The lungs have been protected from developing pulmonary hypertensive vascular obstruction disease.
366 Color Atlas of Congenital Heart Surgery
pulmonary artery
area of plication
truncal vessel ceph R<-
->L
caud FIGURE
17-44. At the time of corrective surgery, the area of plication can be seen.
area of plication
pulmonary artery
aortotomy
17-45. After establishing cardiopulmonary bypass and aortic cross-clamping, the pulmonary arteries are detached from the adjacent aorta. There is osteal stenosis at the area of previous plication. FIGURE
17
Truncus Arteriosus 367
right and left pulmonary arteries
ceph R<-
-•L
caud FIGURE 17-46. The distal right and left pulmonary artery orifices are seen. During the truncus repair, the proximal right and left pulmonary arteries are incised through the area of osteal stenosis, after which a valved conduit is stitched to the unobstructed pulmonary arteries.
17-5. Truncal Valve Stenosis Truncal valve stenosis or insufficiency may require valvotomy or valve replacement at the time of total repair of truncus arteriosus.
truncal valve
17-47. In this child, after the pulmonary arteries are detached, the truncal valve is easily exposed looking through the aortomy. The valve has three leaflets with commissural stenosis and thickening of each leaflet. A valvotomy is performed. FIGURE
>R
368 Color Atlas of Congenital Heart Surgery
truncal valve
caud L
-•R
ceph
17-48. In another infant with truncal valve stenosis, there is commissural stenosis with leaflet fibrosis that is amenable to valvotomy. FIGURE
18
Aortic Root Anomalies
18-1. Anomalous Origin of the Left Coronary Artery In this anomaly, the left coronary artery usually arises from the main pulmonary artery, usually resulting in left ventricular and septal ischemia and development of extensive collaterals between the right and left coronary arteries. Surgical repair is performed at any age immediately following the establishment of the diagnosis. Early repair is performed to avoid congestive heart failure and myocardial fibrosis caused by inadequate myocardial perfusion accentuated by a steal phenomenon with retrograde flow from the left coronary into the main pulmonary artery. Surgery should always establish a two-coronary system. 18-1-L
Repair by Intrapulmonary
Artery Tunnel (Takeuchi
Procedure) ascending aorta main pulmonary artery
left anterior descending coronary artery
branches of right coronary artery ceph R^
->L
caud
18-1. Extensive collaterals have formed between the right and left coronary arteries and there is marked enlargement of the left anterior descending coronary artery. FIGURE
369
370 Color Atlas of Congenital Heart Surgery
main pulmonary artery anomalous left coronary artery
ceph R<-
->L
caud FIGURE 18-2. The main left coronary artery arises from the left lateral aspect of the main pulmonary artery.
orifice of left coronary artery pulmonary
valve aortotomy
R<
18-3. After the cardiopulmonary bypass is established and with aortic clamping, cardioplegia, and profound local cardiac cooling, a transverse incision is made in the proximal main pulmonary artery. The orifice of the left coronary artery is immediately above the pulmonary valve. An aortotomy is made to visualize the aortic valve. FIGURE
18
Aortic Root Anomalies 371
punch aortotomy main pulmonary arteriotomy
R<
18-4. Using a circular punch, adjacent openings are made in the proximal ascending aorta and proximal main pulmonary artery. Semilunar valves are visualized to avoid injury to these structures.
FIGURE
A-P window
adjacent aortotomy
ceph R<-
-•L
caud
FIGURE 18-5. The adjacent circular openings are stitched together to create an aortopulmonary (A-P) artery window.
372 Color Atlas of Congenital Heart Surgery
pericardial patch tunnel
ceph R«-
-•L
caud FIGURE 18-6. A pericardial patch is stitched across the posterior wall of the main pulmonary artery immediately above the valve, creating a tunnel to connect the orifice of the left coronary artery to the iatrogenic A-P artery window. If indicated, the proximal main pulmonary artery is enlarged with a second pericardial patch placed in its anterior wall to avoid obstruction of the pulmonary artery by the posterior pericardial patch tunnel.
R<
18-7. In another child whose anomaly was not discovered until age 4 years, the apex of the left ventricle is extensively scarred due to longstanding ischemia.
FIGURE
18
Aortic Root Anomalies 373
fibrosis
18-8. After the coronary artery is repaired, the scarred aneurysm is incised. Myocardial fibrosis is seen.
FIGURE
fibrosis
ceph R^-
-•L
caud FIGURE 18-9. The incision in the left ventricular apex is extended to normal-appearing myocardium.
374 Color Atlas of Congenital Heart Surgery
ceph R«-
->L
caud
18-10. The aneurysm and scarred myocardium are excluded during closure of the left ventricular apex by interrupted felted mattress sutures followed by an over-and-over running stitch. FIGURE
18-1-2. Repair by Direct Re-Implantation ascending aorta main pulmonary artery
left anterior descending coronary artery main right coronary artery
R«*
18-11. In another patient, the left anterior descending coronary artery is markedly enlarged as is the main right coronary artery. Collateral vessels have developed from the right coronary across the surface of the right ventricle. FIGURE
18
Aortic Root Anomalies 375
main pulmonary artery main left coronary artery
18-12. The main pulmonary artery is retracted to display the origin of the main left coronary artery from the pulmonary artery.
FIGURE
orifice of left coronary artery pulmonary valve
ceph R<-
-•L
caud
18-13. A transverse opening in the proximal main pulmonary artery is made. The orifice of the anomalous left coronary artery is seen adjacent to normal pulmonary valve leaflets.
FIGURE
376 Color Atlas of Congenital Heart Surgery
aortotomy
left coronary artery
caud
18-14. The anomalous left coronary artery is explanted from the main pulmonary artery with a button of surrounding tissue. A higher ascending aortotomy is made in order to identify the aortic valve leaflets. FIGURE
pericardial patch
ceph 4 R<- ->L caud
18-15. A pericardial autograft is stitched over the coronary explant site. A site for the implant of the left coronary artery is marked on the aortic root remote from the aortic valve leaflets. FIGURE
18
Aortic Root Anomalies 377
R^ caud FIGURE 18-16. An oval opening is made in the medial aortic root avoiding damage to the valve leaflets. The left coronary artery will be shifted to the aortic root for a direct anastomosis.
coronary anastomosis
R^
FIGURE
18-17. The left coronary artery to aorta anastomosis is being constructed.
378 Color Atlas of Congenital Heart Surgery
main pulmonary artery pericardial patch left coronary artery
R<
18-18. The coronary to aorta anastomosis is complete. A pericardial patch is stitched over the explant site in the main pulmonary artery. FIGURE
18-1-3. Coronary Artery Origin from Right Pulmonary
Artery
ascending aorta right pulmonary artery anomalous left coronary artery
ceph R«-
-•L
caud
18-19. In another baby, the anomalous left coronary artery arises from the caudad surface of the proximal right pulmonary artery.
FIGURE
18
Aortic Root Anomalies
379
anomalous left coronary artery
R^ caud
FIGURE 18-20. After placing the child on bypass, the anomalous coronary vessel is inspected. It can be seen entering the epicardial surface of the heart, behind the ascending aorta.
coronary artery button
FIGURE 18-21. The anomalous
coronary vessel is explanted from the right pulmonary artery with a button of surrounding tissue.
380 Color Atlas of Congenital Heart Surgery
ascending aorta coronary anastomosis
18-22. The button is anastomosed to the posterior ascending aorta, being careful not to twist the coronary during implantation. FIGURE
coronary anastomosis
R^
FIGURE
18-23. The coronary anastomosis is complete.
18
Aortic Root Anomalies 381
left coronary artery
ceph R+-
-•L
caud
18-24. The coronary implant site is viewed through an anterior opening in the ascending aorta. Probing it confirms patency.
FIGURE
right pulmonary artery pericardial patch
R^
FIGURE 18-25. A pericardial patch is stitched over the explant site in the proximal right pulmonary artery.
382 Color Atlas of Congenital Heart Surgery
18-2. Anomalous Ostium and Intramural Coronary Artery In many cases, this diagnosis is first entertained after a bout of syncope or a near sudden death episode with resuscitation. A careful echocardiography study can be indicative or diagnostic but in some cases a computerized tomography (CT) scan and/or angiogram is useful in establishing the diagnosis. Repair is performed using cardiopulmonary bypass with moderate hypothermia and cardioplegic arrest.
aorta
main pulmonary artery
right coronary artery
18-26. The plane between the great vessels has been dissected and the right coronary artery is seen to arise more to the left and posterior than usual.
FIGURE
caud
left coronary artery
>L
18-27. The left coronary artery is in its usual position.
FIGURE
18
Aortic Root Anomalies 383
right coronary artery
ceph R«-
-•L
caud FIGURE 18-28. After establishing bypass, aortic cross-clamping, and cardioplegic arrest, a proximal ascending aortotomy is made. The orifice of the right coronary artery is adjacent to the commissure between right and left coronary cusps.
right angle clamp
ceph R<-
-•L
caud
18-29. A right-angle clamp is placed into the orifice and the wall of the intramural portion of the vessel is incised.
FIGURE
384 Color Atlas of Congenital Heart Surgery
coronary orifice
R^
18-30. Multiple stitches of fine polypropylene are used to close the intima at the site of incision to ensure that minor perforations are closed. FIGURE
18-31. A probe is passed into the orifice to ensure patency. Ideally, the wall of the aorta is incised extensively to transfer the orifice anteriorly. The coronary artery should now arise at a right angle to the wall of the aorta.
FIGURE
18
Aortic Root Anomalies 385
18-3. Coronary Artery Fistula Fistulae occur more commonly between the right coronary artery and right heart chambers. Repair is indicated to prevent congestive heart failure or extensive dilation and permanent deformity of the coronary arteries. In come cases, simple ligation of the fistula can be performed without cardiopulmonary bypass when it is easily localized on the surface of the heart. In most cases, it is necessary to perform the repair by working within the cardiac chambers with cardiopulmonary bypass.
ascending aorta
proximal right coronary artery
coronary artery aneurysm
18-32. The right coronary artery is dilated and there is an aneurysm of the distal main vessel opposite the site of the coronary artery to right ventricle fistula. FIGURE
fistula
18-33. Using cardiopulmonary bypass with aortic clamping, cardioplegia is delivered antegrade while compressing the fistula or retrograde in order to maximize delivery. Ice is packed around the heart for profound local cooling. The aneurysm is entered and the fistula identified. FIGURE
>L
386 Color Atlas of Congenital Heart Surgery
tricuspid valve coronary fistula
18-34. A right atriotomy is made, and the internal orifice of the fistula is seen beneath the anterior tricuspid valve leaflet near the annulus. FIGURE
repaired fistula
R<
18-35. Working on the surface of the heart and within the aneurysm, the fistula is closed with interrupted sutures.
FIGURE
18
Aortic Root Anomalies 387
repaired fistula
18-36. Additional sutures are placed in the right ventricle to close the internal orifice of the fistula.
FIGURE
repaired aneurysm
R^ caud
18-37. The coronary artery is repaired primarily after partial resection of the aneurysm.
FIGURE
388 Color Atlas of Congenital Heart Surgery
ascending aorta
right coronary artery
FIGURE
18-38. In another child, an enlarged right coronary artery is seen.
right coronary artery
ceph R+-
-•L
caud
18-39. After establishing cardiopulmonary bypass and with aortic clamping, cardioplegia, and profound local cooling, the right atrium is opened. An enlarged and tortuous right coronary artery is seen protruding in the upper atrial septum beneath the endocardium.
FIGURE
18
Aortic Root Anomalies 389
coronary fistula
R^
18-40. The internal orifice of thefistulais located in the distal part of the enlarged and tortuous coronary artery.
FIGURE
opened fistula tract
R<
18-41. For improved exposure, the fistula tract is opened and the neck or internal orifice is seen.
FIGURE
390 Color Atlas of Congenital Heart Surgery
repaired fistula
ceph R<-
-•L
t caud FIGURE
18-42. The fistula is repaired with interrupted felted sutures.
left anterior descending coronary artery
R^
18-43. In another patient, there was a large isolated fistula between the markedly enlarged left anterior descending coronary artery and right ventricular apex.
FIGURE
18
Aortic Root Anomalies 391
area of dissection
left anterior descending coronary artery
18-44. Without cardiopulmonary bypass, the apex of the heart is tipped up and dissection into right ventricular myocardium at the apex is carried out to the left of the presumed fistula site.
FIGURE
fistula
ceph R<-
-^L
caud FIGURE 18-45. Similar dissection is performed to the right of the coronary artery and an isolated fistula is located. If difficulty had been encountered, the child would have been placed on cardiopulmonary bypass for completion of the dissection.
