Cases in Adult Congenital Heart Disease - Expert Consult: Online and Print: Atlas, 1e

519 Pages·2009·69.43 MB·English

by  Michael A. Gatzoulis

CASES IN
ADULT
CONGENITAL
HEART
DISEASE
Michael A. Gatzoulis, MD, PhD, FACC, FESC
Professor of Cardiology and Congenital Heart Disease
Consultant Cardiologist
Head, Adult Congenital Heart Centre
and Centre for Pulmonary Hypertension
Royal Brompton Hospital
National Heart and Lung Institute, Imperial College
London, United Kingdom
Gary D. Webb, MD, FRCPC, FACC
Professor of Medicine
Director of the Philadelphia Adult Congenital Heart Center
University of Pennsylvania School of Medicine
The Hospital of the University of Pennsylvania
Philadelphia, Pennsylvania
Craig S. Broberg, MD
Assistant Professor
School of Medicine
Director, Adult Congenital Heart Disease Program
Oregon Health & Science University
Portland, Oregon
Hideki Uemura, MD
Consultant Cardiac Surgeon
Royal Brompton Hospital
London, United Kingdom
1600 John F. Kennedy Boulevard
Suite 1800
Philadelphia, PA 19103-2899
CASES IN ADULT CONGENITAL HEART DISEASE ISBN: 978-0-443-06712-9
Copyright © 2010 by Churchill Livingstone, an imprint of Elsevier Inc.
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Notice
Knowledge and best practice in this field are constantly changing. As new research and
experience broaden our knowledge, changes in practice, treatment and drug therapy may become
necessary or appropriate. Readers are advised to check the most current information provided
(i) on procedures featured or (ii) by the manufacturer of each product to be administered, to
verify the recommended dose or formula, the method and duration of administration, and
contraindications. It is the responsibility of the practitioner, relying on their own experience and
knowledge of the patient, to make diagnoses, to determine dosages and the best treatment for
each individual patient, and to take all appropriate safety precautions. To the fullest extent of the
law, neither the Publisher nor the Editors assume any liability for any injury and/or damage to
persons or property arising out or related to any use of the material contained in this book.
The Publisher
Library of Congress Cataloging-in-Publication Data
Cases in adult congenital heart disease : atlas with DVD / [edited by]
Michael A. Gatzoulis … [et al.]. – 1st ed.
p. ; cm.
Includes bibliographical references.
ISBN 978-0-443-06712-9
1. Congenital heart disease–Case studies–Atlases. I. Gatzoulis, Michael A. II. Title.
[DNLM: 1. Heart Defects, Congenital–Atlases. 2. Heart Defects, Congenital–Case
Reports. 3. Adult. WG 17 C338 2009]
RC687.C375 2009
616.1’2043–dc22
2009011148
Executive Publisher: Natasha Andjelkovic
Developmental Editor: Agnes H. Byrne
Design Direction: Steven Stave
Working together to grow
libraries in developing countries
Printed in China www.elsevier.com | www.bookaid.org | www.sabre.org
Last digit is the print number: 9 8 7 6 5 4 3 2 1
To my late father Athanasios Konstantinos Gatzoulis (1921–2008).
MAG
To the patients I have had the privilege of serving in Toronto and Philadelphia, to
the excellent colleagues who have helped me care for them, and to all the ACHD
patients whose lives we value.
GDW
To patients with congenital heart disease throughout the world, who have done the
real work represented herein.
CSB
To all the patients for whom I have had the privilege of providing care and to all my
colleagues and mentors.
