Hypoplastic Left Heart Syndrome

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232. A . Bayes de Luna, F . Furlanello, B . J . Maron and D . P . Zipes (eds.): Arrhythmias and Sudden Death in Athletes. 2000 I S B N : 0-7923-6337-X

233. J -C. Tardif and M . G . Bourassa (eds): Antioxidants and Cardiovascular Disease. 2000. I S B N : 0-7923-7829-6

234. J. Candell-Riera, J. Castell-Conesa, S. Aguade Bruiz (eds): Myocardium at Risk and Viable Myocardium Evaluation by SPET. 2000.ISBN: 0-7923-6724-3

235. M . H . Ellestad and E . Amsterdam (eds): Exercise Testing: New Concepts for the New Century. 2001. I S B N : 0-7923-7378-2

236. Douglas L . Mann (ed.): The Role of Inflammatory Mediators in the Failing Heart. 2001 I S B N : 0-7923-7381-2

237. Donald M . Bers (ed.): Excitation-Contraction Coupling and Cardiac Contractile Force, Second Edition. 2001 I S B N : 0-7923-7157-7

238. Brian D . Hoit, Richard A . Walsh (eds.): Cardiovascular Physiology in the Genetically Engineered Mouse, Second Edition. 2001 ISBN 0-7923-7536-X

239. Pieter A . Doevendans, A . A . M . Wilde (eds.): Cardiovascular Genetics for Clinicians 2001 ISBN 1-4020-0097-9

240. Stephen M . Factor, Maria A.Lamberti-Abadi, Jacobo Abadi (eds.): Handbook of Pathology and Pathophysiology of Cardiovascular Disease. 2001

ISBN 0-7923-7542-4 241. Liong Bing L iem, Eugene Downar (eds): Progress in Catheter Ablation. 2001

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of Coronary Heart Disease: Diet, Lifestyle and Risk Factors in the Seven Countries Study. 2002 ISBN 1-4020-7123-X

244. Antonio Pacifico (ed.), Philip D . Henry, Gust H . Bardy, Martin Borggrefe, Francis E . Marchlinski, Andrea Natale, Bruce L . Wi lkof f (assoc. eds): Implantable Defibrillator Therapy: A Clinical Guide. 2002

ISBN 1-4020-7143-4 245. Hein J.J. Wellens, Anton P . M . Gorgels, Pieter A . Doevendans (eds.):

The ECG in Acute Myocardial Infarction and Unstable Angina: Diagnosis and Risk Stratification. 2002" ISBN 1-4020-7214-7

246. Jack Rychik, G i l Wernovsky (eds.): Hypoplastic Left Heart Syndrome. 2003 ISBN 1-4020-7319-4

Previous volumes are still available

Hypoplastic Left Heart Syndrome

edited by

Jack Rychik, M.D., and GilWernovsky,M.D. Division of Cardiology, Children's Hospital of Philadelphia

SPRINGER SCIENCE+BUSINESS MEDIA, LLC

Library of Congress Cataloging-in-Publication Data

A C.I.P. Catalogue record for this book is available from the Library of Congress.

ISBN 978-1-4613-4988-4 ISBN 978-1-4615-0253-1 (eBook) DOI 10.1007/978-1-4615-0253-1

Hypoplastic Left Heart Syndrome Edited by: Jack Rychik, MD and Gil Wernovsky, MD

Copyright © 2003 by Springer Science+Business Media New York Originally published by Kluwer Academic Publishers in 2003 Softcover reprint of the hardcover 1st edition 2003 All rights reserved. No part of this work may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording, or otherwise, without the written permission from the Publisher, with the exception of any material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work.

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Dedication

This book is dedicated to the patients with hypoplastic left heart syndrome, their courageous parents, and their supportive families and friends.

TABLE OF CONTENTS

List of Contributors ............................................................................ ix Foreword ............................................................................................. xi

Chapters

1 Epidemiology and Genetics ................................... .., ................ 1 Elizabeth Goldmuntz, MD.

2 Fetal Development and Prenatal Evaluation .......................... 9 Meryl S. Cohen, M.D.

3 Delivery and Obstetrical Issues ........................................... 29 Samuel Parry, MD. and Sara J. Marder, MD.

4 Echocardiographic Imaging ................................................... 39 Jack Rychik, MD.

5 Pre-Operative Management ................................................. 69 Sarah Tabbutt MD., Ph.D. and Gil Wernovsky MD.

6 Stage I Reconstruction .......................................................... 89 Thomas L. Spray, MD.

7 Stage I Postoperative Management ................................... 105 Gil Wernovsky, MD, DoffB. McElhinney, MD,

and Sarah Tabbutt MD, PhD

8 The Bidirectional Cavopulmonary Shunt ............................ 129 Tom R. Karl, MS., MD.

9 The Fontan Operation and Modifications .......................... 149 J. William Gaynor, MD.

10 Anesthetic Management for HLHS .................................... 167 James M Steven, MD., and Susan C. Nicolson, MD.