392 Color Atlas of Congenital Heart Surgery
fistula
18-46. A felted stitch is passed from the left toward the right side of the fistula beneath the enlarged left anterior descending coronary artery at the apex of the heart. FIGURE
I i gated fistula
R^
18-47. The fistula is closed with the simple mattress suture. Postrepair intraoperative echocardiography confirmed closure.
FIGURE
18
Aortic Root Anomalies
393
18-4. Aortopulmonary (A-P) Window Repair of this anomaly is performed immediately after the diagnosis is established regardless of age because there is unrestricted pulmonary blood flow with pulmonary hypertension. Otherwise, there is a danger of early development of pulmonary vascular obstructive disease as well as severe congestive heart failure.
ascending aorta A-P window
FIGURE 18-48. In some cases, the surgeon is tempted to repair this anomaly without using cardiopulmonary bypass. Because of the possibility of catastrophic injury to the left coronary artery or other surrounding structures, cardiopulmonary bypass is always used. The large aorto-pulmonary window is seen connecting the distal ascending aorta to the main pulmonary artery.
main pulmonary artery
left pulmonary artery
FIGURE 18-49. After cardiopulmonary bypass is established and with aortic clamping, cardioplegia is delivered antegrade while compressing the aorto-pulmonary window. A longitudinal arteriotomy is made in the main pulmonary artery. The window is readily visualized.
right pulmonary artery A-P window
>L
394
Color Atlas of Congenital Heart Surgery
window patch
R^
FIGURE 18-50. Repair is performed by placing a Dacron® patch over the window using a continuous suture. When the window is near the semilunar valves, care must be exercised to avoid injury to the left coronary artery, which is located posterior to the proximal main pulmonary artery.
right pulmonary artery
R +
FIGURE 18-51. The adjacent orifice of the right pulmonary artery is seen, and the patch does not compromise this opening.
18
Aortic Root Anomalies 395
18-5. Aortic Origin of Pulmonary Artery In this rare anomaly, the right pulmonary artery arises from the ascending aorta. Early repair is indicated to avoid unilateral development of pulmonary vascular obstructive disease.
ascending aorta
right pulmonary artery
ceph
R<-
->L
caud
18-52. The right pulmonary artery arises from the posterior and right lateral aspect of the ascending aorta.
FIGURE
patent ductus arteriosus ascending aorta right pulmonary artery main pulmonary artery
ceph R«-
->L
caud FIGURE 18-53. The great vessels are separated. The right pulmonary artery is seen arising from the posterior aorta rather than the main pulmonary artery. There also is a patent ductus arteriosus that is closed prior to cardiopulmonary bypass.
396
Color Atlas of Congenital Heart Surgery
right pulmonary artery
R<
FIGURE 18-54. Using cardiopulmonary bypass, with aortic clamping, cardioplegia, and profound local cooling, the right pulmonary artery is separated from the aorta. The aortotomy is closed primarily.
ductal stump
anastomosis main pulmonary artery ceph R<-
-•L
caud
FIGURE 18-55. The right pulmonary artery is shifted behind the ascending aorta and anastomosed to the distal main pulmonary artery in an anatomic position. Hie ductal stump is seen.
18
Aortic Root Anomalies 397
18-6. Left Ventricle to Aorta Tunnel Corrective surgery should be performed as soon as the diagnosis is made to avoid left ventricular compromise and severe hemodynamic instability resulting from free regurgitation into the left ventricle.
ascending aorta main pulmonary artery
R +
FIGURE 18-56. The ascending aorta is severely dilated as a result of the significant to-andfro flow through the tunnel. The main pulmonary artery is normal in size.
aortic valve
18-57. Immediately after cardiopulmonary bypass is established, the aortic clamp is applied to avoid left ventricular dilatation secondary to regurgitation through the tunnel. Profound local cooling is used, and cardioplegia solution is delivered directly in the coronary orifices after the proximal ascending aorta is opened. An alternative method of myocardial preservation is retrograde cardioplegia that is delivered through the coronary sinus. A trileafed aortic valve of normal size and architecture is seen. FIGURE
398 Color Atlas of Congenital Heart Surgery
right coronary orifice aortic valve
18-58. By retracting the proximal aorta, the orifice of the right coronary artery is exposed anteriorly. FIGURE
tunnel
aortic valve R^
FIGURE
18-59. The tunnel is located posterior to the aortic valve annulus.
18
Aortic Root Anomalies 399
tunnel
left coronary orifice aortic valve
R^
FIGURE 18-60. The proximity of the left coronary artery to the tunnel is seen. The coronary artery passes posterior to the tunnel and near the wall of the aorta.
patch
aortic valve R^
left coronary orifice FIGURE 18-61. Repair is performed using a Dacron® patch with multiple interrupted felted mattress sutures. The anterior stitches are placed in the aortic valve annulus. Posterior repair sutures are placed in the wall of the aorta, taking care to avoid injury to the adjacent left coronary artery. After placement of the patch, the left coronary artery is probed to ensure its patency.
400 Color Atlas of Congenital Heart Surgery
18-7. Sinus of Valsalva Aneurysm Sinus of Valsalva aneurysm is a thin-walled enlargement or outpouching of the aortic wall and usually originates from the right sinus or anterior aspect of the noncoronary sinus. It then penetrates the base of the heart into an adjacent lowpressure chamber. If the aneurysm is unruptured, it should be closed working only through the aortic root. If rupture has occurred, both ends of the aneurysm should be repaired.
aneurysm tricuspid valve
ceph R<-
-•L
caud
18-62. The tip of the aneurysm is seen in the right ventricle near the tricuspid valve annulus. FIGURE
aneurysm
ceph R<-
->L
caud FIGURE
18-63. The aneurysm sac is stretched like a windsock, but here is not ruptured.
18
Aortic Root Anomalies 401
orifice of aneurysm
ceph R<-
->L
caud
18-64. A proximal ascending aortotomy is made. The right coronary cusp is shown and the orifice of the aneurysm is at the base of same near the annulus.
FIGURE
aortic valve leaf aneurysm repair
ceph R<-
-•L
t caud FIGURE
18-65. The orifice of the aneurysm is closed with multiple polypropylene sutures.
402 Color Atlas of Congenital Heart Surgery
18-8. Aortic Atresia and Hypoplastic Left Heart Syndrome The Norwood procedure or Stage I palliation or plastic reconstruction of the aortic root is performed in neonates as an urgent operation after medical stabilization, including maintenance of ductal patency to provide adequate systemic perfusion. pH and blood gases are corrected while pulmonary blood flow may be controlled with manipulation of pH and p C 0 2 . Stage II or the hemi-Fontan procedure or bidirectional cavopulmonary shunt is usually carried out at 2 to 4 months of age, while Stage III or the completion Fontan operation is generally performed around age 2 years. The clinical condition of the child, including the hemodynamic state, peripheral oxygenation, and other factors, may alter this timing. With appropriate surgery at an experienced center, survival today should be equivalent to all other major congenital heart operations. Stage I palliation is performed with cardiopulmonary bypass, deep hypothermia (20°C), and cardioplegic arrest. Low-flow cerebral perfusion is used during arch reconstruction and total circulatory arrest is only necessary for the few minutes during which atrial septectomy is carried out. 18-8-1.
Norwood
Procedure
with a Blalock
Shunt
left carotid artery innominate artery transverse arch hypoplastic ascending
aorta main pulmonary artery
18-66. The severely hypoplastic ascending aorta and large main pulmonary artery are seen. The larger arch and head vessels are dissected. Silks for snaring are passed around the head vessels for later use. FIGURE
Gore-Tex graft attached to innominate artery
purse strings for subsequent cannulation
>L
FIGURE 18-67. A 3.5-mm tubular Gore-Tex® graft is anastomosed to the innominate artery. The arterial perfusion cannula will be placed in the graft and utilized for cardiopulmonary bypass, as well as low-flow cerebral perfusion. A single right atrial appendage venous cannula is used for venous drainage. Purse strings are placed in the proximal main pulmonary artery for later arterial cannulation.
18
Aortic Root Anomalies 403
FIGURE 18-68. Two patches of pulmonary artery homograft wall material are cut. The smaller will be used to close the distal main pulmonary artery or pulmonary arterial confluence, while the larger one is used to construct the neoaorta.
18-69. The child has been placed on bypass with arterial inflow through the Gore-Tex® graft and venous return through a single right atrial cannula. Much of the dissection is performed during the cooling phase. Silks for snaring are passed around the pulmonary artery branches and head vessels. The ductus arteriosus, aortic isthmus, and upper descending thoracic aorta are dissected. The first dose of cardioplegia can be delivered with a catheter in the proximal main pulmonary artery after snaring all head vessels and the descending thoracic aorta below the ductus. A snare is in place around the proximal innominate artery below the Gore-Tex® graft which will be used later for low-flow cerebral perfusion. The main pulmonary artery is divided proximal to the branches. A drainage catheter is passed through the pulmonary valve to collect excess blood. The ductus arteriosus has been closed with two hemoclips. The smaller homograft pulmonary artery wall patch is being stitched to the open confluence of the pulmonary artery branches that have been snared. FIGURE
404 Color Atlas of Congenital Heart Surgery
left subclavian artery
ductus arteriosus
pulmonary artery branch confluence homograft patch
ceph R*-
->L
caud
18-70. Homograft patch closure of the pulmonary artery confluence is complete. Hemoclips on the large ductus arteriosus are seen. FIGURE
head vessel snares
arch stump aortotomy
descending aorta
ceph R«-
->L
caud
18-71. The distal aortic arch beyond the left subclavian artery is doubly clipped with hemoclips and divided. The aortic isthmus, including any coarctation along with the distal divided ductal stump, are excised. A clamp is placed on the descending thoracic aorta that is moved into thefield.The transverse aortic arch is incised along its inferior margin and the aortotomy is extended down the medial aspect of the hypoplastic ascending aorta. Low flow cereleral perfusion is carried out through the Gore-Tex® graft. FIGURE
18
Aortic Root Anomalies 405
arch aortotomy
ceph R<-
-•L •
caud
18-72. The descending thoracic aorta is anastomosed to the distal arch and this forms part of the back wall of the neoaorta.
FIGURE
probe
ascending aorta suture line proximal main pulmonary artery ceph R<-
-•L
caud
pulmonary artery branch confluence
FIGURE 18-73. A short incision is made in the medial aspect of the proximal main pulmonary artery. The hypoplastic ascending aorta is incised down to this point. These two vessels are anastomosed with 7.0 Prolene. A 1-mm probe is passed proximally through the ascending aorta to confirm patency down to the coronary orifices.
406 Color Atlas of Congenital Heart Surgery
homograft patch
posterior suture line proximal main pulmonary artery
18-74. The larger pulmonary artery homograft wall patch is stitched posteriorly to the transverse arch and ascending aorta to complete the back wall of the neoaorta. FIGURE
head vessel and subclavian artery snares native arch homograft patch in neoaorta aortic cannula
ductal stump
R^
18-75. The homograft patch is tailored and then stitched anteriorly to the transverse arch and ascending aorta. It is then connected to the proximal main pulmonary artery, completing construction of the neoaorta. A new arterial perfusion catheter is placed in the proximal neoaorta for arterial inflow. The catheter in the Gore-Tex® graft is removed and this graft will be moved caudad for anastomosis to the pulmonary arteries. The pulmonary artery branch confluence has been moved to beneath the ascending aorta for construction of the modified Blalock shunt. FIGURE
18
Aortic Root Anomalies 407
atrial septal defect
ceph R«*-
->L
caud
18-76. A brief period of circulatory arrest is used for atrial septectomy. The venous cannula can be removed from the appendage and exposure of the atrial septum accomplished through this opening. FIGURE
modified Blalock shunt
MV02 catheter
ceph R<-
-•L
caud
right atrial catheters
18-77. During construction of the Gore-Tex® graft to pulmonary artery anastomosis, rewarming on full bypass is carried out. Here, a metal clip is placed on the Blalock shunt to control flow through same prior to removal from bypass. An MV0 2 catheter is placed in the superior vena cava and passed into the upper right atrium while two additional catheters are placed directly in the right atrium for fluid and medication infusion.
FIGURE
408
Color Atlas of Congenital Heart Surgery
18-8-2. Modified Norwood Procedure with Right Ventricle to Pulmonary Artery Shunt (Sano Procedure)
innominate artery
bulldog clamp
ceph -•L
R<-
caud
FIGURE 18-78. The innominate artery is isolated with a distal snare and a proximal bulldog clamp. An incision is made in this vessel for the graft anastomosis.