HU
Contributors
Dominic J. Abrams, MD, MRCP Lee N. Benson, MD
Consultant Cardiologist, St. Bartholomew’s and Great Professor of Pediatrics, University of Toronto School of
Ormond Street Hospitals, London, United Kingdom Medicine; Director, Cardiac Diagnostic and Interventional
Catheter Ablation for Atrial Arrhythmia Unit, The Hospital for Sick Children, Toronto, Ontario,
Canada
William Alazawi, MA (Cantab), MB BChir, PhD, Catheter Intervention for Baffle Leak or Venous Obstruction
MRCP
Academic Specialist Registrar, Barts and The London School Philipp Bonhoeffer, MD
of Medicine & Dentistry, Queen Mary University of London, Professor of Cardiology, Institute of Child Health; Chief
London, United Kingdom of Cardiology, Director of the Cardiac Catheterisation
Syncope in a Patient with Noonan Syndrome Laboratory, Great Ormond Street Hospital for Children,
London, United Kingdom
Dimitrios Alexopoulos, MD Catheter Implantation of Stented Pulmonary Valve
Professor and Director of Cardiology, Patras University
Hospital, Rion, Patras, Greece Beatriz Bouzas, MD
Dyspnea in a Patient with a Loud Murmur Consultant, Cardiology Department, Complejo Hospitalario
Universitario Juan Canalejo, Coruña, Spain
Khalid Alnajashi, MD Pregnancy and Fetal Death in a Patient with Ebstein Anomaly;
Division of Pediatric Cardiology, Prince Sultan Cardiac Congenital Pulmonary Stenosis Turned to Pulmonary
Center, Riyadh, Saudi Arabia Regurgitation
Late Outcome Following Systemic Tricuspid Valve Replacement
William Bradlow, BM, BS
Naser M. Ammash, MD Cardiovascular Magnetic Resonance Unit, Royal Brompton
Associate Professor of Medicine, Mayo Medical School; Hospital, London, United Kingdom
Consultant, Division of Cardiovascular Diseases, Department The Role of Pulmonary Vasodilators in Pulmonary Hypertension
of Internal Medicine, Mayo Clinic, Rochester, Minnesota
Subaortic Stenosis: Indications for Surgery Craig S. Broberg, MD
Assistant Professor, School of Medicine; Director, Adult
Kai Andersen, MD, PhD Congenital Heart Disease Program, Oregon Health & Science
Consultant Cardiologist, Rikshospitalet University Hospital, University, Portland, Oregon
Oslo, Norway Straddling of the Tricuspid Valve: Long-Term Outcome; Long-Term
The Criss-Cross Heart Follow-Up of Atrioventricular Septal Defect; Congenital Pulmonary
Stenosis Turned to Pulmonary Regurgitation; Pulmonary Atresia
Panagiotis D. Arvanitis, MD with Intact Ventricular Septum; Late Complications after the
Honorary Clinical/Research Fellow, Adult Congenital Heart Arterial Switch Operation; Palliative Mustard for Transposition
Programme, Royal Brompton Hospital, London, United and Ventricular Septal Defect; The Criss-Cross Heart; Long-Term
Kingdom Survival in Eisenmenger Syndrome; Iron Deficiency in Cyanotic
Acute Presentation with Cerebral Hemorrhage Heart Disease; Pulmonary Artery Thrombosis and Recurrent
Hemoptyses in Eisenmenger Syndrome; Contraception Counseling
Sonya V. Babu-Narayan, MBBS, MRCP
Honorary Clinical Research Fellow, National Heart and Lung Qi-Ling Cao, MD
Institute, Imperial College London; Specialist Registrar in Associate Professor, Department of Cardiology, Rush
Adult Cardiology and Adult Congenital Heart Disease, Royal Medical College, Rush Center for Congenital and Structural
Brompton Hospital, London, United Kingdom Heart Disease, Rush University Medical Center, Chicago,
Timing of Pulmonary Valve Replacement; Management Illinois
of Tachyarrhythmia in Tetralogy: Ablation versus Catheter Closure of Ventricular Septal Defects
Surgery; Consideration for Automatic Implantable Cardioverter-
Defibrillator in Tetralogy Pedro A. Catarino, MD
Department of Cardiothoracic Surgery, Royal Brompton
Elisabeth Bédard, MD Hospital, London, United Kingdom
Adult Congenital Heart Centre, Royal Brompton Hospital; Successful Tricuspid Valve Repair of Ebstein Anomaly
Imperial College of Science and Medicine, London, United
