FOETAL CARDIOLOGY cover v2.qxd 07/11/2012 14:59 Page 1 Fetal cardiology has developed into an exciting new subspecialty over the last 30 years. Most health professionals involved in examining the fetal heart are not ‘experts’ F Fetal Cardiology e t in fetal cardiology and may find interpreting images difficult, particularly in cases with a l C a cardiac abnormality. This book is designed as a practical guide, to be kept near the a r S I M P L I F I E D d ultrasound machine, for all those performing fetal heart scans without the expertise of io lo g a fetal cardiologist. The aim of the book is to provide a logical and clear approach to y S scanning the normal heart and how to easily recognise the common forms of fetal IM A P M RACTICAL ANUAL P L cardiac anomalies. The book also provides information on the associated lesions and IF IE D outcomes from fetal life. – A P R The book has a large number of clearly labelled illustrations to allow the reader to A C T recognise the different types of cardiac problem they may encounter and the various IC A L M forms in which they can manifest. A N U A L This book reflects over 20 years of personal experience as a specialist fetal cardiologist, which has included teaching a range of healthcare professionals on how to look at the fetal heart in a structured way. It is aimed at all sonographers, fetal medicine specialists, obstetricians, cardiac technicians/physiologists and radiologists performing obstetric ultrasound scans, as well as paediatric cardiologists with an interest in fetal cardiology. ISBN 978-1-903378-55-7 f t m Gurleen Sharland 9 781903 378557 Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page i Fetal Cardiology S I M P L I F I E D A PRACTICAL MANUAL Gurleen Sharland i Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page ii Fetal Cardiology Simplified – A Practical Manual tfm Publishing Limited, Castle Hill Barns, Harley, Nr Shrewsbury, SY5 6LX, UK Tel: +44 (0)1952 510061; Fax: +44 (0)1952 510192 E-mail: [email protected]; Web site: www.tfmpublishing.com Design & Typesetting: Nikki Bramhill BSc Hons Dip Law First Edition: © 2013 Paperback ISBN: 978-1-903378-55-7 E-book editions: 2013 ePub ISBN: 978-1-908986-93-1 Mobi ISBN: 978-1-908986-94-8 Web pdf ISBN: 978-1-908986-95-5 The entire contents of Fetal Cardiology Simplified – A Practical Manualis 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 any means, electronic, digital, mechanical, photocopying, recording or otherwise, without the prior written permission of the publisher. Neither the author 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. The author 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 apologizes 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, PLA 19, Malta Tel: +356 21897037; Fax: +356 21800069 ii Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page iii Contents page v Foreword vi Acknowledgements vii Dedication viii Abbreviations Screening for congenital heart disease 1 Chapter 1 The normal fetal heart 9 Chapter 2 Abnormalities of cardiac size, position and situs 55 Chapter 3 Abnormalities of the four-chamber view (I) 67 Chapter 4 Abnormalities of veno-atrial and atrioventricular connection Abnormalities of the four-chamber view (II) 109 Chapter 5 Abnormalities of atrioventricular valves and the ventricular septum with normal connections Abnormalities of the four-chamber view (III) 127 Chapter 6 Obstructive lesions at the ventriculo-arterial junction that may be associated with an abnormal four-chamber view Great artery abnormalities (I) 175 Chapter 7 Abnormalities of ventriculo-arterial connection iii Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page iv Fetal Cardiology Simplified – A Practical Manual Great artery abnormalities (II) 203 Chapter 8 Abnormalities of ventriculo-arterial connection Aortic arch abnormalities 239 Chapter 9 Cardiomyopathies 261 Chapter 10 Cardiac tumours 275 Chapter 11 Other cardiac anomalies 283 Chapter 12 Rhythm disturbances in the fetus 303 Chapter 13 Counselling and outcome following prenatal diagnosis of 331 Chapter 14 congenital heart disease What could cardiac findings mean? 339 Chapter 15 361 Further reading 375 Index iv Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page v Foreword This handbook is designed to be an aid to those involved in the detection, diagnosis and management of fetal heart abnormalities. This will include obstetric sonographers, obstetricians, fetal medicine specialists, cardiac technicians/physiologists and paediatric cardiologists training in fetal cardiology, as well as paediatric cardiology consultants with less experience of fetal cardiology. This book will also be useful as a teaching tool for anyone involved in scanning the fetal heart. It is assumed that the reader will be familiar with scanning the fetus and the fetal heart and it is not the aim of this book to teach the practicalities of fetal cardiac scanning, as there are