Gore-Tex graft
ceph R<-
-•L
caud
FIGURE 18-79. A beveled 3.5-mm tubular Gore-Tex® graft will be used.
18
Aortic Root Anomalies 409
innominate artery
main pulmonary artery ascending aorta
R<
FIGURE
18-80. The Gore-Tex® graft is anastomosed to the innominate artery.
arterial cannula
18-81. The arterial perfusion cannula is placed in the Gore-Tex® graft for arterial inflow.
FIGURE
410 Color Atlas of Congenital Heart Surgery
light atrial cannula
Re-
FIGURE
18-82. A single right atrial venous cannula is utilized.
homograft patch
Gore-Tex conduit
18-83. A circular homograft pulmonary artery wall patch is used in the construction of a composite Gore-Tex® graft.
FIGURE
18
Aortic Root Anomalies 411
homograft patch
FIGURE 18-84. An incision is made in the middle of the homograft patch and the GoreTex® graft is anastomosed to same.
pulmonary artery branch snares pulmonary artery branch confluence composite homograft-Gore-tex conduit ceph R«-
-•L
caud FIGURE 18-85. The child has been placed on cardiopulmonary bypass and cooled, during which the extensive dissection is performed. Cardioplegic arrest is induced and the ductus arteriosus closed. The main pulmonary artery is divided proximal to the branches. The composite Gore-Tex® conduit is brought into the field for anastomosis to the pulmonary artery branches.
412 Color Atlas of Congenital Heart Surgery
composite Gore-Tex graft proximal main pulmonary artery
R^
18-86. The posterior row of the anastomosis between the homograft patch cuff and the posterior pulmonary artery branch confluence is complete.
FIGURE
composite conduit to pulmonary artery anastomosis
FIGURE
18-87. The anterior part of this anastomosis is complete.
18
Aortic Root Anomalies 413
ascending aorta
proximal main pulmonary artery ceph
caud
18-88. A clamp has been placed on the proximal main pulmonary artery for hemostasis and to prevent air from entering the venous catheter during cardiopulmonary bypass. At this point low-flow cerebral perfusion is induced and the arch and ascending aorta opened. FIGURE
arch descending aorta homograft patch ascending aorta proximal main pulmonary artery composite Gore-Tex graft R^
18-89. The ductus has been divided, the distal arch beyond the left subclavian artery has been divided, the isthmus has been excised, and the descending aorta anastomosed to the posterior distal arch. A homograft patch has been stitched to the posterior ascending aorta and will now be folded over to form the anterior wall of the neoaorta. FIGURE
414 Color Atlas of Congenital Heart Surgery
homograft patch
18-90. The homograft patch has been anastomosed anteriorly. Only the homograft to proximal main pulmonary artery anastomosis is necessary to complete the neoaorta. After this, a brief period of circulatory arrest will be used during which the atrial septectomy is performed. Next, full cardiopulmonary bypass will be used for rewarming with arterial inflow through the innominate artery Gore-Tex® graft and venous return through the right atrial appendage cannula. FIGURE
neoaorta pulmonary artery branch confluence
right ventricle to composite Gore-Tex graft anastomosis
ceph R«-
-•L
caud
18-91. A small high circular ventriculotomy is made immediately below the neoaortic valve using care to avoid injury to the leaflets. The proximal composite Gore-Tex® graft is stitched to the ventriculotomy. Here, the graft passes to the right of the neoaorta. Alternatively, it may also be placed to the left of the neoaorta. FIGURE
19
Interrupted Aortic Arch
Survival in infants with this interrupted aortic arch depends on ductal patency for lower torso perfusion, so prostaglandin-E (PGE) infusion is always used during medical stabilization followed by immediate surgical repair. Repair of Type A interruption (interruption beyond the left subclavian artery) is performed through a left thoracotomy. Associated anomalies [e.g., ventricular septal defect (VSD)] may be palliated with pulmonary artery banding through the same thoracotomy or repaired primarily working through a separate median sternotomy. Type B (interruption beyond left carotid artery) or Type C (interruption between innominate and left common carotid arteries) interruption and associated anomalies are repaired through a median sternotomy with cardiopulmonary bypass, deep hypothermia, and temporary low-flow cerebral perfusion.
19-1. Type A Interruption
19-1. Working through a left thoracotomy, dissection of the aortic arch, left subclavian artery, patent ductus arteriosus, and descending thoracic aorta is carried out. A thin fibrous cord connects the two aortic segments.
FIGURE
415
Color Atlas of Congenital Heart Surgery
ductal stump
aortic anastomosis
ant caud^-
-•ceph
post
19-2. The ductus arteriosus is divided and the pulmonary artery end is closed. A generous incision is made in the aortic arch and an end-to-end anastomosis is performed. A continuous over-and-over suture is used for the posterior anastomosis, and interrupted everting mattress sutures are placed anteriorly. FIGURE
19-2. Type B Interruption
ascending aorta main pulmonary artery
19-3. Working through a median sternotomy, the typical Y shape of the ascending aorta and common carotid arteries is seen. FIGURE
19
Interrupted Aortic Arch 417
carotid arteries
left subclavian artery
patent ductus arteriosus
ceph -•L
caud
19-4. During the dissection, large silk sutures are passed around the common carotid and left subclavian arteries for later snaring during arch reconstruction. No transverse arch is present and the ductus arteriosus connects the main pulmonary artery to the descending aorta. FIGURE
left subclavian artery descending aorta ductus arteriosus
FIGURE
19-5. The ductus arteriosus and upper descending thoracic aorta are dissected.
418 Color Atlas of Congenital Heart Surgery
right carotid artery
purse string stitches
ceph R<-
-•L
caud
19-6. Purse string sutures for aortic cannulation are placed in the ascending aorta and proximal main pulmonary artery (or patent ductus). The baby is placed on bypass with a cannula in the ascending aorta and a longer cannula that is passed through the main pulmonary artery and ductus into the descending aorta. Alternatively, a 3.5-mm tubular Gore-Tex® graft is stitched to the right carotid artery, which accepts the arterial perfusion cannula. This allows for low-flow cerebral perfusion during arch reconstruction, avoiding total circulatory arrest. FIGURE
descending thoracic aorta
ceph R+-
-•L
caud
19-7. During cooling, the left pleural space is entered and the mid descending aorta is dissected to enhance mobility for a primary aortic anastomosis. FIGURE
19
Interrupted Aortic Arch 419
ductus arteriosus
occluding clamp on descending thoracic aorta
19-8. Arch vessels are snared; cardioplegia solution is injected; and, here, circulatory arrest is induced. Currently, low-flow cerebral perfusion would be used. Arterial and venous cannulae are removed. The ductus arteriosus is divided and the pulmonary artery end is ligated. A clamp is placed on the upper thoracic aorta for hemostasis and to prevent air from entering the descending aorta. The clamp is useful in shifting the lower aortic segment cephalad for the anastomosis. FIGURE
ductus remnant descending aorta
19-9. Ductal tissue attached to the thoracic aorta is excised so that sutures can be placed in the more substantial aortic wall.
FIGURE
420 Color Atlas of Congenital Heart Surgery
left subclavian artery
ceph
R<-
-•L
t caud
19-10. To enlarge the lower aortic segment, the incision is extended into the base of the left subclavian artery. FIGURE
ascending aorta descending aorta
R^
FIGURE 19-11. A generous aortotomy is made in the ascending aorta, extending into the proximal left common carotid artery. A continuous suture is used to construct the posterior row of the anastomosis, working within the lumen of the aorta.
19
Interrupted Aortic Arch 421
aortic anastomosis cannulation site
caud
19-12. For the anterior anastomosis a continuous suture is used, but it is interrupted in a few places to allow growth of the aorta at the anastomosis. The aortic clamp used for traction on the lower aorta is removed after the anastomosis is completed.
FIGURE
artrial septal defect
19-13. Working through a right atriotomy, the atrial septal defect (ASD) is closed primarily.
FIGURE
422 Color Atlas of Congenital Heart Surgery
ventricular septal defect tricuspid valve
FIGURE
19-14. The tricuspid valve is retracted to expose the VSD.
patch
ceph R<-
-•L
caud FIGURE
sutures.
19-15. A Dacron® patch is placed over the VSD using interrupted felted mattress
19
Interrupted Aortic Arch 423
snares
stenotic orifice ascending aorta
ceph R<-
-•L
caud
19-16. In another child, 4 years following initial repair, anastomotic stenosis is repaired. After the patient is cooled to deep hypothermia with cardiopulmonary bypass, head vessel snares are pulled tight and circulatory arrest is induced. Currently, low-flow cerebral perfusion would be used. A longitudinal aortic opening is made proximal to the stenosis exposing the narrow area of transverse arch. FIGURE
descending aorta
ridge at stenosis site ascending aorta
FIGURE
aorta.
19-17. The aortotomy is extended across the stenosis and into the descending
424 Color Atlas of Congenital Heart Surgery
patch
ceph R^-
-•L
caud
19-18. Arch angioplasty is performed by stitching a Dacron® patch over the aortomy. Currently, a patch of homograft aorta or pulmonary artery wall would be used.
FIGURE
20
Coarctation of the Aorta
There are many techniques to repair aortic coarctation, and each has advantages and disadvantages in accomplishing relief of the aortic obstruction with the lowest mortality and chance of recurrence. No single technique is superior for use in all patients.
20-1. Resection and Primary Anastomosis This repair is historically the gold standard to which to compare the results of other techniques, although it may require a more extensive dissection to mobilize the arch and the descending aorta for adequate length of aortic segments used in the primary repair. When this operation is performed in small infants, the recurrence rate is higher at some centers, but it continues to be my preferred operation for use in children beyond infancy. Use of the extended end-to-end anastomosis may help to avoid recurrence. After resection of the coarctation, the upper aortic segment is incised longitudinally along the inferior surface of the arch while the lateral descending aorta is incised. This results in an oblique anastomosis that is usually longer than the aortic diameter.
FIGURE 20-1. Working through a left thoracotomy, the parietal pleura is incised and the vagus and recurrent nerves are reflected toward the mediastinum. Dissection is performed around the distal arch, left subclavian artery, ductus arteriosus, and upper half of the descending aorta.
left subclavian artery patent ductus arteriosus coarctation
>ceph
425
426 Color Atlas of Congenital Heart Surgery
ductus stump
aortic arch left subclavian artery anastomosis
ant caud**-
->ceph
post
20-2. The ductus arteriosus is doubly clamped, divided, and the pulmonary artery end is closed. Vascular clamps are applied to the aorta. The aorta, ductal stump and coarctation are resected. An end-to-end anastomosis is performed with a continuous over-andover suture in the posterior row. Interrupted mattress sutures are used to construct the anterior anastomosis. The anastomosis incorporates the base of the subclavian artery, as well as the distal arch because of a size discrepancy between the upper and lower aortic segments. Alternatively, and used in most cases, an extended end-to-end anastomosis is performed by incising longitudinally the inferior surface of the arch up to the level of the left carotid artery. The opened lower aorta is incised longitudinally on its lateral aspect, after which a direct anastomosis is performed between the aortic segments. The resulting suture line is longer than the diameter of the aorta. FIGURE
coarctation
FIGURE
20-3. The resected specimen reveals a severe aortic narrowing.
20
Coarctation of the Aorta 427
patent ductus arteriosus aortic arch left subclavian artery hypoplastic isthmus
ant caud^-
-^ceph
post
20-4. In another patient with a typical preductal coarctation, the aortic isthmus is hypoplastic.
FIGURE
aortic arch
left subclavian artery anastomosis
ant caud<-
-^ceph
post
FIGURE 20-5. The repair includes ductal division and closure of the pulmonary artery end of that structure. The hypoplastic aorta and discrete coarctation are resected. An end-toend anastomosis is performed between the two aortic segments with a size discrepancy. The proximal aorta may be incised to the region of the mid arch if the arch is hypoplastic, and an extended end-to-end anastomosis performed.
428 Color Atlas of Congenital Heart Surgery
20-2. Subclavian Artery Flap Angioplasty This is the preferred operation for small infants. It can be performed rapidly, with less dissection and a simple and predictable anastomosis. Relief of the aortic obstruction is excellent, and recurrence is rare because the aortic suture is not circumferential.
aortic arch
left subclavian artery hypoplastic isthmus ant caud<-
area of coarctation
->ceph
post
20-6. Working through a left thoracotomy, the parietal pleura is incised and the nerves are reflected anteriorly. The left subclavian artery and distal arch are dissected. The descending aorta is freed only a short distance beyond the ductus or ligamentum.
