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Key Questions in
CONGENITALCARDIACSURGERY
Narain Moorjani
Nicola Viola
Foreword by William J. Brawn
Key Questions in
CONGENITALCARDIACSURGERY
Narain Moorjani
Nicola Viola
Christopher A. Caldarone
Foreword by William J. Brawn
Key Questions in CONGENITAL CARDIAC SURGERY
tfm Publishing Limited, Castle Hill Barns, Harley, Shrewsbury, SY5 6LX, UK Tel: +44 (0)1952 510061; Fax: +44 (0)1952 510192 E-mail: info@tfmpublishing.com; Web site: www.tfmpublishing.com
Design & Typesetting: Nikki Bramhill BSc (Hons) Dip Law First Edition: © 2022
Paperback ISBN: 978-1-903378-94-6
E-book editions: © 2022 ePub ISBN: 978-1-910079-46-1 Mobi ISBN: 978-1-910079-47-8 Web pdf ISBN: 978-1-910079-48-5
The entire contents of ‘Key Questions in CONGENITAL CARDIAC SURGERY’ is copyright tfm Publishing Ltd. Apart from any fair dealing for the purposes of research or private study, or criticism or review, as permitted under the Copyright, Designs and Patents Act 1988, this publication may not be reproduced, stored in a retrieval system or transmitted in any form or by
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any means, electronic, digital, mechanical, photocopying, recording or otherwise, without the prior written permission of the publisher.
Neither the authors nor the publisher can accept responsibility for any injury or damage to persons or property occasioned through the implementation of any ideas or use of any product described herein. Neither can they accept any responsibility for errors, omissions or misrepresentations, howsoever caused.
Whilst every care is taken by the authors and the publisher to ensure that all information and data in this book are as accurate as possible at the time of going to press, it is recommended that readers seek independent verification of advice on drug or other product usage, surgical techniques and clinical processes prior to their use.
The authors and publisher gratefully acknowledge the permission granted to reproduce the copyright material where applicable in this book. Every effort has been made to trace copyright holders and to obtain their permission for the use of copyright material. The publisher apologises for any errors or omissions and would be grateful if notified of any corrections that should be incorporated in future reprints or editions of this book.
Printed by Gutenberg Press Ltd., Gudja Road, Tarxien, GXQ 2902, Malta Tel: +356 2398 2201; Fax: +356 2398 2290 E-mail: info@gutenberg.com.mt; Web site: www.gutenberg.com.mt
Contents
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Preface vi
Foreword viii
Acknowledgements x
Contributors xii
Abbreviations xxii
Chapter 1 Congenital cardiac anatomy 1
Chapter 2 Congenital cardiac physiology 61
Chapter 3 Congenital cardiac pharmacology 101
Chapter 4 Congenital echocardiography 127
Chapter 5 Congenital cardiac imaging 171
Chapter 6 Congenital angiography and catheter 205
interventions
Chapter 7 Anaesthesia and congenital heart disease 241
Chapter 8 Paediatric cardiac intensive care 277
Chapter 9 Adult congenital cardiac intensive care 337
Chapter 10 Cardiopulmonary bypass 351
Chapter 11 Extracorporeal membrane oxygenation 385
iii
Chapter 12 Informed consent in congenital cardiac surgery 427
Key Questions in CONGENITAL CARDIAC SURGERY
Chapter 13 Atrial septal defects 451
Chapter 14 Ventricular septal defects 477
Chapter 15 Atrioventricular septal defects 519
Chapter 16 Anomalous pulmonary venous connections 537
Chapter 17 Ebstein’s anomaly 565
Chapter 18 Tricuspid atresia 595
Chapter 19 Hypoplastic right heart 613
Chapter 20 Right ventricular outflow tract 639
Chapter 21 Tetralogy of Fallot 675
iv
Chapter 22 Pulmonary atresia with ventricular septal defect 703
Chapter 23 Transposition of the great arteries 729
Chapter 24 Congenitally corrected transposition of 759
the great arteries
Chapter 25 Double-outlet right ventricle 781
Chapter 26 Hypoplastic left heart syndrome 811
Chapter 27 Congenital abnormalities of the mitral valve 839
Chapter 28 Left ventricular outflow tract lesions 863
Chapter 29 Truncus arteriosus 907
Chapter 30 Anomalies of coronary arteries and 935
coronary fistulae
Chapter 31 Aortopulmonary window 961
Contents
Chapter 32 Vascular rings and slings 979
Chapter 33 Patent ductus arteriosus 995
Chapter 34 Aortic coarctation 1019
Chapter 35 Interrupted aortic arch 1047
Chapter 36 Univentricular heart 1057
Chapter 37 Surgical palliation in congenital heart disease 1073
Chapter 38 Heart and lung transplantation 1087
Chapter 39 Surgery for cardiac arrhythmias 1111
Chapter 40 Cardiac tumours 1139
Chapter 41 Infective endocarditis 1157
v
Index 1177
Preface
Congenital cardiac surgery is a continually expanding field with the development of novel techniques and operations, as well as the refinement of well-established surgical procedures. These developments fuel the demand for knowledge regarding congenital cardiac surgical disease processes and the optimal therapeutic strategies currently available. Although several large-volume texts exist, there are none which aim to deliver this knowledge base in one concise book, and that address the important questions pertaining to congenital cardiac surgery.