11 Cardiac Catheterization ....................................................... 193 Jacqueline Kreutzer, MD., and Jonathan J. Rome, MD.

12 Nursing Care ........................................................................ 229 Rhonda Foltz, R.N. and Kathryn M Dodds R.N., MS.N., c.P.N.P.

13 Infant Heart 1ransplantation .............................................. 241 Timothy M Hoffman, MD., and Thomas L. Spray, MD.

viii Hypoplastic Left Heart Syndrome

14 N eurologicaJ Issues ............................................................. 251 Robert Ryan Clancy, MD.

15 Feeding Issues and Somatic Growth ................................... 273 Mitchell 1. Cohen MD

16 Cardiac Structure and Function .......................................... 289 William Mahle, MD

17 Arrhythmias After Surgery ................................................. 307 Ronn E. Tanel, MD. and Larry A. Rhodes, MD.

18 Exercise Physiology and Capacity ...................................... 329 Steven Paridon, MD.

19 Complications After the Fontan .......................................... 347 Jack Rychik, MD.

20 Decision Analysis Strategies .................................. .., .......... 361 Bernard J. ClarkIIL M.D.

21 What the Pediatrician Needs to Know ................................ 379 Marie M Gleason, MD.

22 The Personal Perspective ................. 00 ................................. 393 Stephen Klein, Anna Jaworski, and Kristen Aversa

23 Financial Implications ................. u ....................................... 407 William J. Greeley, MD., MB.A.

Epilogue .................................................................................................... 417 Index ..................................................... 8 ••••••••••••••••••••• 0 ••••••••••••••••••••• 419

Editors Jack Rychik, M.D., Associate Professor, Department of Pediatrics, University of

Pennsylvania School of Medicine; Director, Non-Invasive Cardiovascular Laboratories and the Fetal Heart Program, Division of Cardiology, The Children's Hospital of Philadelphia. rychik@email.chop.edu

Gil Wernovsky, M.D., Associate Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Staff Cardiologist, Cardiac Intensive Care Unit; Director of Program Developement, The Cardiac Center, The Children's Hospital of Philadelphia. wernovsky@email.chop.edu

Contributors

Kristen Aversa, M.D.

Robert Ryan Clancy, M.D., Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Neurology, The Children's Hospital of Philadelphia

Bernard J. Clark III, M.D., Associate Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Meryl S. Cohen, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Mitchell I. Cohen M.D., Associate Cardiologist, Arizona Pediatric Cardiology Consultants

Kathryn M. Dodds R.N., M.S.N., C.P.N.P., Pediatric Nurse Practitioner, Cardiac Intensive Care Unit, Children's Hospital of Philadelphia

Rhonda Foltz, R.N., Pediatric Nurse Practitioner, Cardiac Intensive Care Unit, Children's Hospital of Philadelphia

J. William Gaynor, M.D., Associate Professor of Surgery, University of Pennsylvania School of Medicine; Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia

Marie M. Gleason, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Elizabeth Goldmuntz, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

William J. Greeley, M.D., M.B.A., John J. Downes Professor, Departments of Anesthesiology and Pediatrics, University of Pennsylvania School of Medicine; Chairman, Department of Anesthesia, Children's Hospital of Philadelphia

x Hypoplastic Left Heart Syndrome

Timothy M. Hoffman, M.D., Assistant Professor of Pediatrics, The Ohio State University College of Medicine; Medical Director, Heart Transplant Program, Columbus Children's Hospital

Anna Jaworski

Tom R. Karl, M.S., M.D., Professor of Surgery, University of California-San Francisco School of Medicine; Chief of Pediatric Cardiothoracic Surgery, Children's Hospital of the University of California San Francisco

Stephen Klein

Jacqueline Kreutzer, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

William Mahle, M.D., Sibley Heart Center, Children's Healthcare of Atlanta

Sara J. Marder, M.D., Assistant Professor, Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Washington University Medical Center

Doff B. McElhinney, MD, Fellow in Cardiology, Boston Children's Hospital

Susan C. Nicolson, M.D., Professor, Departments of Anesthesiology and Pediatrics, University of Pennsylvania School of Medicine; Chair, Division of Cardiac Anesthesia, Children's Hospital of Philadelphia

Steven Paridon, M.D., Associate Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Samuel Parry, M.D., Assistant Professor, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania

Larry A. Rhodes, M.D., Associate Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Jonathan J. Rome, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

Thomas L. Spray, M.D., Professor of Surgery, University of Pennsylvania School of Medicine; Chief, Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia

James M. Steven, M.D., Professor, Departments of Anesthesiology and Pediatrics, University of Pennsylvania School of Medicine; Chair, Division of Cardiac Anesthesia, Children's Hospital of Philadelphia

Sarah Tabbutt M.D., Ph.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Divisions of Cardiology and Critical Care Medicine, The Children's Hospital of Philadelphia