FIGURE
coarctation
ant caud^-
-*-ceph
post
20-7. The distal left subclavian artery is ligated immediately beyond the vertebral artery, which is ligated separately. Leaving some distal branches to intercommunicate results in improved left arm perfusion. Snares are pulled tight around the distal arch and the upper descending aorta. The aorta is incised below the coarctation and this incision is extended cephalad across the isthmus and the subclavian artery. If the ductus is patent it is closed with a hemoclip. FIGURE
20
Coarctation of the Aorta
429
subclavian artery flap
1; *****'
transverse incision at lower extreme of aortotomy
ant -•ceph post
20-8. The subclavian artery is divided distally immediately proximal to the ligature and the artery flap is moved caudad for anastomosis over the aortotomy. The lower end of the aortotomy has been opened transversely in T fashion, resulting in a larger aortic lumen distally after completion of the anastomosis. FIGURE
subclavian artery flap
ant A -•ceph post FIGURE
suture.
20-9. The flap is anastomosed to the aorta with a continuous fine monofilament
430 Color Atlas of Congenital Heart Surgery
hemoclip on ductus subclavian artery flap
ant -•ceph
caud<-
post
20-10. The completed repair includes an adequate aortic lumen composed of endogenous and viable aortic tissue without a circumferential aortic suture line. The aorta should grow at the site of the previous coarctation, resulting in less chance of recurrence. The lower end of the flap is wide, resulting in a larger aortic lumen caudad to the coarctation shelf. FIGURE
ant caud<-
-•ceph
post
20-11. In another baby in whom the lower end of the aortic incision is not opened in T fashion, the distal part of the flap is narrow, and there is a chance of residual obstruction. FIGURE
20
Coarctation of the Aorta 431
distal arch left subclavian artery ductus arteriosus anomalous right subclavian artery
ant caud<-
-^•ceph
coarctation
post
20-12. In another infant, an anomalous right subclavian artery arises from the aortic isthmus.
FIGURE
distal arch left subclavian artery ductus arteriosus subclavian artery flap
ant caud^—4—• ceph post
20-13. The right subclavian artery is dissected in the mediastinum and is ligated distally. The ductus arteriosus is ligated. The right subclavian artery is divided, and an incision is made along its proximal part and onto the upper descending thoracic aorta to a point beyond the coarctation. The anomalous right subclavian artery flap is moved to a caudad position for anastomosis over the aortotomy. FIGURE
432 Color Atlas of Congenital Heart Surgery
subclavian artery flap
ant caud<-
-•ceph
post FIGURE
20-14. The flap repair is more posterior but is adequate to relieve the obstruction.
left subclavian artery coarctation
ant caud<-
-•ceph
post
20-15. In another infant, the coarctation is located between the left common carotid and left subclavian arteries.
FIGURE
20
Coarctation of the Aorta 433
left carotid artery subclavian artery reverse flap ant cauch
•ceph
post
20-16. A reverse subclavian artery flap is performed. The left subclavian artery is divided distally and then incised proximally along its medial border. This incision extends across the arch coarctation and onto the base of the left carotid artery. The subclavian artery flap is shifted medially and anastomosed over the aortotomy using a continuous suture.
FIGURE
aortic arch left carotid artery subclavian artery reverse flap ant caud^-
-•ceph
post FIGURE
20-17. The completed repair shows the reverse flap.
434 Color Atlas of Congenital Heart Surgery
left carotid artery hypoplastic arch left subclavian artery
ant caud<-
ductus arteriosus
-•ceph
coarctation
post FIGURE 20-18.
In another infant, there is severe hypoplasia of the distal aortic arch beyond the left common carotid artery and, in addition, an isthmic coarctation.
patent ductus arteriosus left carotid artery
subclavian artery reverse flap coarctation
ant caud*<-
-*-ceph
post FIGURE 20-19. The hypoplastic arch is repaired with a left subclavian artery reverse flap. During this part of the surgery, the ductus remains patent and supplies blood to the lower aorta.
20
Coarctation of the Aorta 435
ductus stump
Gore-Tex patch
ant caud^-
-•ceph
post
20-20. The ductus arteriosus is divided and each end is oversewn. A Gore-Tex® patch is then stitched over a long aortotomy made from the subclavian artery flap and across the isthmus.
FIGURE
left subclavian artery reverse flap
ant caud<-+->ceph post FIGURE
20-21. The reverse flap and Gore-Tex® patch are visualized.
436 Color Atlas of Congenital Heart Surgery
distal arch area of recurrent coarctation ant caud^-
->ceph
post FIGURE 20-22. In another infant, who previously underwent subclavian artery flap angioplasty, a recurrence occurred requiring repeat surgery 1 year later. Alternatively, balloon dilatation can be performed as an invasive cardiology procedure.
recurrent coarctation
ant caud*<-
->ceph
post
20-23. Repair is performed using synthetic patch angioplasty. A longitudinal aortotomy is made from the distal arch, across the recurrent stenosis, and onto the upper descending thoracic aorta.
FIGURE
20
Coarctation of the Aorta 437
patch
ant caud<-
-^ceph
post
20-24. A Dacron® patch is stitched over the aortotomy to relieve the obstruction. Alternatively, a Gore-Tex® patch may be used.
FIGURE
20-3. Subclavian Artery Translocation Angioplasty This operation can be performed at any age, but is simpler to carry out in an older child with a larger aorta and subclavian artery. Extensive dissection of the left subclavian artery and its branches is necessary, as is dissection of much of the thoracic aorta. The repair uses viable tissue without the need for a circumferential suture line. Normal blood flow through the subclavian artery is preserved.
aortic arch Internal mammary artery left subclavian artery vertebral artery
ant caud<-
-^•ceph
coarctation
post
20-25. The distal aortic arch, left subclavian artery and its branches, and descending thoracic aorta are widely dissected. The ligamentum arteriosum or ductus arteriosus is divided for maximal aortic mobilization.
FIGURE
438 Color Atlas of Congenital Heart Surgery
left subclavian artery vertebral artery ant caud<-
-•ceph
post FIGURE 20-26. Mobilization of the subclavian artery is accomplished by dissecting all branches of this vessel at the thoracic inlet.
coarctation
left subclavian artery patch
ant caud<-
-•ceph
post
20-27. The subclavian artery is divided at its origin from the aorta, and the medial aspect is incised to near the origin of the vertebral artery. The aortotomy is extended caudad to a point beyond the coarctation. The proximal subclavian artery is shifted caudad and the opened artery is anastomosed to the large aortotomy. The posterior anastomosis is performed, first working within the lumen of the aorta. FIGURE
20
Coarctation of the Aorta 439
distal arch distal left subclavian artery subclavian artery patch
ant 4caud^- -•ceph post
FIGURE 20-28. The anterior anastomosis completes the repair. The subclavian artery is intact but has been shifted caudad. Its wide base overlies the area of the coarctation, significantly enlarging the aorta at that site.
20-4. Dacron® Patch Angioplasty Advantages of this operation include its simplicity, without the need for extensive aortic dissection, and relief of the aortic obstruction is usually successful. This repair is used only as a last resort, when other repairs are not applicable because of the late complication of aneurysm formation in a significant number of patients.
aortic arch
left subclavian artery coarctation
ant caud<-
-**ceph
post
20-29. The pleura is incised and the nerves are reflected medially. The distal arch, proximal left subclavian artery, and upper thoracic aorta are dissected.
FIGURE
440 Color Atlas of Congenital Heart Surgery
coarctation
ant caud<-
-^ceph
post FIGURE 20-30.
An incision is made from the base of the subclavian artery across the coarctation and into the descending thoracic aorta.
Dacron patch ant caud<-
-•ceph
post
20-31. The repair is completed by suturing a Dacron® patch over the aortotomy. A Gore-Tex® patch may be used as an alternative.
FIGURE
20
Coarctation of the Aorta 441
FIGURE 20-32. In this child, 7 years after initial Dacron® patch angioplasty, an aneurysm was suspected after examining a plain chest film. The angiogram reveals a large aortic dilatation into the base of the left subclavian artery.
tape around arch aneurysm
descending aorta
ant caud^-
-^ceph
post
20-33. At surgery a large thoracic aneurysm is found. The aorta is mobilized for proximal and distal control. Repair is performed with partial cardiopulmonary bypass and exclusion of the aneurysm between aortic clamps. One cannula for venous return to the pump is placed in the left atrial appendage, and the arterial perfusion cannula is placed in the descending thoracic aorta. Normal flow from the functioning left ventricle will perfuse the head and right subclavian artery.
FIGURE
442 Color Atlas of Congenital Heart Surgery
aneurysm sac
ant caud*<-
->ceph
post FIGURE 20-34. After establishing partial bypass and then excluding the aneurysm between vascular clamps, the thin-walled aneurysm is entered.
Dacron patch
ant caud<-
-•ceph
post
20-35. After opening the aneurysm widely, intercostal vessels entering the sac are oversewn. The previously placed Dacron® patch is seen in the posterior sac.
FIGURE
20
Coarctation of the Aorta 443
left subclavian artery graft aortic graft ant -•ceph
caud<-
post
20-36. A woven tubular Dacron® conduit is inserted within the aneurysm to replace the aorta. A smaller conduit as a side arm replaces the proximal left subclavian artery.
FIGURE
ant caud^-
-•ceph
post FIGURE
20-37. For hemostasis, the aneurysm sac is stitched around the Dacron® conduit.
21
Patent Ductus Arteriosus
Repair of this anomaly is usually performed through a left fourth interspace thoracotomy. In rare cases, when the ductus is located in the right chest, a right fourth interspace incision is used. Division of the isolated ductus rather than ligation is used, except in premature infants or before cardiopulmonary bypass when working through a median sternotomy. Division is safer and should avoid ductal fracture or incomplete closure, which are risks of ligation.
vagus nerve distal arch left subclavian artery
ant caud<-
ductus arteriosus
->ceph
post
21-1. The parietal pleura is incised over the left subclavian artery and upper descending aorta to below the anticipated area of the ductus arteriosus. Loose adventitial tissue has also been incised over these structures. The vagus nerve is reflected anteriorly and stay sutures are inserted in the pleura and draped over the lung. The proximal left subclavian artery is dissected and followed caudad to identify the distal aortic arch. Tissue immediately beneath the arch is incised and this readily allows identification of the cephalad portion of the ductus arteriosus. FIGURE
444
21
Patent Ductus Arteriosus 445
pericardial lappet of Gross ductus arteriosus
ant caud<-
->ceph
post FIGURE 21-2. The ductus is dissected along its caudad margin, taking care to avoid injury to the recurrent laryngeal nerve that courses around it. The posterior aspect of the ductus is dissected, and a right-angle clamp is passed around the ductus to identify the remaining posterior fibrous strands of tissue that are to be divided. The pericardial lappet of Gross, located over the anterior surface of the ductus, is dissected from the ductal structure. The ductus can be seen entering the pulmonary artery, and this defines the extent of the dissection. Wide dissection is necessary to provide space on the ductus to apply vascular clamps.
corner stitch pulmonary artery end of ductus aortic end of ductus
ant caud^-
-^•ceph
post
21-3. Vascular clamps are applied to each end of the ductus, after which it is divided, leaving an ample ductal stump near each vascular clamp. The pulmonary artery end of the ductus is sutured first. Using a continuous monofilament stitch, a running locking suture is placed as the first layer of the closure. The aortic stump of the ductus is retracted.
FIGURE
446 Color Atlas of Congenital Heart Surgery
pulmonary artery stump
ant caud<-
-•ceph
post
21-4. After the first layer of the pulmonary artery stump closure is completed, the same continuous suture is placed as an over-and-over stitch, forming the second layer of the closure. The suture is tied to the original corner stitch that is placed at the time of the first layer closure. FIGURE
pulmonary artery end of ductus
recurrent laryngeal nerve aortic end of ductus
ant caud^-
-^•ceph
post
21-5. The aortic stump of the ductus is closed with a similar two-layer closure stitch. A sponge is packed between the two ductal stumps and the vascular clamps removed. When needle-hole bleeding stops, the sponge is removed. For more significant bleeding, adventitial figure-of-eight stitches are placed over the stump. The recurrent laryngeal nerve is seen arising from the vagus nerve, after which it passes behind the ductus stump and into the mediastinum posteriorly. FIGURE
21
Patent Ductus Arteriosus 447
ductus arteriosus
ant caud<-
-^ceph
post
21-6. In another infant who is small and premature, the thoracotomy incision is short and posterior in the fourth interspace. A limited parietal pleura incision is made over the patent ductus. The distal arch is identified, below which the ductus arteriosus is located. Limited dissection is performed freeing only the ductus laterally, cephalad, and caudad, and medially behind the ductus.