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the main topics involved in the contemporary practice of a congenital cardiac surgeon, using numerous illustrations to enhance the understanding of the reader. The book incorporates the current
vi
international guidelines for practice, with up-to-date information based on current scientific literature and experience from established congenital cardiac surgical units. Each chapter contains important references for further reading and greater in-depth study. The chapters have been written by practising international specialists in congenital cardiac surgery, congenital cardiology, congenital cardiac anaesthesia and intensive care, to reflect the truly interdisciplinary nature of congenital cardiac practice. The images have been drawn from an operative perspective where appropriate.
This book is relevant to all cardiothoracic surgical trainees and residents, at any stage of their training programme, as it provides them with the necessary knowledge base to carry out their daily duties. Paediatric cardiologists, adult congenital cardiologists, congenital intensive care unit specialists, radiologists, nursing staff, physiotherapists and other allied professionals working with congenital cardiac surgical patients, either pre­operatively or postoperatively, will also find this book key to facilitating their understanding of the principles surrounding congenital cardiac surgical disease management. Importantly, the book is also an ideal revision aid for trainees and residents undertaking their cardiothoracic surgery board examinations around the world. Its concise yet complete coverage of the important topics make it the perfect guide to answer the key questions in congenital cardiac surgery that are often asked within the confines of an
systematically covers all
Preface
examination. In summary, this book is a succinct yet encompassing congenital cardiac surgical text, which allows the reader to obtain information in an easily readable format.
Furthermore, this book completes the key question series of cardiothoracic surgical textbooks, following on from the successful publication of
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and
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Narain Moorjani MB ChB MRCS MD FRCS (CTh) MA
Royal Papworth Hospital
University of Cambridge, UK
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University Hospital Southampton
University of Southampton, UK
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Texas Children’s Hospital and
Baylor College of Medicine
Houston, Texas, USA
vii
Foreword
I have been a congenital heart surgeon for over 35 years during which time there have been tremendous advances in the management of patients of all ages with congenital heart disease. It is a pleasure, therefore, to contribute a foreword for it covers these advances in which I have had the privilege to have been involved.
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as
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This book is the third volume in the ‘Key Questions’ series after
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Key Question format, so successful in the first two volumes, is very suited to enable an understanding of the management of congenital heart disease. This is because the question and answer format allows the reader to more easily navigate through the complexities of congenital cardiac abnormalities.
The editors have gathered world-class contributors to write each chapter and the chapters are clearly written and comprehensively cover the abnormal cardiac morphologies and their diagnosis and treatment.
Each chapter is clearly illustrated with easily understandable diagrams and photographs — all of superb quality. Importantly, the cardiac anatomy and congenital malformations are clearly described and illustrated with outstanding, coloured illustrations and photographs. Some chapters cover the important topics of cardiopulmonary bypass, physiology and diagnostic techniques. A separate chapter is devoted to the issue of consent, particularly important now for patients with complex lesions where difficult palliative surgery may be the only surgical option.
and
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. The
This is an exceptional textbook and its main strength is the question and answer format, clear description of the congenital heart defect and the excellent illustrations and photographs.
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understanding of the complex subject of congenital heart disease and
contains all the basic information to enable a clear
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Foreword
provides a sound foundation in the subject. At the end of each chapter, selected references have been included that will guide the reader to further exploratory reading.
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surgical and medical trainees and all other professionals involved in the care of patients with all forms of congenital heart disease.
is essential reading for
William J. Brawn CBE FRCS
Honorary Consultant in Congenital Heart Surgery
Birmingham Women’s and Children’s NHS Trust
Birmingham, UK
ix