Kingdom Aikaterini Chamaidi, MD
Right Heart Enlargement of Uncertain Cause (Sinus Venosus Consultant Cardiologist, University Hospital Larissa, Larissa
Atrial Septal Defect); Left Atrioventricular Valve Regurgitation: Medical School, Larissa, Greece
Criteria for Intervention; Late Complications after the Arterial Long-Term Outcome after Rastelli Repair
Switch Operation; Intracardiac Thrombus in the Fontan
Circulation; Contraception Counseling
Cases in Adult Congenital Heart Disease vii
Joseph Y.S. Chan, MRCP Richard M. Donner, MD
s Associate Consultant, Division of Cardiology, Department of Clinical Professor of Pediatrics, University of Pennsylvania
or
ut Medicine and Therapeutics, Prince of Wales Hospital, Hong School of Medicine; Senior Cardiologist, The Children’s
b
ontri KTroanngscatheter Options for Atrial Arrhythmia after the Mustard HReocsoparictatal toiofn P: hCirlaitdereilap fhoria I,n Ptehrivlaendteiolpnh; iCay, aPneonsinss iynl vEabnstieain
C
Procedure Anomaly and Catheter Closure of Atrial Septal Defect
Jonathan B. Choy, MD, FRCPC, FACC Nigel E. Drury, BM (Hons), MRCS
University of Alberta; Director, Adult Echocardiography Fellow in Congenital Cardiology, Wessex Cardiothoracic
Laboratory, Associate Clinical Professor of Medicine, Centre, Southampton General Hospital, Southampton,
University of Alberta Hospital, Edmonton, Alberta, Canada United Kingdom
Anomalous Left Coronary Artery from the Pulmonary Artery Right Ventricular Outflow Obstruction in a Pregnant Woman
Louise Coats, PhD, MRCP Mark J. Earley, MD, MRCP
Clinical Research Fellow, Institute of Child Health; Clinical Consultant Cardiologist, St. Bartholomew’s Hospital,
Research Fellow, Great Ormond Street Hospital for Children, London, United Kingdom
London, United Kingdom Catheter Ablation for Atrial Arrhythmia
Catheter Implantation of Stented Pulmonary Valve
Michael A. Gatzoulis, MD, PhD, FACC, FESC
Andrew Crean, BM, MRCP, MSc, FRCR, MPhil Professor of Cardiology and Congenital Heart Disease,
Assistant Professor, University of Toronto; Staff Cardiologist Consultant Cardiologist; Head, Adult Congenital Heart
and Radiologist, Toronto Congenital Cardiac Centre for Centre and Centre for Pulmonary Hypertension, Royal
Adults, Toronto General Hospital, Toronto, Ontario, Canada Brompton Hospital, National Heart and Lung Institute,
Cor Triatriatum; Recoarctation: Criteria for Intervention Imperial College London, United Kingdom
Aortic Stenosis and Endocarditis during Pregnancy; Extraanatomic
Piers E. F. Daubeney, MA, DM, MRCP, MRCPCH Bypass Graft Repair of Coarctation; Transcatheter Options for
Honorary Senior Lecturer, National Heart and Lung Atrial Arrhythmia after the Mustard Procedure
Institute, Imperial College London; Consultant Paediatric
and Fetal Cardiologist, Royal Brompton Hospital, London, Deborah R. Gersony, MD
United Kingdom Assistant Professor of Clinical Medicine, Department of
Pulmonary Atresia with Intact Ventricular Septum Medicine and Pediatrics, Columbia University Medical
Center, New York, New York
Periklis A. Davlouros, MD Pregnancy and the Systemic Right Ventricle
Senior Lecturer in Cardiology, Patras University Hospital,
Rion, Patras, Greece Marc Gewillig, MD, PhD
Dyspnea in a Patient with a Loud Murmur Professor of Pediatric Cardiology, University Leuven,
Leuven, Belgium
Barbara J. Deal, MD Protein-Losing Enteropathy
Professor of Pediatrics, Northwestern University Feinberg
School of Medicine; Marvin E. Wodika Research Professor Derek G. Gibson, MB, FRCP
of Cardiology and Division Head, Cardiology, Children’s Royal Brompton Hospital, London, United Kingdom
Memorial Hospital, Chicago, Illinois Pericardial Constriction after Relief of Subaortic Stenosis
Arrhythmia Intervention at the Time of Total Cavopulmonary
Conversion Omer Goktekin, MD
Adult Congenital Heart Centre, Royal Brompton Hospital,
Joseph A. Dearani, MD London, United Kingdom