FIGURE
ant caud^-
-•ceph
post FIGURE
21-7. A suture buttressed with Teflon® felt pledgets is placed around the ductus.
448 Color Atlas of Congenital Heart Surgery
ant caud^-
-•ceph
post FIGURE 21-8. The ductus is ligated. The felt pledgets provide support for the ligature, which helps avoid laceration of the ductus.
ductus arteriosus
caud^ post
21-9. In another premature infant, limited dissection of the ductus is performed anteriorly, cephalad, and caudad to that structure.
FIGURE
21
Patent Ductus Arteriosus 449
ductal closure clip
ant caud<-
-•ceph
post FIGURE 21-10. The ductus is then ligated with a single hemoclip. I currently prefer this technique in premature infants because the mediastinal surface of the ductus does not require dissection.
ductus arteriosus
main pulmonary artery
aorta
cept R«-
-•L
caud
21-11. In another child, intracardiac repair of anomalies will be performed using cardiopulmonary bypass while working through a median sternotomy. An associated patent ductus arteriosus is dissected near the distal main pulmonary artery. It is identified by locating the proximal right and left pulmonary arteries. The ductus is found between these two vessels and is ligated with a hemoclip. FIGURE
450 Color Atlas of Congenital Heart Surgery
repaired ductus
main pulmonary artery aorta cept
R<-
->L
caud
21-12. In another patient, internal ductal closure is performed. The child is placed on cardiopulmonary bypass, and a longitudinal incision is made in the distal main pulmonary artery. For improved exposure in the blood-filled field, pump flow can be significantly reduced momentarily in conjunction with hypothermia. The ductus is repaired with interrupted sutures that are placed within the main pulmonary artery. After the first stitch is placed and tension is placed on it, hemostasis is usually adequate for exposure, and pump flow can be increased to normal. FIGURE
22
Vascular Ring and Vascular Sling
Vascular ring operations are performed through a left third or fourth interspace thoracotomy and all anatomic structures are dissected. Angiography is rarely necessary to define vascular anatomy, although magnetic resonance imaging (MRI) or computerized tomography (CT) is used by many surgeons for this purpose. Vascular sling repair is also performed through a left thoracotomy, except in cases when associated intracardiac surgery is carried out. In this circumstance, a median sternotomy approach is used. Magnetic resonance imaging, CT, or angiography is helpful in defining this anomaly. An aortopexy is part of most vascular ring repairs because this pulls the pulsatile aorta away from the trachea. The aorta may continue to obstruct the trachea if left in its original position even after division of the ring.
22-1. Double Aortic Arch
ligamentum arteriosum distal anterior arch left subclavian artery ant caud^-
descending aorta
^ceph
post
22-1. Working through a left thoracotomy, the distal aortic arch and ligamentum arteriosum are dissected.
FIGURE
451
452 Color Atlas of Congenital Heart Surgery
ligamentum stump
ant caud^-
-^•ceph
post
22-2. The ligamentum arteriosum is divided after ligating or suture closing each end to ensure hemostasis. FIGURE
ligamentum stump distal anterior arch
left subclavian artery esophagus
distal posterior arch
ant caud^-
-^•ceph
post
22-3. The distal posterior aortic arch is dissected. Dissection of anterior and posterior arches is necessary to identify the smaller of the two, which will be divided during the repair. The head vessels should be identified before division of the smaller arch to be sure that each has an adequate arch connection to provide satisfactory vessel blood flow after the repair. FIGURE
22
Vascular Ring and Vascular Sling 453
trachea stump of divided posterior arch esophagus ant -•ceph
caud^-
post FIGURE 22-4. The slightly smaller distal posterior arch is divided. The mediastinum is dissected, including the esophagus and trachea, and surrounding fibrous bands near the trachea are incised. If left, these fibrous bands may cause persistent postoperative constriction.
thymus remnant pericardium
ant caud^-
-^•ceph
post
22-5. To prepare for the aortopexy, the left thymic lobe is resected to allow for better approximation of the aorta to the back of the sternum.
FIGURE
454 Color Atlas of Congenital Heart Surgery
adventitial stitch innominate artery proximal arch
ant caud<——• ceph post
22-6. The pericardium is opened and heavy multifilament stitches are placed deep in the adventitia of the distal ascending aorta and/or proximal arch. FIGURE
pericardium
aortopexy stitches
ascending aorta proximal arch
ant caud<-
-^•ceph
post
22-7. The stitches are passed through the adjacent pericardium and attached to the posterior sternum, or they are passed through the sternum and tied. The stitches should be positioned so that, as much as possible, the aorta is pulled directly anterior. FIGURE
22
Vascular Ring and Vascular Sling 455
22-2. Right Arch with Left Ligamentum
esophagus
ligamentum arteriosum left subclavian artery ant ->ceph
caud**-
post FIGURE 22-8. Working through a left thoracotomy, the mediastinal pleura is incised. The ligamentum arteriosum is seen compressing the esophagus.
stumps of divided ligamentum
esophagus
ant caud^-
-^•ceph
post
22-9. The ligamentum arteriosum is divided, after which each end is stitched closed to ensure hemostasis. The constricted esophagus has been released.
FIGURE
456 Color Atlas of Congenital Heart Surgery «
4 ji
I
trachea aortic arch
*
.^NNk "•%
left subclavian artery
ant -•ceph
caud^-
post
22-10. Dissection of the mid and distal aortic arch is carried out, and all fibrous bands lateral to the trachea are incised. If left intact, these bands may constrict the soft trachea of an infant or young child. FIGURE
pericardium
aortopexy stitches ascending aorta main pulmonary artery
ant caud^-
-•ceph
post
22-11. The left thymic lobe has been removed. The pericardium is opened longitudinally and aortopexy stitches are placed in the adventitia of the ascending aorta. FIGURE
22
Vascular Ring and Vascular Sling 457 sternum
aortopexy stitches ascending aorta
ant caud<-
-^•ceph
post
22-12. Aortopexy stitches are passed through the adjacent pericardium and then are passed full thickness through the sternum.
FIGURE
sternum
aortopexy stitches
ascending aorta ant caud*<-
-•ceph
post
22-13. Aortopexy stitches are tied anterior to the sternum, and the aorta is shifted directly anterior and adjacent to the back surface of the sternum.
FIGURE
458 Color Atlas of Congenital Heart Surgery
22-3. Vascular Sling
thymus
phrenic nerve
left pulmonary artery
ant caud<-
-•ceph
post
22-14. Working through a left thoracotomy, the left pulmonary artery is dissected in the posterior mediastinum. FIGURE
main pulmonary artery
divided ligamentum arteriosum
left pulmonary artery
ant caud^-
->ceph
post
22-15. The pericardium is opened anterior to the vagus nerve. The ligamentum arteriosum is divided.
FIGURE
22
Vascular Ring and Vascular Sling 459
main pulmonary artery right pulmonary artery proximal left pulmonary artery ant
cau6<-
-^ceph
post FIGURE 22-16. Within the pericardium, the proximal right pulmonary artery is retracted, and the origin of the anomalous left pulmonary artery is identified arising from it.
divided proximal left pulmonary artery
ant caud^-
-•ceph
post
22-17. The left pulmonary artery is divided using a side-biting clamp on its origin. Hemostasis in the distal vessel is achieved with snares on the branches.
FIGURE
460
Color Atlas of Congenital Heart Surgery
stump of left pulmonary artery
ant caud<-
-•ceph
post
FIGURE 22-18. The proximal stump of the left pulmonary artery is closed with a continuous suture.
main pulmonary artery
left pulmonary artery ant caud<-
-•ceph
post
FIGURE 22-19. The left pulmonary artery is removed from its posterior mediastinal position and shifted forward for an end-to-side anastomosis to the distal main pulmonary artery. This is performed as a closed operation with a side-biting clamp.
22
Vascular Ring and Vascular Sling 461
proximal left pulmonary artery
ascending aorta
right pulmonary artery ceph R<-
->L t caud FIGURE 22-20. In another patient, who will undergo repair of intracardiac anomalies, a median sternotomy incision is used. The left pulmonary artery arises from the proximal right pulmonary artery.
distal left pulmonary artery
ceph R<-
-•L
caud
22-21. The left pleural space is entered and the distal left pulmonary artery is dissected.
FIGURE
462 Color Atlas of Congenital Heart Surgery
ascending aorta
left pulmonary artery main pulmonary artery
22-22. On cardiopulmonary bypass, the left pulmonary artery is divided at its origin and the stump is closed. It is then shifted from the posterior mediastinum and moved forward for an end-to-side anastomosis with the main pulmonary artery. FIGURE
23
Miscellaneous
23-1. Ectopia Cortis Ectopia cortis is a dramatic anomaly even to the experienced heart surgeon. Infants born with this anomaly always have associated midline defects, including an omphalocele and sternal and/or rib deficiencies. Associated intracardiac problems are common. Initial surgery is aimed at covering the heart and abdominal wall defect with skin. Ideally, repair of intracardiac defects is delayed until a later age, as is chest wall reconstruction.
heart
FIGURE 23-1. The ectopic heart is outside the chest wall and uncovered and unprotected. There is a large associated omphalocele.
463
464 Color Atlas of Congenital Heart Surgery
omphalocele
FIGURE 23-2.
In the worst form of ectopia cortis, the heart is tipped up with the apex pointing toward the baby's chin. It is very difficult to manage this problem because shifting the heart caudad often kinks the great vessels, which significantly alters hemodynamics.
area over heart area of previous omphalocele
skin graft
23-3. The omphalocele has been repaired primarily and there is skin coverage over the cardiac structures. To accomplish this, adjacent releasing incisions have been made bilaterally. These areas are closed with skin grafts. The baby had a ventricular septal defect (VSD), which was later repaired. Chest wall reconstruction was also carried out late by filleting the lower anterior rib cage and shifting rib grafts immediately. FIGURE
23
Miscellaneous 465
23-4. Another infant was born with ectopia cortis and a large omphalocele with a short sternum and anterior rib deficiencies. There was 75% skin coverage at birth and the remaining coverage occurred naturally by ingrowth of surrounding skin. The infant returned at this time for definitive intracardiac repair and repair of the omphalocele. The baby has Pentalogy of Cantrell, which includes a large left ventricular diverticulum with a smaller right ventricular diverticulum associated with tetralogy of Fallot. FIGURE
attaching tissue
R^
23-5. The short upper sternum was incised and the heart and omphalocele exposed through a longitudinal incision. There is a large lower pericardial and anterior midline diaphragm deficiency. The upper pericardium is well developed, however, in the lower area of absent pericardium there were adhesions between the heart and surrounding tissue. FIGURE
466
Color Atlas of Congenital Heart Surgery
area of pericardial and diaphragm deficiency
ceph R«-
ectopia cordis or diverticulum
->L
caud
FIGURE 23-6. The large left ventricular diverticulum, which extended into the abdominal space, is exposed and exteriorized.
diverticulum
ceph R <
>L
caud
FIGURE 23-7. The child is placed on cardiopulmonary bypass and excision of the diverticulum is carried out.
23
Miscellaneous 467
apical ventriculotomy
diaphragm deficiency
23-8. A two-layer closure of the diverticulum is performed with interrupted felted mattress sutures followed by an epicardial continuous stitch.
FIGURE
apical ventriculotomy
ceph R«-
- > L '£'
caud
FIGURE 23-9. The ventriculotomy closure is complete.
