Heart Diseasein PregnancySecond edition

EDITED BY

Celia Oakley, MD, FRCP, FESC, FACCProfessor (Emeritus) of Clinical Cardiology, Imperial College School ofMedicine at Hammersmith Hospital, London, UK

Carole A Warnes, MD, FRCP, FACCProfessor of Medicine, Mayo Clinic Consultant, Division of CardiovascularDiseases, Internal Medicine and Pediatric Cardiology, Mayo Clinic,Rochester, Minnesota, USA

Heart Disease in Pregnancy

Heart Diseasein PregnancySecond edition

EDITED BY

Celia Oakley, MD, FRCP, FESC, FACCProfessor (Emeritus) of Clinical Cardiology, Imperial College School ofMedicine at Hammersmith Hospital, London, UK

Carole A Warnes, MD, FRCP, FACCProfessor of Medicine, Mayo Clinic Consultant, Division of CardiovascularDiseases, Internal Medicine and Pediatric Cardiology, Mayo Clinic,Rochester, Minnesota, USA

© 2007 by Blackwell Publishing© 1997 BMJ Publishing GroupBMJ Books is an imprint of the BMJ Publishing Group Limited, used under licence

Blackwell Publishing, Inc., 350 Main Street, Malden, Massachusetts 02148-5020, USABlackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UKBlackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia

The right of the Author to be identified as the Author of this Work has been asserted inaccordance with the Copyright, Designs and Patents Act 1988.

All rights reserved. No part of this publication may be reproduced, stored in a retrievalsystem, or transmitted, in any form or by any means, electronic, mechanical, photocopying,recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act1988, without the prior permission of the publisher.

First published 1997Second edition 2007

1 2007

Library of Congress Cataloging-in-Publication Data

Heart disease in pregnancy / edited by Celia Oakley, Carole A. Warnes. – 2nd ed. p. ; cm.Includes bibliographical references and index.ISBN-13: 978-1-4051-3488-0 (hardcover)ISBN-10: 1-4051-3488-7 (hardcover)1. Heart diseases in pregnancy. I. Oakley, Celia. II. Warnes, Carole A. [DNLM: 1. Pregnancy Complications, Cardiovascular. WQ 244 H436 2007]RG580.H4H43 2007 618.3–dc222006024341ISBN: 978-1-4051-3488-0

A catalogue record for this title is available from the British Library

Set in 9.5/12pt Meridien by SNP Best-set typesetter Ltd., Hong KongPrinted and bound in Singapore by Fabulous Printers Pte Ltd.

Commissioning Editor: Mary BanksEditorial Assistant: Victoria PitmanDevelopment Editor: Fiona PattisonProduction Controller: Debbie Wyer

For further information on Blackwell Publishing, visit our website:http://www.blackwellpublishing.com

The publisher’s policy is to use permanent paper from mills that operate a sustainableforestry policy, and which has been manufactured from pulp processed using acid-free andelementary chlorine-free practices. Furthermore, the publisher ensures that the text paperand cover board used have met acceptable environmental accreditation standards.

Blackwell Publishing makes no representation, express or implied, that the drug dosages inthis book are correct. Readers must therefore always check that any product mentioned inthis publication is used in accordance with the prescribing information prepared by themanufacturers. The author and the publishers do not accept responsibility or legal liabilityfor any errors in the text or for the misuse or misapplication of material in this book.

Contents

Contributors, viiPreface, xAcknowledgments, xi

1 Overview, 1Celia Oakley

2 Physiological changes in pregnancy, 6Candice K Silversides, Jack M Colman

3 Cardiovascular examination in pregnancy and the approach to diagnosis ofcardiac disorder, 18Petros Nihoyannopoulos

4 Acyanotic congenital heart disease, 29Heidi M Connolly, Celia Oakley

5 Cyanotic congenital heart disease, 43Carole A Warnes

6 Pregnancy and pulmonary hypertension, 59Joseph G Parambil, Michael D McGoon

7 Rheumatic heart disease, 79Bernard Iung

8 Mitral valve prolapse, 96Bernard Iung

9 Artificial heart valves, 104James R Trimm, Lynne Hung, Shahbudin H Rahimtoola

10 Management of pregnancy in Marfan syndrome, Ehlers–Danlos syndromeand other heritable connective tissue disorders, 122Lilian J Meijboom, Barbara JM Mulder

11 Heart disease, pregnancy and systemic autoimmune diseases, 136Guillermo Ruiz-Irastorza, Munther A Khamashta, Graham RV Hughes

12 Pulmonary disease and cor pulmonale, 151Claire L Shovlin, Anita K Simonds, JMB Hughes

v

13 Hypertrophic cardiomyopathy and pregnancy, 173Jorge R Alegria, Rick A Nishimura

14 Peripartum cardiomyopathy, other heart muscle disorders and pericardialdiseases, 186Celia Oakley

15 Coronary artery disease, 204Celia Oakley

16 Heart rhythm disorders, 217David Lefroy, Dawn Adamson

17 Pulmonary embolism, 243Celia Oakley

18 Hypertensive disorders of pregnancy, 264Alexander Heazell, Philip N Baker

19 Management of labour and delivery in the high-risk patient, 281Kirk D Ramin

20 Anesthesia and the pregnant cardiac patient, 290Gurinder Vasdev

21 Cardiac percutaneous intervention and surgery during pregnancy, 304Patrizia Presbitero, Giacomo Boccuzzi, Felice Bruno