Consultant, Division of Cardiovascular Surgery; Director, Extraanatomic Bypass Graft Repair of Coarctation
Thoracic Surgery Resident; Consultant, Division of Surgery;
Professor of Surgery, Mayo Clinic, Rochester, Minnesota Massimo Griselli, FRCS
Subaortic Stenosis: Indications for Surgery Adult Congenital Heart Centre, Department of Cardiac
Surgery, Royal Brompton Hospital, London, United Kingdom
Gerhard-Paul Diller, MD Long-Term Outcome after Rastelli Repair
Imperial College, London, United Kingdom; University
Hospital, Muenster, Germany Maarten Groenink, MD
Atrial Septal Defect Associated with Pulmonary Hypertension— Associate Professor, Department of Cardiology, Academic
Cause and Effect; Erythrocytosis with Normal Oxygen Saturation Medical Center, Amsterdam, The Netherlands
Recurrent Aortic Dissection in Marfan Syndrome
Konstantinos Dimopoulos, MD, MSc, PhD
Consultant and Senior Fellow, Adult Congenital Heart Sheila G. Haworth, MD, FRCP, FRCPATH,
Centre for Pulmonary Hypertension, Royal Brompton FRCPCH, FMedSci
Hospital and National Heart and Lung Institute, London, Professor of Developmental Cardiology, Institute of Child
United Kingdom Health, University College London; Honorary Consultant
Ebstein Anomaly and Sudden Cardiac Death in Paediatric Cardiology, Great Ormond Street Hospital for
Children, London, United Kingdom
Pulmonary Hypertension after Repair of Congenital Diaphragmatic
Hernia
viii  Cases in Adult Congenital Heart Disease
Howard C. Herrmann, MD Omar Khalid, MD
Professor of Medicine; Director, Interventional Cardiology The University of Chicago Pritzker School of Medicine, C
o
and Cardiac Catheterization Laboratories, University of Chicago, Illinois n
Pennsylvania Medical Center, Philadelphia, Pennsylvania Catheter Closure of Ventricular Septal Defects trib
u
Cyanosis in Ebstein Anomaly and Catheter Closure of Atrial Septal to
Defect Sachin Khambadkone, MBBS, DCH, MD, DNB, rs
MRCP(UK), CCT
Ziyad M. Hijazi, MD, MPH, FSCAI, FACC Honorary Senior Lecturer, Institute of Child Health;
Professor of Pediatrics and Internal Medicine; Director, Rush Consultant Paediatric Cardiologist, Cardiothoracic Unit,
Center for Congenital and Structural Heart Disease, Rush Great Ormond Street Hospital for Children, London, United
University Medical College, Chicago, Illinois Kingdom
Catheter Closure of Ventricular Septal Defects Catheter Implantation of Stented Pulmonary Valve
Eric M. Horlick, MD, FRCPC Arif Anis Khan, FCPS
Assistant Professor of Medicine, Toronto General Hospital, Research Fellow, Cardiology Department, Royal Brompton &
Toronto, Ontario, Canada Harefield NHS Trust, London, United Kingdom
Catheter Closure of a Patent Ductus Arteriosus Patent Foramen Ovale with Transient Ischemic Attack; Arrhythmia
and Syncope in a Patient with a Childhood Murmur; Pulmonary
Emmeline F. Hou, MD Atresia with Intact Ventricular Septum; Late Complications after
Cardiologist, The Permanente Medical Group, Inc., Roseville, the Arterial Switch Operation
California
Scimitar Syndrome; Endocarditis in a Young Man; Long-Term Christoph Kiesewetter, MD
Survival in Eisenmenger Syndrome Kings College London; Consultant Cardiologist, Guy’s and St
Thomas’s NHS Foundation Trust, London, United Kingdom
Ross J. Hunter, MRCP Right Ventricular Outflow Obstruction in a Pregnant Woman
Research Fellow, Cardiology Research Department, St
Bartholomew’s Hospital, London, United Kingdom Philip J. Kilner, MD, PhD
Arrhythmia and Syncope in a Patient with a Childhood Murmur Reader in CMR, Imperial College London; Consultant in
CMR, Royal Brompton Hospital, London, United Kingdom
Toru Ishizaka, MD, PhD Consideration for Automatic Implantable Cardioverter-Defibrillator
Chief Cardiovascular Surgeon, Higashi Takarazuka Satoh in Tetralogy
Hospital, Takarazuka City, Hyogo, Japan
Atrial Septal Defect with Cyanosis: The Hypoplastic Right Igor Knez, MD