468 Color Atlas of Congenital Heart Surgery
outflow tract ventriculotomy
muscle bundles
ceph R<-
-•L
t caud
23-10. Because the heart is rotated severely to the right, transatrial repair of the tetralogy was not possible. A short high right ventriculotomy has been made and obstructing muscle bundles exposed and excised. FIGURE
outflow tract patch
R^
23-11. The VSD was closed with a Dacron® patch, after which the outflow tract below the pulmonary valve was reconstructed with a homograft pulmonary artery wall. After completion of the cardiac operation, primary repair of the omphalocele was performed, including abdominal wall repair. The diaphragm deficiency was repaired with a Dacron® patch. FIGURE
23
Miscellaneous 469 short sternum
lower rib cage
area of heart R«*
FIGURE 23-12. One year following the cardiac-omphalocele repair, the same child returned for definitive chest wall reconstruction. The sternotomy scar was entered and subcutaneous tissue dissected bilaterally across the existing lower rib cage to the mid clavicular line. Anterior chest wall muscle was dissected free of the ribs in the same area.
filleted ribs
23-13. The lower anterior ribs were filleted in the longitudinal plane of the rib or coronal body plane leaving the anterior perichondrium intact and attached to the anterior filleted rib sections. FIGURE
470 Color Atlas of Congenital Heart Surgery
left filleted rib patch
right filleted rib patch ceph R<-
-•L
caud FIGURE 23-14. Patches of the anterior filleted ribs were moved centrally to position them for anterior wall reconstruction. In other cases, these filleted ribs may be inverted to allow more effective use of these patches; here, it was more appropriate to leave the patches uninverted.
left and right sided filleted rib patches
R^
23-15. The anterior rib patches were placed in the midline over the area of deficient chest wall and sutured in place. Leaving perichondrium attached posteriorly to the native ribs and anteriorly to the filleted portions allows regrowth of all rib segments. FIGURE
23
Miscellaneous 471
23-2. Permanent Cardiac Pacemaker When a permanent cardiac pacemaker is required in infants or small children, it is accomplished with epicardial wire placement. This avoids insertion of larger transvenous leads in small vessels, reserving that technique for older children.
epicardial lead
ant caud <-
-^•cept
post FIGURE 23-16. A short submammary incision in the fifth intercostal space is made. The pericardium is incised transversely. Ventricular and/or atrial steroid-emitting tip leads are attached to the surface of the heart. The lead is positioned with the tip of the foot plate pointed caudad and the wire looped toward the head. This loop is placed inside of the pericardium and helps to avoid later tension on the foot plate with growth of the baby. The pericardium is closed over the wire.
leads in thorax leads in abdominal
wall
ant caud^-
->ceph
post
23-17. A short transverse incision is made in the left upper abdominal quadrant and a pocket created between the external and internal oblique muscles. Placing the battery pack deep to abdominal wall muscle helps to prevent erosion of the box. If the battery back is placed between the internal oblique and transversalis muscles, there is a greater danger that the abdominal cavity will be entered at a later date when the battery pack is changed. FIGURE
472 Color Atlas of Congenital Heart Surgery
ant caud^-
-•ceph
post
23-18. The wires pass from the thoracic cavity to the abdominal wall after piercing the diaphragm and passing them under the lowest anterior rib. The wires should not be passed between ribs because a kink here increases the risk of wire fracture. FIGURE
box
ant caud<-
-^•ceph
post
23-19. The wires are attached to a battery pack and excess wire is rolled up and placed behind the battery pack in the generous abdominal wall pocket. FIGURE
A Abdominal aorta, 20 Anastomosis, 8, 27,101,104,168,172, 212,218, 230,233, 264, 268, 396, 412 anterior, 7,103,123,144 aortic, 416,421 coronary, 377-378, 380 end-to-end, 421, 426,427,462 extracardiac direct, 24-25 posterior, 7,28,120,143 primary, resection and, 425-427 side-to-side, 126 Aneurysm, 176,374,400-401,441^43 coronary artery, 385 Aneurysmal right pulmonary artery, 177 Angioplasty, balloon, closed, 129. See also Coarctation of aorta Annulus,244,251,269,279 common, 194,197 pulmonary valve, 82,146,151,155, 158,160,180,183,186,190 tricuspid valve, 29-30, 32, 34, 87,130, 136,400 truncal valve, 351, 353 Anomalous collateral arteries, multiple, recruitment of, 208-213 Anomalous left upper lobe pulmonary venous connection, with intact atrial septum, 25-27 Anomalous mitral valve papillary muscle, 254-255 Anomalous origin of left coronary artery coronary artery origin from right pulmonary artery, 378-381 direct re-implantation as repair for, 374-378 intrapulmonary artery tunnel as repair for, 369-374 Anomalous ostium and intramural coronary artery, 382-384
Anomalous papillary muscle extension, 254, 255 Anomalous pulmonary veins, 15,16,17, 18,20,21,22,221,224 Anomalous pulmonary venous connection, total intracardiac to coronary sinus, 225-227 intracardiac to right atrium, 223-225 subdiaphragmatic, 228-230 supracardiac, 214-219 supracardiac at superior vena cava to right atrial junction, 220-223 Anomalous systemic venous return, 1-8 left superior vena cava draining to roof of left atrium, 2-4 right superior vena cava draining to roof of left atrium, 4-8 Anterior-posterior window, 371, 372, 393 Aorta, 15, 66,171,193,195, 289, 382, 450. See also Coarctation of aorta abdominal, 20 ascending, 27, 97,101,124,125,126, 128,143-144,165,166,168,169, 170,171,172,177-181,183,188, 201,220,228, 259,263,265,275, 282,283,286,287,288,297,298, 313, 316, 319, 322, 344, 348, 349, 355, 362, 363, 364,365, 369, 374, 378, 380, 385, 388, 393, 395, 397, 402,405,409,413,416, 420, 423, 454,456, 457, 461,462 descending, 210, 212,404,405, 413, 415,417-420,423,441,451 native, 281 proximal ascending, 83, 84,246, 254, 275,398 thoracic, 417,419 Aortic anastomosis, 416,421 Aortic arch, 425, 426, 427, 428,430,431, 433, 436,437, 439,456 double, 451^54
interrupted, 415-416 transverse, 415 Aortic atresia and hypoplastic left heart syndrome: Norwood procedure with Blalock shunt, 402-414 Aortic cannula, 288, 406 Aortic cannulation, 171 Aortic homograft, 184 Aortic homograft-valved conduit, 348-353 Aortic insufficiency, 9 Aortic isthmus, 427,428 Aorticopulmonary window, 393-394 Aortic origin of pulmonary artery, 395-396 Aortic root anomalies anomalous origin of left coronary artery, 369-381 anomalous ostium and intramural coronary artery, 382-384 aortic atresia and hypoplastic left heart syndrome: Norwood procedure, 402-414 aorticopulmonary window, 393-394 aortic origin of pulmonary artery, 395-396 coronary artery fistula, 385-392 left ventricle to aortic tunnel, 397399 Sinus of Valsalva aneurysm, 400-401 Aortic root enlargement procedures: posterior root enlargement, 268-281 Aortic suture line, 345 Aortic tunnel, 397-399 Aortic valve, 11,77, 82, 83, 86,189,190, 193,196,199,247,251, 256,260, 266,308,317,397,398,399 cusps of, 82, 261, 268, 271, 275, 276 leaflets of, 81, 264, 279,401 Aortic wall, 85 Aortopexy, 371,453,454,456, 457
474
Index
Aortotomy, 170,257, 268,269,271,272, 274,275, 277,278,283,344, 350, 370, 376, 396,401,404,405,420, 429,431 Apical left ventricle to ascending aorta conduit, 282-284 Arch, 287. See also Aortic arch distal, 444,447,451^53 proximal, 454 right, left ligamentum with, 455^62 transverse, 402 Arrhythmias, 9 Arterial cannula, 409 Arterial switch: Jatene operation, 285-307 Arteries. See Specific arteries Arteriotomy, 102,126,131,170,197, 311, 371 Ascending aorta, 27, 97,101,124,125, 126,128,143-144,165,166,168, 169,170,171,172,177-181,183, 188, 201,220,228, 259,263, 265, 275, 282, 283,286,287,288,297, 298, 313, 316,319, 322,344, 348, 349, 355, 362, 363, 364, 365, 369, 374, 378, 380, 385, 388, 393, 395, 397,402,405,409,413,416,420, 423,454,456,457,461,462 proximal, 83, 84,246,254,275, 398 Ascending aorta conduit, 282-284 Ascending connecting vein, 25,26, 27, 28 Ascending pulmonary venous channel, 215 ASD (atrial septal defect), 2-6, 92,135, 216,221,231,234, 327, 337,407, 421 closure of, 10-11,14,230,233 coronary sinus, 12,108,224,225-227, 332 Dacron® patch for, 44, 57, 58, 63, 64, 65,70,71,219, 222,223,225,227, 236, 273 after failed device closure, 12 isolated, 10-14 with partial pulmonary venous connection, 10-11,15-28 primum, 38-41,45, 50, 52, 57, 61 residual, 9,12 secundum, 45 tricuspid insufficiency and, 29-36 Atretic outflow tract, 198 Atretic valve, 137 Atrial appendage left, 26,27,28,42,232 right, 7, 335 Atrial chambers, 239 distal left, 238 proximal left, 238 Atrial junction, right, 220-223
Atrial Atrial Atrial Atrial
obstructive lesions, left, 237-244 septal defect. See ASD septal flaps, 329,330, 331 septum, 2, 3,4,21, 22, 52, 218, 233,234, 328, 331, 332 intact, 25-27 Atriotomy, 4, 7, 8,15,16,20, 21, 38,41, 45, 53, 74,75, 79, 89, 92, 93,106, 118,122,133,216, 217,219,229, 230, 320,327, 333,334, 335, 336, 337, 386,421 Atrio-ventricular valves. See AV valve Atrium, 1,11 common, endocardial cushion defects of, 41^13 left, 2-A, 4-8, 24,25,228,229, 237, 349 right, 15,17,18,20,21, 23, 32, 33, 88, 117,118,121,223-225,335 Autograft, 278,279,280,281, 339 AV canal. See Complete AV canal AV node, 225, 227 AV (atrio-ventricular) valve, 37, 38,45, 46, 53,127 AV valve leaflets, 45^18, 53, 54, 55, 59, 65, 67, 68, 69,101 Azygos vein, 15,17,101,140, 213 B Balloon angioplasty, closed, 129 Balloon septostomy, 135 Balloon valvuloplasty, 135 Bicaval cannulation, xiii-xiv, 285, 326 Bidirectional caval pulmonary shunt, 105 Blalock-Taussig (subclavian artery to pulmonary artery) shunt, 138-139,140,209 left, 166 right, 165-166 Blind main pulmonary artery pouch, 104,105,106,107,113,114 Blind superior vena cava pouch, 106, 113,114 Bulboventricular foramen enlargement of, by resection of septal tissue, 127-128 obstruction of, 124-128 restrictive, 128 C Cannula, 2,4 aortic, 288, 406 arterial, 409 caval, 2,15,16,18,19, 20 Cannulation aortic, 171 bicaval, xiii-xiv, 285, 326 high, of superior vena cava, 2-4,101 Cardiac catheterization, 1, 9
Cardiac hernia sac, 467 Cardiac pacemaker, permanent, 4 7 1 ^ 7 2 Cardiopulmonary bypass, xiii, xiv, 1 Carotid artery, 417 common, 416 left, 287,402,415, 432,433, 434,441 right, 418 Catheterization, cardiac, 1, 9 Cava atrial junction, 4,15,17, 20,101, 328 Caval cannula, 2,19, 20 superior vena, 15,16,18 Caval drainage, high superior, of partial pulmonary venous connection, 17-20 Caval tunnels, 326-332, 334,339, 340, 341, 342 Central shunt (ascending aorta to main pulmonary artery), 143-144 Cephalad septum, anterior, 11 Circumflex coronary artery, 164 Closed balloon angioplasty, 129 Closed heart surgery, xiii Coarctation of aorta Dacron® patch angioplasty, 439-443 resection and primary anastomosis, 425-427 subclavian artery flap angioplasty, 428-437 subclavian artery translocation angioplasty, 437^439 Collateral arteries, 208-213 Commissures incised, 130,131,155, 247,262, 267, 304 stenotic, 246 Commissurotomy, 155 Common annulus, 194,197 Common atrium, endocardial cushion defects of, 41-43 Common carotid artery, 416 Common pulmonary veins, 228,229 Common transverse pulmonary vein, 214,217,218 Complete AV canal, 45-65,255 modified single-patch repair, 59-65 tetralogy of Fallot and, 65-73,129 two-patch repair, 53-58 Conduit, 322 aortic homograft-valved, 348-353 ascending aorta, 282-284 Dacron®, 346, 359, 360, 363,443 Gore-Tex®, 410 homograft, 206,207,208, 362 intra-atrial, 123-124 obstructed, late replacement of, 359-364 porcine-valved, valved conduit repair with, 343-348, 360 pulmonary homograft-valved, 353-355