many publications already available to help with this. The purpose of this book is to help interpret cardiac findings and aid in making a correct cardiac diagnosis. The focus of this book is on structural cardiac malformations, though a section on arrhythmias is also included. It is envisaged that many of those using this manual will not have a background in paediatric cardiology. For this reason, the abnormalities have been grouped depending on whether the four-chamber view is likely to be abnormal or not. However, paediatric cardiologists will examine the heart by initially examining the cardiac connections and then looking for further associated abnormalities. This concept has been maintained, both in descriptions of the normal heart and in discussions of abnormal heart anatomy. Whilst some information is included regarding management and outcome, this is not a textbook of paediatric cardiology and further information can be sought in larger textbooks or publications and by consulting paediatric cardiology colleagues, who have wider in-depth knowledge and experience in managing congenital heart disease. It is well recognised that the outcome and associations documented from fetal life may differ from those reported in postnatal series. Therefore the outcomes and associations noted in a large fetal series are referred to here. This information is based on a single-centre experience of fetal cardiac abnormalities seen between 1980 and 2010 at the Evelina Children’s Hospital, which is part of Guy’s and St Thomas’ NHS Foundation Trust, in London, UK. Gurleen Sharland BSc MD FRCP Reader/Consultant in Fetal Cardiology Fetal Cardiology Unit Evelina Children’s Hospital Guy’s & St Thomas’ NHS Foundation Trust London, UK v Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page vi Acknowledgements I would like to thank all my family and dear friends for endless encouragement and support. I would also like to thank and acknowledge my colleagues and all members of the fetal cardiology team at Evelina Children’s Hospital, London. Their continuing dedication and professionalism has enabled the development of a first class service providing high standards of care for patients and their families. vi Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page vii Dedication To Mike, Peter and Emma with all my love and much more and with love and thanks to my very dear parents, Mani and Puran vii Prelims foetal cardiology_Prelims foetal cardiology.qxd 27-09-2013 15:57 Page viii Abbreviations A atrial Abs PV absent pulmonary valve syndrome ALSCA aberrant left subclavian artery Ao aorta AoA aortic arch AoAt VSD aortic atresia with a ventricular septal defect AoV aortic valve ARSCA aberrant right subclavian artery AS aortic stenosis Asc Ao ascending aorta AVSD atrioventricular septal defect AV valve atrioventricular valve AVVR atrioventricular valve regurgitation CAT common arterial trunk CCAML congenital cystic adenomatoid malformation of the lung CCTGA congenitally corrected transposition of the great arteries CHB congenital heart block CHD congenital heart disease Coarct coarctation of the aorta Coll a collateral vessel CS coronary sinus DAo descending aorta Diabetic maternal diabetes DIV double-inlet ventricle DORV double-outlet right ventricle Ebstein’s Ebstein’s anomaly ECG electrocardiogram Fabn fetal abnormality Farr fetal arrhythmia FH family history Fhyd fetal hydrops FO foramen ovale HLH hypoplastic left heart syndrome INFD death in infancy Int AA interrupted aortic arch IUD intrauterine death IVC inferior vena cava LA left atrium LAI left atrial isomerism LAVV left atrioventricular valve viii Prelims foetal cardiology_Prelims foetal cardiology.qxd 10-05-2013 09:31 Page ix Abbreviations LCA left coronary artery LPA left pulmonary artery LSVC left superior vena cava LTFU lost to follow-up LV left ventricle LVDD left ventricular diastolic dimension LVSD left ventricular systolic dimension MAT mitral atresia MPA main pulmonary artery MR mitral regurgitation MV mitral valve NND death in neonatal period NT nuchal translucency PA pulmonary artery PAPVD partial anomalous pulmonary venous connection PAT IVS pulmonary atresia with an intact ventricular septum PAT VSD pulmonary atresia with a ventricular septal defect PE pericardial effusion PV pulmonary valve PS pulmonary stenosis RA right atrium RAI right atrial isomerism RAVV right atrioventricular valve RCA right coronary artery RPA right pulmonary artery RSVC right superior vena cava RV right ventricle SVC superior vena cava SVT supraventricular tachycardia T trachea TAPVD total anomalous pulmonary venous connection or drainage TAT tricuspid atresia Tetralogy tetralogy of Fallot TGA transposition of the great arteries ToF tetralogy of Fallot TOP termination of pregnancy TR tricuspid regurgitation TV tricuspid valve TVD tricuspid valve dysplasia UV umbilical vein V ventricle or ventricular VSD ventricular septal defect ix