Ventricle; Ventricular Septal Defect and the Aortic Valve; Total Professor of Cardiovascular Surgery, Medical University of
Cavopulmonary Conversion: When and How Graz; Clinical Department of Cardiac Surgery, University
Clinic of Surgery, Graz, Austria
Bengt Johansson, MD, PhD Congenitally Corrected Transposition of the Great Arteries with
Cardiovascular Magnetic Resonance Unit, Royal Brompton Pulmonary Stenosis and Ventricular Septal Detect: When to
Hospital; Imperial College of Science and Medicine, London, Intervene
United Kingdom
Intracardiac Thrombus in the Fontan Circulation Masahiro Koh, MD
Clinical Fellow, Paediatric Cardiac Surgery, Royal Brompton
Sofian Johar, MB BChir, PhD, MRCP Hospital, London, United Kingdom
Specialist Registrar in Cardiology, Royal Brompton Hospital, Partial Anomalous Pulmonary Venous Return; Ebstein Anomaly
London, United Kingdom and Wolff-Parkinson-White Syndrome; Complications of
Aortic Stenosis and Endocarditis during Pregnancy Ventricular Septation in a Patient with a Single Ventricle
Lesley Jones George Krasopoulos, MD, PhD, FRCS-CTH
Clinical Nurse Specialist, Adult Congenital Heart Centre, Consultant Cardiac Surgeon, Liverpool Heart & Chest NHS
Royal Brompton Hospice, London, United Kingdom Trust, Liverpool, United Kingdom
Contraception Counseling Successful Tricuspid Valve Repair of Ebstein Anomaly
Henryk Kafka, MD, FRCPC, FACP, FACC Yat-Yim Lam, MRCP
Departments of Cardiology and Radiology, Queen’s Associate Professor, Division of Cardiology, Department of
University, Kingston, Ontario, Canada Medicine and Therapeutics, Prince of Wales Hospital, The
Late Repair of Tetralogy of Fallot; Assessment of Systemic Right Chinese University of Hong Kong, Hong Kong
Ventricle Function; The Criss-Cross Heart Transcatheter Options for Atrial Arrhythmia after the Mustard
Procedure
Thomas K. Kaltsas, MD
Clinical and Research Fellow, Department of Adult Astrid E. Lammers, MD
Congenital Heart Disease, Royal Brompton Hospital, University College London; Fellow, Pulmonary
London, United Kingdom Hypertension, Great Ormond Street Hospital for Children,
Outflow Tract Obstruction after Atrioventricular Septal Defect London, United Kingdom
Repair Pulmonary Hypertension after Repair of Congenital Diaphragmatic
Hernia
Ageliki A. Karatza, DS
Senior Lecturer in Paediatric Cardiology, Patras University
Hospital, Rion, Patras, Greece
Dyspnea in a Patient with a Loud Murmur
Cases in Adult Congenital Heart Disease ix
Wei Li, MD, PhD Michael J. Mullen, MD, FRCP
s Adult Congenital Heart Disease Centre, Royal Brompton Consultant Cardiologist, Cardiology Department, Royal
or
ut Hospital; National Heart Lung Institute, Imperial College Brompton & Harefield NHS Trust, London, United Kingdom
b
ontri SRcighhoto Hl oeafr St cEiennlacreg eamnedn tM oef dUincicneretain Cause (Sinus Venosus RSyanticoonpael ei nfo ar PSeaptiteanl tD weiftehct a C Clohsiuldrhe oiond t Mhe uErlmdeurrly; Arrhythmia and
C
Atrial Septal Defect); Rationale for Septal Defect Closure in the
Elderly Ed Nicol, MD, MRCP
Cardiology and General (Internal) Medicine Specialist
Per Lunde, MD Registrar, John Radcliffe Hospital, Oxford, United Kingdom;
Senior Consultant Cardiologist, University Hospital of Cardiac Imaging Fellow, National Heart and Lung Institute,
Northern Norway, Tromsø, Norway Royal Brompton Hospital, London, United Kingdom
Long-Term Follow-Up of Atrioventricular Septal Defect; Pacemaker Fever in a Patient with a Single Ventricle
Infection in a Cyanotic Patient; Iron Deficiency in Cyanotic Heart
Disease Elena Nikiphorou, MD
Royal Brompton Hospital, London, United Kingdom
Jonathan Lyne, MRCP Outflow Tract Obstruction after Atrioventricular Septal Defect
Royal Brompton Hospital, Cardiovascular Magnetic Repair
Resonance Unit, London, United Kingdom
Ventricular Arrhythmia Following a Ross Procedure Koichiro Niwa, MD, PhD
Director, Department of Adult Congenital Heart Disease,