Index Conduit repair extracardiac, 117-123, 321 valved, 343-355, 360 Conduit valve, 309, 310, 347,348 Conus left anterior descending coronary artery, tetralogy of Fallot relating to, 163-165 Conus muscle, 316 Coronary anastomosis, 377-378, 380 Coronary arteries, 301, 304 anterior descending, left, 163-165 circumflex, 164 conus left anterior descending, tetralogy of Fallot relating to, 163-165 intramural, 382-384 left, anomalous origin of coronary artery origin from right pulmonary artery, 378-381 direct re-implantation as repair for, 374-378 intrapulmonary artery tunnel as repair for, 369-374 main left, 193, 261,263,286,293,296, 298, 369-381, 382, 390, 391, 399 main right, 164,193, 263, 275,285,286, 293, 296, 298, 316, 322, 369, 374, 382,383,385, 388 Coronary artery aneurysm, 385 Coronary artery buttons explantation of, 293,294,296, 299, 303, 379, 380 implantation of, 290,292,293,294 left, 290,291, 292,294-296,299 right, 291,292, 293, 294-296,299 Coronary artery fistula, 385-392 Coronary artery origin from right pulmonary artery, 378-381 Coronary cusp left, 83, 84, 85,246, 254 non, 83, 246, 249,260 right, 83, 84, 86, 246,252,254,260 Coronary implant site, flaps at, 292 Coronary sinus ASD, 12,108, 224, 225-227, 332 Corpora arantii, 83, 84 Cor triatriatum, 237-240 membranes relating to, 237-240 D Dacron® conduit, 346,359, 360, 363, 443 Dacron® patch. See Patch Dacron® patch angioplasty, 439443 Descending aorta, 210,212,404,405, 413,415, 417-420,423,441,451 DeVega annuloplasty, 29-30 Diaphragm deficiency, 466,467 Direct re-implantation, 374-378
Distal arch, 444,447 anterior, 451,452 posterior, 452,453 Distal left atrial chambers, 238 Distal right pulmonary artery, 178,179 Diverticulum, 466-467 Double aortic arch, 451-454 Double chamber right ventricle, 131— 134 Double outlet ventricle left, 193-197 right, 188-192 Double ventricle, left, 35-36 Double-switch operation, 319,326-342 d-Transposition of great arteries arterial switch: Jatene operation, 285-307 Kawashima operation, 316-318 Rastelli operation, 307-315, 326 Ductal stump, 396,406,416, 435 E Ebstein's anomaly, 29 mild, 30-32 severe, 32-36 Ectopia cortis, 463—470 Ectopic heart, 463 Endocardial cushion defects common atrium, AI-A3 complete atrioventricularis communis or AV canal, 45-65 tetralogy of Fallot and, 65-73,129 inlet VSD, 43-44 primum ASD, 38-41,45, 50,52, 57, 61 Endocardial incision, 328 Endocardium, 222, 328, 338 Endothelialized patch, 79 Epicardial bead, 471 Esophagus, 452,453,455 Eustachian valve, 108,109 Extracardiac conduit repair, 117-123, 321 Extracardiac direct anastomosis, 24-25 F Failed device closure, ASD after, 12 Fenestration, 109-112,115,116,121,122, 123,124 Fibromuscular ledge, 248 Fibrous collar, 250 Fontan operation, 135 bulboventricular foramen, obstruction of, relating to, 124-128 first-stage, 101-105 second-stage, 101,103,402 extracardiac conduit repair, 117-123, 321 intra-atrial conduit, 123-124 intra-atrial lateral tunnel repair, 106-116,123
475
Formalin infiltration, 135,137 Fossa ovalis, 10,22 G Gore-Tex® conduit, 410 Gore-Tex® graft, 140,142,167,402, 403, 406,407,408,409,411^114,418 Gore-Tex® hood, 355, 362 Gore-Tex® patch, 18,19,72,101,108, 109,110,115,119,126,127,160, 192,200, 314, 315, 364, 435, 437, 440 Grafts, 141. See also Autograft; GoreTex® graft; Homograft; PTFE Great arteries. See d-Transposition of great arteries; I-Transposition of great arteries Great vessels, 297,299, 300,303, 304, 316,464 H Heart. See also Hypoplastic left heart syndrome ectopic, 463 retracting of, 21 surgery for closed, xiii open, xiii Heart syndrome, left, hypoplastic, 402-414 High cannulation, of superior vena cava, 2-4,101 High marginal branches, 164 High superior caval drainage, of partial pulmonary venous connection, 17-20 His bundle, 39, 40,43,44, 50, 51, 58, 62, 71,76, 88, 92,127,148,149,190, 227,257, 272, 320, 321, 323, 324 Homograft, 173-177,185,186,187, 206-208, 277,278,281, 312, 314 aortic, 184 pulmonary, 361, 362 Homograft conduit, 206, 207, 208, 362 Homograft mitral leaflet, 352, 353, 361 Homograft patch, 161,162,163, 204,205, 264,276, 278, 303, 304, 305, 403, 404,406,410,411,414 Homograft pulmonary valve cusp, 161, 162,163,186 Homograft valve, 275-278 Homograft-valved conduit aortic, 348-353 pulmonary, 353-355 Hypoplasia, 135,136 Hypoplastic left heart syndrome, 140, 402-414 Hypoplastic right ventricular outflow tract, repair of, 157-163
476
Index
I Incised commissures, 130,131,155, 247, 262, 267, 304 Inferior vena cava, 1,21, 23, 24,101,116, 117,118,119, 327, 337 Infundibular stenosis, 163 double chamber right ventricle and, 131-134 Infundibulum, 156,157,160,196, 200. See also Os infundibulum Innominate artery, 137,138,140,141, 166, 287,402,408,409,454 Innominate vein, 17, 25, 215 Intact atrial septum, 25-27 Intercostal space thoracotomy, 24, 25, 97,138,139, 212, 364,451 Internal mammary artery, 437 Interrupted aortic arch Type A, 415^16 Type B, 416-424 Type C, 415 Intimal fibrous ridge, 98, 99 Intra-atrial baffle, 101,112,117 Intra-atrial conduit, 123-124 Intra-atrial lateral tunnel, 123 repair of, 106-116 Intra-atrial procedures, xiii Intracardiac baffle, 113 Intracardiac tunnel repair, 21-23 Intramural coronary artery, 382-384 Intrapulmonary artery tunnel, 369374 Isolated ASD, 10-14 I-Transposition of great arteries double-switch operation, 319, 326342 with VSD and pulmonic stenosis, 319-326 J Jatene operation, 285-307 K Kawashima operation, 316-318 Konno procedure. See also Ross-Konno procedure with homograft valve, 275-278 modified, 255-259 with prosthetic valve, 271-274 L Late replacement, of obstructed conduit, 359-364 Leaflets of aortic valve, 81,264, 279, 401 AV valve, 45-48, 53, 54, 55, 59, 65, 67, 68, 69,101 mitral, 38-42,48-49, 51, 56,184,185, 244, 269, 276, 312, 323, 324 homograft, 352, 353, 361
pulmonary valve, 80, 81,130,137,154, 155 of tricuspid valve, 29-31, 3 3 - 3 5 , 4 3 ^ 5 , 44, 51,52, 75,76,77, 87, 88, 89, 91, 92, 93,133,148,149,151,153,183, 194,199 Le Compte maneuver, 297 Ligamentum arteriosum, 451,452,455, 458 Ligamentum, left, right arch with, 455^162 Ligamentum stump, 452, 455 M Mammary artery, internal, 437 Membranes cor triatriatum relating to, 237-240 supramitral ring relating to, 241-244 Membranous ventricular septum, 79, 88, 149,194 Membranous VSD, 133 Mitral leaflet, 38^2,48-49, 51, 56,184, 185, 244,269, 276,312, 323, 324 homograft, 352, 353, 361 Mitral leaflet tissue deficiency, primum ASD with, 40-41 Mitral stenosis, 241 Mitral valve, 41,42, 237,239, 240, 242, 320, 325 function of, 9 Mitral valve chords, 255 Mitral valve cleft, 39,40-41,70 Mitral valve papillary muscle, anomalous, 254-255 Moderator band, 132,133,134 Modified Blalock-Taussig (subclavian artery to pulmonary artery) shunt, 140-142,211, 213,407 Modified Konno procedure, 255259 Modified Norwood procedure, with RV to PA shunt, 408^14 Modified single-patch repair, for complete AV canal, 59-65 Multiple anomalous collateral arteries, recruitment of, 208-213 Muscle bands, 152 Muscles. See also Papillary muscles conus, 316 Muscular infundibular floor, 146,148, 150 Mustard procedure, 326 Myocardial fibrosis, 373 N Native aorta, 281 Native vessel, 304, 305 Neoaorta, 297-302, 305, 306,405, 406, 414 Neointima, 360, 363
Norwood procedure Blalock shunt with, 402-414 modified, RV to PA shunt with, 408^414 O Omphalocele, 463,464,465 Open heart surgery, xiii Os infundibulum, 131,132,134,146,164 Osteal stenosis, 365, 366 Outflow tract atretic, 198 pulmonary ventricle, 319, 320, 322, 325 ventricular, left, 249, 273 ventricular, right, 256, 271, 274, 278, 318 reconstruction of, 138,144-145 Outflow tract patch, 259,468 Outflow tract reconstruction. See Tetralogy of Fallot P Pacemaker, cardiac, permanent, 471-472 Palliation with plication, 364-367 repair and, of tetralogy of Fallot, with absent pulmonary valve syndrome, 177-187 stage 1,402 surgical, 135,138 Palliative shunts and outflow tract reconstruction, 163 central shunt (ascending aorta to main pulmonary artery), 143-144 classic Blalock-Taussig (subclavian artery to pulmonary artery) shunt, 138-139,140,165-166, 209, 285 modified Blalock-Taussig (subclavian artery to pulmonary artery) shunt, 140-142,211,213,407 reconstruction of right ventricular outflow tract, 144-145 Papillary muscle extension, anomalous, 254, 255 Papillary muscles, 90, 91,132,149, 243 mitral valve, anomalous, 254-255 tricuspid valve, 132 Parietal band, 131,132,133,134,146, 147,151,152, 302 Partial pulmonary venous connection ASD with, 10-11,15-28 high superior caval drainage of, 17-20 Patch. See also Complete AV canal; PTFE Dacron®, 3,4, 6,11,16,17,19,22, 23, 43, 50, 51, 52,159,165, 437, 442 for ASD, 44, 57, 58, 63, 64, 65, 70, 71, 219,222, 223, 225,227, 236, 273
Index for VSD, 55, 56, 59, 65, 69,70, 72, 74, 78, 81, 87, 89, 91, 92, 94,134, 150,153,154,184,191,194,195, 197,199, 201, 203, 204,258, 272, 273, 274, 280, 281, 308, 309, 311, 317, 321, 322, 324, 346,351, 357, 394, 399,422, 424,468 endothelialized, 79 Gore-Tex®, 18,19,72,101,108,109, 110,115,119,126,127,160,192, 200, 314, 315, 364, 435,437, 440 homograft, 161,162,163,204,205, 264, 276, 278, 303, 304, 305,403,404, 406,410,411,414 outflow tract, 259, 468 pericardial, 17,20, 23,41,72,100,104, 114,157,158,159,171,200, 269, 270, 274, 300, 301, 303,330, 340, 341, 342, 350, 351, 353,358, 372, 378, 381 SILASTIC, 307 subclavian artery, 438,439 synthetic, 261,262,326 transannular, 146,151,173-177 Patch angioplasty, Dacron®, 439^43 Patch repair, valveless, 355-358 Patent ductus arteriosus, 137,228, 288, 289, 395,404, 413, 415,416, 417, 419, 425-427, 431,434, 437, 444-450 Pedicle flaps, of right atrium, 17,18,20 Pentalogy of Cantrell, 465 Pericardial autograft, 339 Pericardial lappet of Gross, 445 Pericardial patch, 17,20,23,41,72,100, 104,114,157,158,159,171,200, 269, 270, 274, 300, 301, 303, 330, 340, 341, 342, 350, 351,353, 358, 372, 378, 381 Pericardiotomy, 97 Pericardium, 24,26,117,215,269,453, 454, 456,458, 459,465,471 Perimembranous VSD, 74-79,152 Permanent cardiac pacemaker, 471^72 Phrenic nerve, 24,458 Polytetrafluoroethylene grafts. See PTFE grafts/patch Porcine-valved conduit, valved conduit repair with, 343-348, 360 Potts shunts, 172 Primum ASD as endocardial cushion defects, 38^41, 45, 50, 52,57, 61 with mitral leaflet tissue deficiency, 40-41 Prosthetic valve, 270-274 Proximal arch, 454 Proximal ascending aorta, 83, 84,246, 254,275,398 Proximal left atrial chamber, 238
