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Key Questions in
CONGENITALCARDIACSURGERY
Narain Moorjani
Nicola Viola
Christopher A. Caldarone
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
ii
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
======================= é~ÖÉ
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.
hЙу=nмЙлнбзел=бе=`зеЦЙебн~д=`~кЗб~Е= pмкЦЙку
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
viii
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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hÉó
. 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.
hЙу= nмЙлнбзел= бе= `зеЦЙебн~д= `~кЗб~Е=pмкЦЙку
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
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