Color Atlas of Congenital Heart Surgery Second Edition
Color Atlas of Congenital Heart Surgery Second Edition
S. Ber...
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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 RL 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 RL
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 RL
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 RL
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 RL
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 RL
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'
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
RL
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