Vaikom S. Mahadevan, MD, MRCP (UK) Chiba Cardiovascular Center, Chiba, Japan
Consultant Cardiologist, Manchester Heart Centre, Aortopathy in Tetralogy of Fallot
Manchester Royal Infirmary, Manchester, United Kingdom
Catheter Closure of a Patent Ductus Arteriosus; Recoarctation: Erwin Oechslin, MD
Criteria for Intervention; Catheter Intervention for Baffle Leak or Associate Professor, University of Toronto; Director, Toronto
Venous Obstruction Congenital Cardiac Centre for Adults, Toronto, Ontario,
Canada
Constantine Mavroudis, MD Management of Acute Hemoptysis
Professor of Surgery, Case Western Reserve University
School of Medicine; Ross Professor of Surgery, Chairman of George Pantely, MD
Pediatric and Congenital Heart Surgery, Cleveland Clinic Professor of Medicine (Cardiology), Adult Congenital Heart
Foundation, Cleveland, Ohio Disease Program, Oregon Health & Science University,
Arrhythmia Intervention at the Time of Total Cavopulmonary Portland, Oregon
Conversion Scimitar Syndrome; Endocarditis in a Young Man; Long-Term
Survival in Eisenmenger Syndrome
Peter R. McLaughlin, MD, FRCP(C)
Clinical Adjunct Professor of Medicine, University of Sabrina D. Phillips, MD
Toronto; Chief of Staff, Peterborough Regional Health Assistant Professor of Medicine, Mayo College of Medicine;
Centre, Peterborough, Ontario, Canada Consultant, Cardiovascular Disease and Internal Medicine,
Catheter Closure of a Patent Ductus Arteriosus; Catheter Mayo Clinic, Rochester, Minnesota
Intervention for Baffle Leak or Venous Obstruction Interrupted Aortic Arch in a Patient with DiGeorge Syndrome;
Coronary Artery Fistulae and Their Significance
Victor Menashe, MD
Department of Pediatrics, Oregon Health and Science Antonia Pijuan-Domenech, MD
University, Portland, Oregon Cardiologist, Hospital Vall d’Hebron, Barcelona, Spain
Scimitar Syndrome; Endocarditis in a Young Man; Long-Term Palliative Mustard for Transposition and Ventricular Septal Defect;
Survival in Eisenmenger Syndrome The Criss-Cross Heart
David B. Meyer, MD Daniele Prati, MD
Cardiothoracic Surgery, Schneider Children’s Hospital, New Research Fellow, Adult Congenital Heart Disease
Hyde Park, New York Programme, Royal Brompton Hospital, London, United
Considerations for the Ross Procedure Kingdom
Palliative Mustard for Transposition and Ventricular Septal Defect
Shelley D. Miyamoto, MD
Assistant Professor of Pediatrics, University of Colorado Peter J. Pugh, MD, MRCP, FESC
Denver Health Sciences Center; Director, Cardiomyopathy Consultant Cardiologist, Addenbrooke’s Hospital,
and Heart Failure Program, The Children’s Hospital, Cambridge University Hospitals NHS Foundation Trust,
University of Colorado, Denver, Colorado Cambridge, United Kingdom
Timing and Merits of Transplantation in a Fontan Patient Liver Dysfunction after the Mustard Procedure
Barbara J.M. Mulder, MD Ivan M. Rebeyka, MD, FRCS
Professor of Cardiology, Academic Medical Center, Clinical Professor, Department of Surgery and Pediatrics,
Amsterdam, The Netherlands University of Alberta; Head, Pediatric Cardiovascular
Recurrent Aortic Dissection in Marfan Syndrome Surgery, Stollery Children’s Hospital, Edmonton, Alberta,
Canada
Anomalous Left Coronary Artery from the Pulmonary Artery
x  Cases in Adult Congenital Heart Disease
Andrew N. Redington Mark S. Spence, MB BCH, MD, BAO (Hons),
Division Head, Department of Cardiology, Hospital for Sick MRCP C
o
CTohrioldnrteon, ;O Snetnairoior ,A Csasnocaidaate Scientist, University of Toronto, HCoonnsourlatrayn St eCcaiordr iLoelocgtuisrte, rR, oQyuael eVni’sst oUrniai vHeorssiptyit aBle, lBfaeslfta; st ntrib
u
Late Outcome Following Systemic Tricuspid Valve Replacement Trust, Belfast, Northern Ireland, United Kingdom to
rs
Multiple Aortic-Pulmonary Collaterals: Too Many or Too Few
Jonathan Rome, MD, FACC
Associate Professor of Pediatrics, University of Pennsylvania Martin St. John Sutton, MB, FRCP