22 Genetic counseling, 316Michael A Patton

23 Contraception for the cardiac patient, 327Philip J Steer

Index, 343

vi Contents

Contributors

Dawn Adamson, MB, BS, MRCP, PhDSpecialist Registrar in Cardiology, Cardiology Department, Hammersmith Hospital, London, UK

Jorge R Alegria, MDFellow in Cardiovascular Diseases, Mayo Graduate School of Medicine, Rochester, Minnesota, USA

Philip N Baker, BMedSci, BM, BS, FRCOG, DMProfessor of Maternal and Fetal Health, Head of the Medical School, University of Manchester, UK

Giacomo G Boccuzzi, MDDepartment of Invasive Cardiology and Coronary Care, Ospedale Humanitas, Milan,Italy

Felice L Bruno, MD, FICS, FACCAssociate Director, El Paso Southwestern Cardiovascular Associates, El Paso; Clinical Associate Professor of Surgery, Texas Technical University, El Paso, Texas, USA

Jack M Colman, MD, FRCPCStaff Cardiologist and Co-director, Heart Diseases in Pregnancy Program, Toronto Congenital Cardiac Centre for Adults, Mount Sinai Hospital and Toronto General Hospital/UHN; and Associate Professor of Medicine, University of Toronto; Toronto Ontario, Canada

Heidi M Connolly, MD, FACCProfessor of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

Alexander Heazell, MBChB (Hons)Clinical Research Fellow University of Manchester, UK

Graham RV Hughes, MD, FRCPLupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London; and TheLondon Lupus Centre, London Bridge Hospital, London, UK

JMB Hughes, DM, FRCPProfessor Emeritus, Imperial College School of Medicine, London, UK

vii

Lynne Hung, MDFellow in Cardiology, From Griffith Center, Division of Cardiovascular Medicine, Depart-ment of Medicine; and LAC USC Medical Center, Keck School of Medicine at University ofSouthern California, Los Angeles, California, USA

Bernard Iung, MDCardiology Department, Bichat Hospital, Paris, France

Munther A Khamashta, MD, FRCP, PhDSenior Lecturer/Consultant Physician, Director, Lupus Research Unit, The Rayne Insti-tute, St Thomas’ Hospital, London, UK

David Lefroy, MD, FRCPSenior Lecturer and Consultant Cardiologist, Cardiology Department, HammersmithHospital, London, UK

Michael D McGoon, MDProfessor of Medicine, Director, Pulmonary Hypertension Clinic, Mayo Clinic College ofMedicine, Rochester, Minnesota, USA

Lilian J Meijboom, MD, PhDDepartment of Radiology, Onze Lieve Vrouwe Ziekenhus, Amsterdam, The Netherlands

Barbara JM Mulder, MD, PhDProfessor of Cardiology, Cardiology Department, Academic Medical Center, Amsterdam,The Netherlands

Petros Nihoyannopoulos, MD, FRCP, FACCProfessor of Cardiology, Hammersmith Hospital, Imperial College School of Medicine,London, UK

Rick A Nishimura, MDJudd and Mary Morris Leighton Professor of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

Celia Oakley, MD, FRCP, FESC, FACCProfessor (Emeritus) of Clinical Cardiology, Hammersmith Hospital, London, UK

Joseph G Parambil, MDAssistant Professor, Cleveland Clinic Lerner College of Medicine, Consultant, Departmentof Pulmonary and Clinical Care Medicine, Cleveland Clinic, Ohio, USA

Patricia Presbitero, MDDirector of Department of Invasive Cardiology and Coronary Care, Ospedale Humanitas,Milan, Italy

viii Contributors

Shahbudin H Rahimtoola, MB, FRCP, MACP, MACC, DSc(Hon)Professor University of Southern California G.C. Griffith Professor of Cardiology; Profes-sor of Medicine Keck School of Medicine at USC; Griffith Center, Division of Cardiovascu-lar Medicine, Department of Medicine; and LAC USC Medical Center, Keck School ofMedicine at University of Southern California, Los Angeles, California, USA

Kirk D Ramin, MDAssociate Professor, Head, Division of Maternal Fetal Medicine, Department of Obstetricsand Gynecology, and Director, Maternal Fetal Medicine Fellowship Program, Universityof Minnesota, Minneapolis, USA

Guillermo Ruiz-Irastorza, MD, PhDConsultant Physician, Professor of Medicine, Department of Internal Medicine, Hospitalde Cruces, University of the Basque Country, Bizakaia, Spain

Claire L Shovlin, PhD, FRCPSenior Lecturer, Cardiac Sciences, NHLI, Imperial College and Honorary Cansultant inRespiratory Medicine, Hammersmith Hospital, London, UK

