Congenital Heart Defects: Decision Making for Cardiac Surgery Volume 2 Less Common Defects PDF
Preview Congenital Heart Defects: Decision Making for Cardiac Surgery Volume 2 Less Common Defects
I Congenital Heart Defects ANTONIO F. CORNO Decision Making for Cardiac Surgery Volume 2 Less Common Defects Dedicated to my loved children Federica, Laura and Jonathan A N T O N IO F. C O R NO Congenital Heart Defects Decision Making for Cardiac Surgery Volume 2 Less Common Defects Foreword by PEDRO J. DEL NIDO With 145 Figures in 340 Separate Illustrations STEINKOPFF ' I iD^AiWRMiKSTimAD»T? ! 1 / 4y Springer ANTONIO E CORNO, MD, FRCS, FETCS Department of Cardiovascular Surgery Centre Hospitalier Universitaire Vaudois (CHUV) 46, rue du Bugnon CH-1011 Lausanne, Switzerland ISBN 978-3-642-62158-1 ISBN 978-3-7985-1934-3 (eBook) DOI 10.1007/978-3-7985-1934-3 Cataloging-in-Publication Data applied for A catalog record for this book is available from the Library of Congress. Bibliographic information published by Die Deutsche Bibliothek Die Deutsche Bibliothek lists this publication in the Deutsche Nationalbibliografie; detailed bibliographic data is available in the Internet at <http://dnb.ddb.de>. This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on mi crofilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Steinkopff Verlag. Violations are liable for prosecution under the German Copy right Law. www.steinkopff.springer.de © Springer-Verlag Berlin Heidelberg 2004 Originally published by Steinkopff Verlag Darmstadt in 2004 Softcover reprint of the hardcover 1st edition 2004 The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guarantee the accuracy of any information about the application of operative techniques and medications contained in this book. In every individual case the user must check such information by consulting the relevant literature. Medical Editor: Sabine Ibkendanz Production: K. Schwind Cover Design: E. Kirchner, Heidelberg Typesetting: K+V Fotosatz GmbH, Beerfelden SPIN 10930595 85/7231-5 4 3 2 1 0 - Printed on acid-free paper Foreword Nearly four decades have passed since attempts at correcting intracardiac de fects in children first began to succeed. In that time great advances have oc curred in our field, including improved diagnostic techniques as well as our ability to manage even the most complex heart defects with relatively low risk and an expected outcome that was unimaginable just twenty years ago. New non-invasive techniques for imaging the heart have largely supplanted more invasive methods such as angiography for the diagnosis of most heart de fects. Continued advances in this area promise to provide the practitioner ac curate quantitative information regarding the hemodynamics and pathophysi ology of simple and complex heart defects that is often not obtainable even with cardiac catheterization. The surgeon facing surgical correction of an anatomic defect in a child today needs to be familiar with all these imaging and diagnostic modalities in order to have a complete understanding of that child's pathology prior to planning surgical management. In turn, decision-making regarding timing of intervention, surgical alterna tives, and optimal surgical approach remains a challenge for surgeons and cardiologists alike. Knowledge of anatomy and pathophysiology must be com plemented with understanding of the potential complications of the various surgical procedures and the expected outcomes. The former has become more difficult as access to morphologic specimens is rapidly becoming a rare phe nomenon, and the latter information is not readily available nor is it easily gleaned from most texts. In today's world, the clinician is expected to make important management decisions not only on the basis of personal experience but also on the accumulated knowledge, often over decades, of rapidly evolv ing practice. Although there is a plethora of textbooks available that provide an encyclo pedic volume of information regarding all the described congenital heart de fects, there is a distinct lack of practical information on how decisions re garding management are made in order to optimize outcome in our patients. The author of this text has taken great care to provide this information in a logical step by step fashion, including interpretation of diagnostic tests, back ground on the morphologic features and common clinical presentation, as well as operative approaches and techniques along with potential pitfalls. This valuable text is arranged in a sequential manner to cover more common de fects in the initial volume, followed by coverage of less common congenital heart defects. The emphasis is on practical information, with ample illustra tions which are essential for an understanding of the morphologic features of the defects as well as the theory and practice of surgical repair. The focus on VI I Foreword decision-making is particularly useful for clinicians treating children with congenital heart defects and this text will be of value to surgeons and cardiol ogists early in their career, as well as those with experience wishing to update their knowledge. It gives me great pleasure to provide these reflections as an introduction to this valuable text. I commend the author for focusing on the practical issues involved in the management of these many complex defects. Doctor Como is to be congratulated for writing a volume which will surely become a reference text for clinicians in our field