Proximal muscle rim, 354 Proximal pulmonary artery left, 204 main, 195, 358, 375,403,405, 406,412, 413 right, 178 PTFE (polytetrafluoroethylene) grafts/patch, 140,145 Pulmonary arteries, 344, 346, 355, 358, 360, 361, 364, 365,366,445. See also Palliative shunts and outflow tract reconstruction aortic origin of, 395-396 bifurcation of, 303 left, 26,116,139,141,142,172,175, 181-183, 205,207, 209-211,220, 223,224,225, 228,287, 306, 344, 348, 350, 364, 367, 393, 458,459, 460, 461, 462 main, 27, 80, 96, 97-100,101,102,103, 104,105,125,126,130,131,143144,145,151,154,157,162,172, 179,180,188,193,195, 200, 201, 204, 215, 220, 228,282, 286, 287, 290, 296,297, 298, 303, 309, 313, 316, 317, 319, 322, 325, 326, 369, 370, 374, 375, 376,382, 393,395, 396, 397,402, 409,416,449,450, 456,458,459,460,462 neomain, 303, 304, 305, 306 proximal left, 204 main, 195, 358, 375,403,405,406, 412, 413 right, 178 right, 25,105,140,141,165,168,169, 170,171,172,175,177,178,179, 180-183,212, 213,214, 220,223, 225,226,306, 349,350, 356, 364, 367, 378-381, 393, 394, 395, 396, 459,461 Pulmonary artery band, 125 Pulmonary artery banding and band removal, at subsequent total repair, 96-100 Pulmonary artery branches, 119,411 Pulmonary artery pouch, blind main, 104,105,106,107,113,114 Pulmonary artery stump, 446 Pulmonary atresia intact ventricular septum and, 135-137 with VSD, 311 multiple anomalous collateral arteries, recruitment of, 208213 total repair of, 198-208 Pulmonary homograft, 361, 362 Pulmonary homograft-valved conduit, 353-355
477
Pulmonary shunt, 135 bidirectional caval, 105 Pulmonary stenosis, 313 infundibular stenosis and double chamber right ventricle, 131-134 valvular, 129-131 Pulmonary valve, 79,144,154,189,193, 194, 317, 325, 370, 375 trileafed, 130 Pulmonary valve annulus, 82,146,151, 155,158,160,180,183,186,190 Pulmonary valve cusp, homograft, 161, 162,163,186 Pulmonary valve leaflets, 80, 81,137, 154,155 Pulmonary valve placement, with transannular patch, 173-177 Pulmonary valve syndrome, absent, palliation and repair of, 177-187 Pulmonary valvotomy, 129 Pulmonary vascular obstructive disease, 96 Pulmonary veins, 4, 6,10, 27,242 anomalous, 15,16,17,18,20,21,22, 221,224 common, 228, 229 common transverse, 214, 217, 218 left, 26,231, 338 lower lobe, 25,26, 232 upper lobe, 25, 26, 232 right, 20, 231, 234,235, 237, 327, 338 lower, 217, 228, 238, 239, 333 upper, 217,228,238, 332, 334 Pulmonary vein stenosis, 231-236 Pulmonary venous anomalies pulmonary vein stenosis, 231-236 total anomalous pulmonary venous connection intracardiac to coronary sinus, 225-227 intracardiac to right atrium, 223-225 subdiaphragmatic, 228-230 supracardiac, 214-219 supracardiac at superior vena cava to right atrial junction, 220-223 Pulmonary venous channel, ascending, 215 Pulmonary venous connection anomalous left upper lobe, with intact atrial septum, 25-27 partial ASD with, 10-11,15-28 high superior caval drainage of, 17-20 Pulmonary ventricle, 319 Pulmonary ventricle outflow tract, 319, 320, 322, 325 Pulmonic stenosis, 319-326 Pulmonic valve, 93,196,197
478
Index
Punch aortotomy, 371 Purse-string stitch, 110, 111, 112,122, 123,402,418 R Rastelli operation, 307-315, 326 Reconstruction. See Palliative shunts and outflow tract reconstruction Recurrent laryngeal nerve, 446 Resection fibromuscular obstruction relating to, 247,248,249,250, 252,253 primary anastomosis and, 425-427 of septal tissue, 127-128 Residual ASD, 9,12 Ribs, 469-470 Right ventricular dilatation. See RV dilatation Ross-Konno procedure, 278-281, 285 RV (right ventricular) dilatation, 9 S SA (sinoatrial) node, 10,15,17,18,106, 107,115,117, 331, 334 Scimitar's syndrome, 20-23 Scimitar's vein, 24,25 Secundum ASD, 45 Senning operation, 326, 327-336 Septal band, 146,147,156 Septal defects. See ASD; VSD Septal flaps, atrial, 329, 330, 331 Septal tissue, resection of, 127-128 Septostomy, balloon, 135 Septum, 226,227, 239,240 atrial, 2, 3,4,21,22, 52,218,233,234, 328, 331, 332 intact, 25-27 cephalad, anterior, 11 ventricular, 189,190,193,196, 249, 256,271,272,276,320 intact, 135-137 membranous, 79, 88,149,194 Shunts. See also Norwood procedure; Palliative shunts and outflow tract reconstruction Potts, 172 pulmonary, 135 bidirectional caval, 105 repair of, 165-172 systemic-to-pulmonary artery, 138 Waterston, 168-171 Sideris occluder, 12,13 SILASTIC patch, 307 Sinoatrial node. See SA node Sinus, coronary, ASD, 12,108, 224, 225-227, 332 Sinuses of Valsalva, 83, 84,259,260, 261, 263,266,277,279,281, 285,298, 300,304 Sinus of Valsalva aneurysm, 400-401
Stage I palliation, 402 Stenosing ring. See Supravalvar stenosing ring Stenosis, 423 infundibular, 131-134,163 mitral, 241 osteal, 365, 366 pulmonary, 313 pulmonary vein, 231-236 pulmonic, 319-326 subaortic, 247-255 supravalvar, 259-268 truncal valve, 367-368 valvular, 245-284 valvular pulmonary, 129-131 Stenotic commissures, 246 Sternotomy, median, xiii, 27, 282, 416, 461,469 Sternum, 457,465,469 Straddling tricuspid valve, 89-91 Subaortic stenosis: fibromuscular obstruction, 247-255 resection relating to, 247,248, 249, 250,252,253 Subclavian arteries, 25,138,139,140, 141,142,165,166,287, 404,405, 415,417,420,425,426,427,428, 431,432, 433, 437,438, 441,444, 451,452,455, 456. See also Palliative shunts and outflow tract reconstruction Subclavian artery flap, 429^35 Subclavian artery flap angioplasty, 428-437 Subclavian artery patch, 438, 439 Subclavian artery translocation angioplasty, 437^439 Subclavian artery trunk left, 166 right, 165,166 Subpulmonary VSD, 74,79-87 supracristal, 79 Subvalvar chamber, 164 Sulcus terminalis, 112, 327, 331, 332 Superior vena cava, 15,103,105,140, 165,178,179, 214, 220-223, 327, 328, 329, 331, 334, 337 high cannulation of, 2-4,101 left, 1,2-A lower segment of, 5,17,18,19 right, 1, 4-8 upper segment of, 5,7,19,20 Superior vena caval cannula, 15,16, 18 Superior vena cava pouch, blind, 106, 113,114 Supracardiac connection, 214-219 at superior vena cava to right atrial junction, 220-223 Supracristal subpulmonary VSD, 79
Supramitral ring, 241-244 membrane relating to, 241-244 Supravalvar stenosing ring, 260, 261, 263, 265 Supravalvar stenosis, 259-268 Surgery, heart, xiii Surgical palliation, 135,138 Synthetic patch, 261, 262, 326 Systemic-to-pulmonary artery shunt, 138 Systemic venous return, anomalous, 1-8 Systemic ventricle, 319 T Takeuchi procedure. See Intrapulmonary artery tunnel Tetralogy of Fallot, 129,190, 465 complete AV canal and, 65-73,129 with conus left anterior descending coronary artery, repair of, 163-165 hypoplastic right ventricular outflow tract, repair of, 157-163 palliation and repair of, with absent pulmonary valve syndrome, 177-187 palliative shunts and outflow tract reconstruction, 138-145,163 pulmonary valve placement with transannular patch after repair of, 173-177 shunts, repair of, 165-172 total repair of transatrial, 151-157 transventricular, 146-150 Thoracic aorta, 417,419 Thoracotomy intercostal space, 24,25, 97,138,139, 212, 364,451 left, 141, 209,415,425,428, 451, 455, 458 right, 25, 97,140,177 Thromboembolism, 9 Thymus, 458 Thymus remnant, 453 Trabeculations, 93, 94,146 Trachea, 453,456 Transannular patch, 146,151 pulmonary valve placement with, 173-177 Transatrial. See Tetralogy of Fallot Transventricular. See Tetralogy of Fallot Transverse aortic arch, 415 Transverse arch, 402 Transverse pulmonary vein, common, 214,217,218 Tricuspid atresia, 29 Tricuspid insufficiency, 135 ASD and, 29-36 DeVega annuloplasty for, 29-30
Index Tricuspid valve, 10,14, 42,129,157,224, 335, 386,400,422 duplication of, with double inlet left ventricle, 35-36 hypoplasia of, 135 leaflets, 29-31,33-35, 43-45,44, 51, 52, 75,76,77,87, 88, 89, 91, 92, 93, 133,148,149,151,153,183,194, 199 straddling, 89-91 Tricuspid valve annulus, 29-30, 32, 34, 87,130,136,400 Tricuspid valve anomalies Ebstein's anomaly, 29,30-36 tricuspid insufficiency, 29-30 Tricuspid valve papillary muscle, 132 Trileafed pulmonary valve, 130 Truncal valve, 356, 367, 368 Truncal valve annulus, 351, 353 Truncal valve stenosis, 367-368 Truncal vessel, 344, 348, 350,356,364, 365,366 Truncus arteriosus late replacement of obstructed conduit, 359-364 palliation with plication, 364-367 truncal valve stenosis, 367-368 valved conduit repair, 343-355,360 valveless patch repair, 355-358 Two-patch repair, for complete AV canal, 53-58 U Unicuspid valve, 245 V Vagus nerve, 139,140,141, 444 Valvar obstruction, 129 Valvectomy, 135,136 Valved conduit repair aortic homograft-valved conduit, 348-353 with porcine-valved conduit, 343-348, 360 pulmonary homograft-valved conduit, 353-355
Valve leaflets, 272, 363 Valves. See Specific valves Valvotomy, 247, 356, 367, 368 Valvular pulmonary stenosis, 129131 Valvular stenosis, 245-284 aortic root enlargement procedures: posterior root enlargement, 268-281 apical left ventricle to ascending aorta conduit, 282-284 modified Konno procedure for, 255-259 subaortic stenosis: fibromuscular obstruction, 247-255 supravalvar stenosis, 259-268 Valvuloplasty, 82,268 balloon, 135 Vascular ring and vascular sling double aortic arch, 451-454 right arch with left ligamentum, 455^162 Ventricles apical left, to ascending aorta conduit, 282-284 double outlet left, 193-197 right, 188-192 left, 5 to aortic tunnel, 397-399 double inlet, 35-36 pulmonary, 319 right, 135,179 double chamber, 131-134 systemic, 319 Ventricular hypertension, 124,135 Ventricular outflow tract left, 249,273 pulmonary, 319,320, 322, 325 right, 256, 271, 274,278, 318 hypoplastic, 157-163 reconstruction of, 138,144-145 Ventricular septal defect. See VSD Ventricular septum, 189,190,193, 196,249,256, 271,272,276, 320
479
intact, 135-137 membranous, 79, 88,149,194 Ventriculotomy, 91, 95,127,128,131, 144,145,146,158,160,162,164, 185,189,193,197,198, 200,202, 208, 256,257,259, 271,274,275, 277, 278,281,282, 307, 309, 310, 312, 314,315,316, 321, 345,347, 348, 351,352, 353, 354, 355, 358, 359, 361, 364,467,468 Vertebral artery, 437,438 VSD (ventricular septal defect), 38,45, 49, 53, 60, 61, 63, 67, 68,146,183, 189-191,193,196, 257,276, 279, 307, 310,316, 345, 364,464 apical, 74, 95 cephalad margin of, 77 Dacron® patch for, 55, 56, 59, 65, 69, 70, 72,74,78, 81, 87, 89, 91, 92, 94, 134,150,153,154,184,191,194, 195,197,199,201,203, 204, 258, 272,274, 280, 281, 308, 309, 311, 317, 321,322, 324, 346, 351, 357, 394,399,422,424,468 inlet, 87-91 endocardial cushion defects of, 43^14 right, 136 with straddling tricuspid valve, 89-91 membranous, 133 muscular, 74, 91-95 perimembranous, 74-79,152 posterior inferior rim of, 148,149, 153 pulmonary artery banding and band removal at subsequent total repair, 96-100 pulmonary atresia with, 198-213 pulmonic stenosis and, I-Transposition of great arteries with, 319-326 subpulmonary, 74,79-87 supracristal, 79 W Waterston shunt, 168-171
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