School of Medicine; Director, Cardiac Catheterization John W. Bryfogle Professor of Medicine, University of
Laboratory, The Children’s Hospital of Philadelphia, Pennsylvania School of Medicine; Director, Cardiovascular
Philadelphia, Pennsylvania Imaging; Director, Cardiovascular Fellowship Program, The
Recoarctation: Criteria for Intervention Hospital of the University of Pennsylvania, Philadelphia,
Pennsylvania
Marlon S. Rosenbaum, MD Cyanosis in Ebstein Anomaly and Catheter Closure of Atrial Septal
Associate Attending, Columbia University College of Defect
Physicians & Surgeons; Director, Schneeweiss Columbia
Adult Congenital Heart Center, Columbia University Philip J. Steer, MBBS, MD, FRCOG
Medical Center, New York, New York Emeritus Professor of Obstetrics, Imperial College London;
Pregnancy and the Systemic Right Ventricle Consultant Obstetrician, Chelsea and Westminster Hospital,
London, United Kingdom
Jonathon B. Ryan, MBBS, PhD, FRACS Pregnant Patient with Unoperated Truncus Arteriosus
Department of Cardiac Surgery, Royal Brompton Hospital,
London, United Kingdom Nilesh Sutaria, MD, MRCP
Left Atrioventricular Valve Regurgitation: Criteria Consultant Cardiologist, Imperial College Healthcare NHS
for Intervention Trust, London, United Kingdom
Pericardial Constriction after Relief of Subaortic Stenosis
Richard J. Schilling, MD, MRCP
Reader, Queen Mary University of London; Consultant Lorna Swan, MB ChB, MD, MRCP
Cardiologist, St. Bartholomew’s Hospital, London, United Senior Lecturer, National Heart & Lung Institute, Imperial
Kingdom College; Consultant Cardiologist, Royal Brompton &
Catheter Ablation for Atrial Arrhythmia Harefield NHS Trust, London, United Kingdom
Ebstein Anomaly and Prepregnancy Counseling
Babulal Sethia, FRCS
Consultant Cardiac Surgeon, Royal Brompton Hospital, Jonathan Swinburn, MBBS, MD
London, United Kingdom Consultant Cardiologist, Royal Berkshire Hospital, Reading,
Management of Tachyarrhythmia in Tetralogy: Ablation versus United Kingdom
Surgery Left Atrial Isomerism
Mary N. Sheppard, MD Ju-Le Tan, MBBS, MRCP
Royal Brompton Hospital, London, United Kingdom Senior Consultant Cardiologist, Adult Congenital Heart
Pericardial Constriction after Relief of Subaortic Stenosis Disease, National Heart Centre, Singapore
Right Heart Enlargement of Uncertain Cause (Sinus Venosus
Elliot A. Shinebourne, MD, FRCP, FRCPH Atrial Septal Defect); Rationale for Septal Defect Closure in the
Consultant Paediatric Cardiologist, Royal Brompton Elderly
Hospital, London, United Kingdom
Timing of Pulmonary Valve Replacement Judith Therrien, MD
Assistant Professor of Medicine, McGill University; Adult
Darryl F. Shore, FRCS Congenital Heart Disease Fellowship Director, MAUDE Unit
Divisional Director, Heart Division, Consultant Cardiac and Beth Raby Adult Congenital Heart Disease Clinic, Jewish
Surgeon, Royal Brompton Hospital and Harefield NHS Trust, General Hospital, Montreal, Quebec, Canada
London, United Kingdom Management of Systemic Ventricular Failure
Extraanatomic Bypass Graft Repair of Coarctation; Consideration
for Automatic Implantable Cardioverter-Defibrillator in Tetralogy Eapen Thomas, MD
Grown-Up Congenital Heart Disease Unit, Queen Elizabeth
Candice K. Silversides, MD, MS, FRCPC Hospital, Edgbaston, Birmingham, United Kingdom
Assistant Professor, University of Toronto; Staff Cardiologist, Anticoagulation in a Pregnant Patient with a Mechanical Valve;
University Health Network, Toronto General Hospital, Early Appraisal of Adults with Norwood Correction
Toronto Congenital Cardiac Centre for Adults, Toronto,
Ontario, Canada Sara Thorne, MBBS, MD
Cor Triatriatum; Considerations for Tricuspid Valve Replacement Honorary Senior Lecturer, Consultant Cardiologist,
in Patients with a Systemic Right Ventricle University of Birmingham, Birmingham, United Kingdom
Anticoagulation in a Pregnant Patient with a Mechanical Valve;
Thomas L. Spray, MD Early Appraisal of Adults with Norwood Correction