Candice K Silversides, MD, FRCPCAssistant Professor of Medicine (Cardiology), University of Toronto; Toronto CongenitalCardiac Centre for Adults; University of Toronto Cardiac Diseases in Pregnancy Program,Mount Sinai Hospital and Toronto General Hospital, Toronto, Ontario, Canada

Anita K Simonds, MD, FRCPConsultant in Respiratory Medicine, Academic Department of Sleep and Breathing,Royal Brompton Hospital, London, UK

Philip J Steer, BSc, MB, BS, MD, FRCOGProfessor of Obstetrics and Gynaecology, Academic Department of Obstetrics and Gynae-cology, Imperial College London, Faculty of Medicine, Chelsea and Westminster Hospital,London UK

James R Trimm, MDFellow in Cardiology, From Griffith Center, Division of Cardiovascular Medicine, Depart-ment of Medicine, LAC USC Medical Center, Keck School of Medicine at University ofSouthern California, Los Angeles, California, USA

Gurinder Vasdev, MD, FRCAnaes, FFARCSIAssistant Professor of Anesthesia and Perinatology,Mayo Clinic College of Medicine, Rochester, Minnesota, USA

Carole A Warnes, MD, FRCP, FACCProfessor of Medicine, Mayo Clinic Consultant, Division of Cardiovascular Disease, Internal Medicine and Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA

Contributors ix

The second edition, like the first one, is intended to provide practical guidanceto clinicians looking after patients with heart disease, or who may be at risk ofcardiac problems, in pregnancy and the puerperium. These will be hospitalphysicians and cardiologists, obstetricians, general practitioners and specialistnurses who provide direct care as well as the anaesthetists responsible for mak-ing delivery safe and the geneticists who answer the many questions posed bycouples with a personal or family history of heart disease.

All of our contributors were chosen for the wealth of their personal clinicalexperience of pregnancy in a particular area of cardiovascular-respiratory dis-ease. While modern cardiology has a broader evidence basis from clinical trialsthan any other speciality such evidence is singularly lacking for pregnancy inwhich practice is based at best on cohort studies, otherwise it relies on literaturereviews, anecdote and personal experience. Clinical trials are sparse even in thearea of hypertension and this will always be so because numbers are inevitablysmall and neither clinicians nor patients feel comfortable about randomisationinto trials at this time. National registries may be a potential solution for the future.

Antenatal cardiac clinics and the practice of shared care with local cardiolo-gists and general practitioners has expanded since the first edition, helped espe-cially by the creation of regional centres for grown up congenital heart diseaseand combined clinics. Regional centres mean longer journeys but shared carereduces their frequency and brings patients access to local help when they needit. We hope you will find what you need in these pages.

x

Preface

We are grateful to all our contributors for responding to the call to write, fortheir enthusiastic participation and, mostly, on-time delivery. We thank MaryBanks, Veronica Pock and Fiona Pattison of Blackwells for guidance. I am grate-ful to my colleague Professor Petros Nihoyannopoulos for the echocardiogramsshown in chapters 4, 14 and 17.

xi

Acknowledgments

CHAPTER 1

OverviewCelia Oakley

It is nearly a decade since the first edition and, in the second edition, I am joinedby my friend and colleague Professor Carole Warnes as co-editor. Together wehave gathered our most wanted contributors from both sides of the Atlantic andfrom Europe.

Much has happened: exponential advances in the practice of cardiology andcontinued evolution of our case mix of pregnant patients with heart disease.The increasing success of neonatal surgery allows more and more infants withcomplex anomalies to reach adulthood, wanting normal lives with jobs andfamilies. Except in developing countries, women with congenital heart diseasenow far outnumber those with rheumatic heart disease which used to be foundin up to 1 per cent of all pregnant women. Career women postponing pregnan-cy account for larger numbers of older patients with hypertension and athero-matous coronary disease.

Heart disease is the third most common cause of maternal death and the lead-ing non-obstetric cause. Some heart conditions, such as pulmonary embolism,arrhythmias, hypertension in pre-eclamptic toxemia and peripartum car-diomyopathy, develop as a complication of pregnancy in previously healthywomen, but women with pre-existing heart disease may be predisposed tosome of these complications and less able to cope with them.

Most women with heart disease who are in New York Heart Association classI or II before pregnancy accomplish pregnancy safely, but exceptions includepatients with fixed left-sided obstruction such as mitral or aortic stenosis orthose who have pulmonary vascular disease or fragile aortas. The risk is obvi-ously high in women with NYHA class III or IV symptoms before pregnancy. Sig-nificant heart conditions are usually known about before pregnancy but thereare important exceptions that paradoxically include just these high-risk condi-tions: pulmonary hypertension, mitral stenosis, some cardiomyopathies, thefragile aorta, atrial septal defect (ASD) and, nowadays, coronary artery diseaseas well.

Pregnant women do not want to travel and so they seek local care but mostpregnant women have normal hearts, so local experience of heart disease islikely to be sparse. Women with known or suspected heart disease planningpregnancy, or pregnant women with unexplained shortness of breath, need to be referred for full diagnosis at a specialist center where the conduct of

1