H YPOPLASTIC L EFT H EART S YNDROME

H^YPOPLASTIC L^EFT H^EART S^YNDROME

Springer

London Berlin Heidelberg New York Hong Kong Milan Paris Tokyo

Robert H. Anderson Marco Pozzi

Suzie Hutchinson

H^YPOPLASTIC L^EFT H^EART S^YNDROME

With 95 Figures

Robert H. Anderson, BSc, MD, FRCPath

Cardiac Unit, Institute of Child Health, University College, London, UK Marco Pozzi, MD, FECTS

Cardiac Unit, Royal Liverpool Children’s Hospital, Liverpool, UK Suzie Hutchinson, RGN, RSCN

Little Hearts Matter, Birmingham, UK

British Library Cataloguing-in-Publication Data Anderson, Robert H. (Robert Henry), 1942–

Hypoplastic left heart syndrome

1. Congenital heart disease in children 2. Children–

Diseases–Treatment

I. Title II. Pozzi, Marco III. Hutchinson, Suzie 618.9′212043

ISBN 1852337656

Library of Congress Cataloging-in-Publication Data Anderson, Robert Henry.

Hypoplastic left heart syndrome / Robert H. Anderson, Marco Pozzi, Suzie Hutchinson.

p. cm.

Includes bibliographical references and index.

ISBN 1-85233-765-6 (alk. paper)

1. Congenital heart disease in children. 2. Children–Diseases–Treatment. I. Pozzi, Marco. II. Hutchinson, Suzie. III. Title.

RJ426.C64A63 2004 618.92′12043–dc22

2004040150

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The introduction of the three-stage Norwood proce- dure, coupled with neonatal cardiac surgical trans- plantation emerging as an option for the management of children with hypoplasia of the left heart, has repre- sented one of the great challenges, and I think one of the great successes, in the care of children with con- genital cardiac disease over the last 20 years. The sur- gical and physiological management has not been without its problems. Whilst progress has at times been difficult, it has been steady. Many centres, some repre- sented by the authors in this book, have achieved outstanding results, and have provided inspiration for others to follow. It has, however, taken a long time for the surgical outcomes to be entirely satisfactory, and there is undoubtedly still a long way to go. Whilst early success may now be being achieved, the long-term outlook is still not normal for these children, whose systemic circulation is dependent upon the morphologi- cally right ventricle after the Fontan procedure.

Complications of the Fontan circulation are inevitable, and many may need cardiac transplantation in adult life.

Naturally, a newborn and seriously ill baby has a major impact on family life. It is therefore entirely appropriate that the views of the families should have an important place in this textbook. In addition, our own unit, and I know other units, have found the posi- tive support of families with children having hypoplasia of the left heart to be overwhelmingly important to all the health professionals involved in the care of these children.

The authors in this textbook are to be congratulated for bringing together all the important aspects of the care of children with hypoplasia of the left heart, and providing a goldmine of information for their colleagues throughout the world. This is a fine textbook, repre- senting the work of the last 20 years for a group of patients for whom previously there was no hope.

William J. Brawn, FRCS, FRACS Consultant Paediatric Cardiac Surgeon Department of Paediatric Cardiac Surgery Birmingham Children’s Hospital

Birmingham, United Kingdom

v

FOREWORD

Foreword . . . v 1. The Morphology of Hypoplasia of the Left Heart . . . 1

AUDREYSMITH, MARCOPOZZI,ANDROBERTH. ANDERSON

2. The Phenotype During Human Fetal Development . . . 19 ANDREWC. COOK

3. Antenatal Diagnosis of Hypoplastic Left Heart Syndrome . . . 39 GURLEENSHARLAND

4. The Genetics of Hypoplasia of the Left Heart . . . 49 HELENCOX ANDDAVID I. WILSON

5. Diagnostic Approach . . . 55 JAMESL. WILKINSON

6. Pre- and Postoperative Management of Infants with Hypoplasia of the Left Heart . . . 63 THOMASJ. KULIK, DENNISC. CROWLEY,ANDJOHN R. CHARPIE

7. Surgical Management of Hypoplasia of the Left Heart . . . 73 EDWARD L. BOVE

8. Evolving Techniques in the Operative Management of Hypoplasia of the Left Heart . . . 89 AKHLAQUEN. BHAT, AITIZAZU. SYED,ANDMARCOPOZZI

9. Anaesthesia for Hypoplasia of the Left Heart . . . 99 MONICA A. STOKES

10. Transplantation in the Management of Infants with Hypoplasia of the Left Heart . . . 105 ANEESJ. RAZZOUK, RICHARDE. CHINNOCK, JOYCEK. JOHNSTON, JAMES A. FITTS,