Ronn E. Tanel, M.D., Assistant Professor, Department of Pediatrics, University of Pennsylvania School of Medicine; Division of Cardiology, The Children's Hospital of Philadelphia

FOREWORD

Despite approximately 50 years of progress in the management of com­plex congenital heart disease, hypoplastic left heart syndrome (HLHS) has been one of the last cardiac malformations to yield to effective surgical treat­ment. The surgical therapies for hypoplastic left heart syndrome have evolved by two quite disparate routes. One approach has been the staged reconstruc­tion operations utilizing an initial operation pioneered by many but developed by Dr. William Norwood and subsequently modified by many other investigators. The first stage reconstruction operation has been refined and standardized such that the majority of infants now proceed to second and third stage reconstruc­tive operations culminating in a Pontan Kreutzer single ventricle physiology. An alternative surgical approach has been the development of neonatal and infant heart transplantation for HLHS. This approach also has had a signifi­cant impact on the treatment of HLHS and other cardiac malformations and has been associated with good intermediate term results. Both of the thera­peutic approaches devised for treatment of hypoplastic left heart syndrome have been effective and the outcomes are continuing to improve with each.

The present monograph summarizes our current level of knowledge of treat­ment of HLHS and its variants based largely on the extensive experience at The Children's Hospital of Philadelphia in over 1,000 consecutive infants with this form of complex congenital heart disease. The knowledge gained from experience with this large number of patients and the contributions from many other centers have created a large body of new information on the anatomy, diagnosis, management and longterm follow-up of children with HLHS. Nev­ertheless, the rapid recent improvements in outcome in children with HLHS has meant that the majority of survivors are only now reaching their early childhood years, so that additional developmental and neurologic testing can now begin. In addition, the oldest patients with staged reconstruction are now entering their teenage years and early adulthood and the effects on longterm function can only now begin to be evaluated. Certainly, as these children con­tinue to grow we will learn more about potential late complications and their management.

xii Hypoplastic Left Heart Syndrome

Although resources that have been committed to treatment of HLHS have been extensive over the past two decades, the impact of these resources on total health care expenditures has been relatively small compared to the great effect on the development of successful therapy for HLHS and on the surgical reconstructive techniques, critical care, and cardiologic management of many other forms of congenital heart disease. Continued challenges in management will occur as more children survive into young adulthood and onward. As in other areas of progress, new unforeseen problems will certainly arise which will continue to challenge medical and surgical knowledge and skills.

The current success with surgical repair for children with HLHS could not have occurred without the dedication and persistence of countless medical, surgical, and nursing teams, but most of all, without the hope, compassion and support of the parents.

Thomas L. Spray, M.D. Chief, Division of Cardiothoracic Surgery,

Executive Director, The Cardiac Center The Children 50 Hospital of Philadelphia

Alice Langdon Warner Professor of Surgery University of Pennsylvania School of Medicine

Acknowledgements

Although there are countless physicians, nurses, and allied professionals who have contributed to the advances in the treatment of hypoplastic left heart syndrome, we wish to acknowledge the insight, perseverance and inestimable contributions of the following pediatric cardiovascular surgeons - Leonard Bailey, Marc de Leval, Francis Fontan, Guillermo Kreutzer, William Glenn, and William Norwood - and pediatric cardiologists Peter Lang and John Murphy.

We would like to express our gratitude to all the authors who contributed to this book. Each produced a clear synthesis of their research and clinical experience of HLHS, and on editing the chapters we realized how fortunate we were to find ourselves among such a dedicated group of contributors and colleagues.

This text was completed thanks to the hard work and enthusiasm of David Reese, who spent many hours editing and formatting 24 distinct chapters into this final version. This project would have been impossible without him.

- J.R. and G. W.

I would like to thank my mentors and colleagues, the cardiologists, surgeons and staff at The Children's Hospital of Philadelphia. These exceptional people have demonstrated to me that through hard work, logic, and compassion, one can make a major difference in the lives of children with heart disease. I would espe­cially like to thank Bill Norwood, Marshall Jacobs, John Murphy and Alvin Chin who contributed heavily to my thinking about complex heart disease. In addition, I would like to thank my parents Hana and Harry Rychik, who taught me the importance of self-sacrifice and education in life. Lastly, I would like to thank my wife Susan, and my children Jordana, Leora, and Tali for providing me with the daily support and encouragement I need to do the things I do. Without them, nothing would get done.

- J.R.

I would first like to thank three pediatric cardiologists who have been role models as compassionate physicians, investigators and teachers: Peter Lang, Jane Newburger, and David Wessel. I would also like to thank Dr. Aldo R. Castaneda, who, in addition to his brilliance as a cardiovascular surgeon, taught me the value of a collaberative approach to patient care among medicine, surgery, and nursing. I would especially like to thank my wife Lauren, and my children Simon and Jenna for their continued support and encouragement through the rigors of academic pediatric cardiology. Finally, I would like to dedicate this book to the memory of my parents, Louis and Janet, who, with great foresight, would not let me become a musician .. ,

- G.w.