for many years to come. Children's Hospital - Boston, Massachusetts, USA PEDRO J. DEL NIDO, M.D. August 2004 Preface Following the program agreed with the Publisher, one year after the publica tion of Volume 1 dealing with the most frequent congenital heart defects, Vol ume 2 appears with the elements for the decision making process in the sur gical treatment of the less common heart malformations. The schema utilized for the construction of the book remain unchanged. The less frequent congenital heart defects are presented with each chapter de voted to a single malformation, with incidence, morphology, associated ano malies, pathophysiology, diagnosis (including clinical pattern, electrocardio gram, chest X-ray, echocardiogram, and cardiac catheterization with angio graphy), indications for surgical treatment, details of surgical treatment, po tential complications and literature references. In comparison with Volume 1 the technical quality of the reproduction of the schematic drawings has been substantially improved, as well as the selec tion of echocardiographic and angiographic images. With regard to the diag nostic tools, photographs with magnetic resonance imaging and computer ized tomography have been selected to add further information on specific defects. More space has been dedicated to the intra-operative photographs, since these were particularly appreciated in Volume 1. Finally,the number of references has been extensively increased for each chapter, following a request frequently received from the readers. As for Volume 1, the acknowledgements to those who contributed to my knowledge in the field of congenital heart defects remain valid for Volume 2. In addition, I would like to thank the individuals providing me with help and support for the preparation of this volume: Bruno Marino and Gaetano Thiene once again allowed me to illustrate the details of cardiac morphology by reproducing wonderful illustrations coming either from their "Atlante di anatomia ecocardiografica delle cardiopatie congenite" or from their personal collections. Michael Rigby kindly made available his library of echocardio graphic images of the most rare congenital heart defects. Adriano Carotti, who was a junior fellowwhen I was in Rome, quickly became a talented sur geon, expert in Pulmonary Atresia with Ventricular Septal Defect. He kindly revised the relevant chapter with constructive criticism. Philippe Monnier and Florian Lang, ENTsurgeons of Lausanne who devel oped the technique of slide tracheoplasty for long-segment tracheal stenosis associated with the simultaneous repair of congenital heart defects, contribut ed to the chapter "Slings and rings" with their precious input. Philippe Clavel, once again with much patience, prepared the graphics following my continu ous requests for improvements. VIII I Preface As for Volume 1, Bruno Marino kindly reviewed the entire text, giving par ticular care to the morphological and cardiologic components. My current Chairman, Ludwig K. von Segesser, with his usual attention to details, stimu lated most of the improvements over Volume 1. He was instrumental in re specting the deadline for completion, by asking with increasing frequency how my job was progressing. A special acknowledgement is due to the Publishers, particularly the CEO Dr. Thomas Thiekotter, Sabine Ibkendanz (head of medical editing) and Sa bine Scheffler (marketing dpt.), A separate mention is reserved for Oliver Frohmeyer (medical editing), who step by step guided me from the beginning through this adventure of the preparation of these books as a single author. Without their continuous support, particularly during the most difficult mo ments, these books simply would not exist. During the preparation of this volume, I had the great chance of personally meeting the individual who entirely changed my vision of the cardiac anato my and physiology: Dr. Francisco Torrent-Guasp. Francisco, now a retired general practitioner in the Spanish village of Denia, not far from Alicante, spent 50 years of his life cultivating his hobby: the investigation of the nor mal cardiac anatomy and physiology. Without any support from a hospital or university, and despite the nihilism and sometimes the sarcasm of most of the other colleagues, he was able through the dissection of more than 1000 hearts to reveal the existence of the "ventricular myocardial band", the unique myocardial structure constituting right and left ventricles. Despite his initial observations having been published 50 years ago when he was a medi cal student, only within the last few years have his theories become available to the scientific public. Several researchers have been recently stimulated by his work to further investigate the ventricular myocardial band. Francisco Torrent-Guasp represents to me the ideal prototype of researcher, who just following his intellectual curiosity and his passion for knowledge, indepen dent from the opinion or the support of anyone else, was able to pursue the truth by "beating to death" the topic attracting his attention. Currently, the potential consequences on the management of congenital heart defects based on the observations by Francisco on the normal cardiac anatomy and physiol ogy are only the object of speculation. Weare only discovering the tip ofthe iceberg, but thanks to Francisco the way has been opened up. Finally, I would like to recognize the unconditional support and indispens able encouragement from my entire