ANDLEONARDL. BAILEY

11. The Nursing Management of Neonates and Infants with Hypoplasia of the Left Heart . . . 119 JANETL. TAYLOR, FIONAHORROX, SHARONGOOMANY, SUE SMITH,ANDAKHLAQUEN. BHAT

CONTENTS

vii

12. The Psychosocial Problems Faced by the Families of Children with Hypoplasia of

the Left Heart . . . 129 SUZIEHUTCHINSON

13. Neuropsychological Functioning and Psychosocial Development in Children with Hypoplasia of

the Left Heart . . . 135 JACQUELINEC. BLYTH

14. Chasing the Light: A Parent’s Perspective on Hypoplastic Left Heart Syndrome . . . 145 LOUISEE. HALL

Index . . . 151

viii Contents

ROBERTH. ANDERSON, BSC, MD, FRCPATH

Cardiac Unit

Institute of Child Health University College London London

UK

LEONARDL. BAILEY, MD Department of Surgery Loma Linda University Loma Linda

San Bernardino, California USA

AKHLAQUEN. BHAT, FRCS

Paediatric Intensive Care Unit and Cardiac Unit Royal Liverpool Children’s Hospital

Merseyside UK

JACQUELINE C. BLYTH, BSC, MSC, CLINPSYD Clinical Psychology Department

Birmingham Children’s Hospital Birmingham

UK

EDWARDL. BOVE, MD Section of Cardiac Surgery

Division of Paediatric Cardiovascular Surgery Department of Surgery

C.S. Mott Children’s Hospital

University of Michigan School of Medicine Ann Arbor, Michigan

USA

JOHNR. CHARPIE, MD, PHD Department of Pediatrics University of Michigan C.S. Mott Children’s Hospital Ann Arbor, Michigan

USA

RICHARD E. CHINNOCK, MD Department of Pediatrics

Loma Linda University School of Medicine Loma Linda

San Bernardino, California USA

ANDREWC. COOK, PHD, BSC

Cardiac Unit

Institute of Child Health University College London London

UK

HELENCOX, BSC, MBCHB, MSC

Human Genetics Division University of Southampton Southampton

UK

DENNISC. CROWLEY, MD Department of Pediatrics University of Michigan C.S. Mott Children’s Hospital Ann Arbor, Michigan

USA

CONTRIBUTORS

ix

JAMESA. FITTS, MD Loma Linda University Loma Linda

San Bernardino, California USA

SHARONN. GOOMANY, RGN, RSCN Paediatric Intensive Care Unit Royal Liverpool Children’s Hospital Merseyside

UK

LOUISEE. HALL, BA Trustee

Little Hearts Matter Birmingham UK

FIONAHORROX, BSC, RSCN, RGN Paediatric Intensive Care Unit Royal Liverpool Children’s Hospital Merseyside

UK

SUZIEHUTCHINSON, RGN, RSCN Director

Little Hearts Matter Birmingham UK

JOYCE K. JOHNSTON, BSN, RN Cardiac Transplant Department Loma Linda University Medical Center Loma Linda

San Bernardino, California USA

THOMASJ. KULIK, MD Department of Pediatrics University of Michigan C.S. Mott Children’s Hospital Ann Arbor, Michigan

USA

MARCO POZZI, MD, FECTS Cardiac Unit

Royal Liverpool Children’s Hospital Liverpool

UK

ANEESJ. RAZZOUK, MD

Division of Cardiothoracic Surgery Department of Surgery

Loma Linda University Loma Linda

San Bernardino, California USA

GURLEEN SHARLAND, MD, FRCP Fetal Cardiology Unit

Guy’s Hospital London UK

AUDREYSMITH, PHD, FIBMS Honorary Senior Research Fellow The Cardiac Unit

Institute of Child Health University College London

UK

SUESMITH, RGN, RSCN Paediatric Intensive Care Unit Royal Liverpool Children’s Hospital Merseyside

UK

MONICA A. STOKES, BMBS, FRCA Department of Anaesthesia Birmingham Children’s Hospital Birmingham

UK

AITIZAZU. SYED, FRCS

Paediatric Intensive Care Unit and Cardiac Unit Royal Liverpool Children’s Hospital

Merseyside UK

JANETL. TAYLOR, RGN, RSCN Royal Liverpool Children’s Hospital Liverpool

UK

JAMESL. WILKINSON, MBCHB, FRCP, FRACP, FACC, FRCPCH

Department of Cardiology Royal Children’s Hospital Melbourne, Victoria Australia

DAVID I. WILSON, BA, MBBS, PHD, FRCP Human Genetics Division

University of Southampton Southampton

UK

x Contributors