family to complete this second book; thank you Josie, Federica, Laura and Jonathan. Lausanne, Switzerland, August 2004 ANTONIO F. CORNO Acknowledgments Since this book is the result of my personal experience, I would like to thank all the individuals who contributed to developing my knowledge in the field of congenital heart defects. Acknowledgment begins with all the sick children and their families en countered during my professional life. Then to all the teachers who contributed to my professional training, col leagues, nurses and technicians met during my career: from everybody I have learned something, from most I learned how, when and what to do in the presence of a child with a congenital heart defect; from others what should not be done which is also extremely important. I Morphology: Thanks to the endless enthusiasm of Gaetano Thiene and his huge specimen collection at the University of Padova, where I received the ru diments of the morphology of congenital heart defects; he spent a tremen dous amount of time and effort teaching the anatomy of the most frequent cardiac malformations. While in Amsterdam for my surgical training, Anton E. Becker, another outstanding cardiac pathologist came into the operating room, scrubbed with the surgeons, explained the details of the intra-cardiac morphology and pro vided precious suggestions for surgical repair. Impossible not to mention are Robert H. Anderson and Richard van Praagh for their educational books and articles as well workshops with prac tical demonstrations. Robert H. Anderson supported the production of this book and provided substantial input for the preparation of a fewchapters. I Pathophysiology: Understanding the pathophysiology is essential in the pro cess of decision making for congenital heart defects. Atthe University of Cali fornia, Los Angeles, Jay M. Iarrnakani and particularly William F. Friedman were always available to explain the myocardial function in normal and sick children. Particularly important were the lessons repeatedly learned from Abraham M. Rudolph, with both editions of his remarkable book and numer ous outstanding papers. I Clinical pattern: Piero Fancini, in Milan, explained cardiac murmurs, elec trocardiograms and chest X-rays, Filippo Casolo taught the basics of angio cardiography. Tom G. Losekoot continued this education in Amsterdam on hemodynamics, followed by Roberta G. Williams at the University of Califor nia, Los Angeles, on pre-operative and post-operative echocardiography and X I Acknowledgments Joseph K. Perloff on the problems of the growing population of adults with congenital heart defects. Asubstantial part of my knowledge on clinical problems has been acquired by daily practice, particularly in the pediatric hospital "Bambino Gesu", Rome, which all the pediatric cardiologists contributed to, particularly Bruno Marino and Salvatore Giannico. A similar positive experience occurred years later in Glasgow, with Neil Wilson. I Surgery: The beginning of my training was at the University of Padova, with the late Vincenzo Gallucci,who taught me how to repair an atrioventric ular septal defect and a tetralogy of Fallot. Further important progress was the exposure to daily practice with Carlo Marcelletti, not only in surgery but also in pre-operative evaluation as well as in post-operative care. Most of the intra-operative photographs of this book have been taken during the period spent with him. Other surgeons substantially contributed to improving my surgical expertise: I Hillel Laks, at the University of California, Los Angeles, who was very de manding and meticulous. I Yves Lecompte, in Paris, thanks to his straightforward style, corrected when needed, before actually teaching how surgery should be done and sometimes how to be inventive. His essential observation was that "every patient is unique". • Ludwig K.von Segesser, my current Chairman, is one of the few remaining surgeons able to operate on a neonate of 2 kg with transposition of the great arteries and single coronary artery, a 86 year-old patient with the rupture of a thoraco-abdominal aneurysm and to support the decision for a Ross operation on a young adult with 4 previous sternotomies. Not to mention his constant intellectual curiosity to develop new techniques and tools in the research laboratory before clinical application. He also pushed me, more than anyone else, to complete this book. Other surgeons participated in extending my surgical knowledge during meetings and workshops, like all the colleagues of the European Congenital Heart Surgeons Foundation. Other contributions came from the visits to the hospitals of Aldo R. Casta neda, Mark R. de Leval,Richard A. Jonas, William I. Norwood, Lucio Paren zan, Claude Planche, Jan M. Quaegebeur, Iaroslav Stark and Pascal Vouhe, I Cardiopulmonary bypass: Yves Durandy, in Paris, demonstrated that cardio pulmonary bypass can and should be performed in a way very similar to the physiological conditions. I Post-operative care: I learned from all the colleagues and nurses in the pe diatric hospital "Bambino Gesu", Roma. Yves Durandy proved that the post-operative period should respect closely the physiological conditions with the fewest medications and interventions. Leonardo Milella, in Glasgow, confirmed that it is possible for the anesthe sist and surgeon to collaborate very well in the post-operative care, with evi dent advantage for the patients.
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