Professor of Surgery, University of Pennsylvania School of
Medicine; Chief, Division of Cardiothoracic Surgery, Alice Jan Till, MD
Langdon Warner Endowed Chair, The Children’s Hospital of Consultant Electrophysiologist, Royal Brompton Hospital,
Philadelphia, Philadelphia, Pennsylvania London, United Kingdom
Considerations for the Ross Procedure Management of Tachyarrhythmia in Tetralogy: Ablation versus
Surgery; Left Atrial Isomerism
Cases in Adult Congenital Heart Disease xi
Filippos Triposkiadis, MD, FESC, FACC Gary D. Webb, MD, FRCPC, FACC
s Professor of Cardiology, University of Thessaly Medical Professor of Medicine, Director of the Philadelphia Adult
or
ut School; Director, Department of Cardiology, Larissa Congenital Heart Center, University of Pennsylvania School
b
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Considerations for Tricuspid Valve Replacement in Patients with a
Etsuko Tsuda, MD Systemic Right Ventricle; Recurrent Aortic Dissection in Marfan
Staff, Department of Pediatrics, National Cardiovascular Syndrome
Center, Suita, Osaka, Japan
Long-Term Management of Kawasaki Disease Tom Wong, MRCP
Consultant Electrophysiologist, Royal Brompton and
Tomohiro Tsunekawa Harefield Hospitals, London, United Kingdom
National Cardiovascular Center, Suita City, Osaka, Japan Ventricular Arrhythmia Following a Ross Procedure
Ventricular Septal Defect and the Aortic Valve
Toshikatsu Yagihara, MD
Anselm Uebing, MD Deputy Director and Chairman, Cardiovascular Surgery,
Fellow in Adult Congenital Heart Disease, Royal Brompton National Cardiovascular Surgery Center, Osaka, Japan
Hospital and Harefield NHS Trust, Adult Congenital Heart Partial Anomalous Pulmonary Venous Return; Ebstein Anomaly
Disease Unit, London, United Kingdom and Wolff-Parkinson-White Syndrome; Complications of
Pregnancy-Related Complications in Coarctation; Fontan and Ventricular Septation in a Patient with a Single Ventricle; Long-
Pregnancy; Pregnant Patient with Unoperated Truncus Arteriosus; Term Management of Kawasaki Disease
Left Coronary Artery Arising from the Right Coronary Sinus
Steve M. Yentis, MBBS, FRCA, MD, MA
Hideki Uemura, MD Honorary Senior Lecturer, Imperial College; Consultant
Consultant Cardiac Surgeon, Royal Brompton Hospital, Anaesthetist, Chelsea and Westminster Hospital NHS
London, United Kingdom Foundation Trust, London, United Kingdom
Atrial Septal Defect with Cyanosis: The Hypoplastic Right Pregnant Patient with Unoperated Truncus Arteriosus
Ventricle; Partial Anomalous Pulmonary Venous Return;
Ventricular Septal Defect and the Aortic Valve; Left Anji T. Yetman, MD
Atrioventricular Valve Regurgitation: Criteria for Intervention; Professor of Pediatrics, Adjunct Professor of Internal
Ebstein Anomaly and Wolff-Parkinson-White Syndrome; Successful Medicine, University of Utah; Director, Adult Congenital
Tricuspid Valve Repair of Ebstein Anomaly; Total Cavopulmonary Cardiology Program, University Hospital; Director, Adult
Conversion: When and How; Complications of Ventricular Congenital Cardiology Program, Primary Children’s Medical
Septation in a Patient with a Single Ventricle; Long-Term Center, Salt Lake City, Utah
Management of Kawasaki Disease Timing and Merits of Transplantation in a Fontan Patient
Gruschen R. Veldtman, MB ChB, MRCP, Dip Panayiotis Zarvos, MD, FCP-SA
Obstet Director, Pediatric Department, Arch. Makarios Hospital,
Consultant Cardiologist, Congenital Cardiac Unit, University Medical Health Services, Nicosia, Cyprus
of Southampton, Southampton University Hospital, Outflow Tract Obstruction after Atrioventricular Septal Defect
Southampton, United Kingdom Repair
Right Ventricular Outflow Obstruction in a Pregnant Woman
Isabelle F. Vonder Muhll, MD
Assistant Professor, University of Alberta; Staff Cardiologist,
Department of Medicine, University of Alberta Hospital,
Edmonton, Alberta, Canada
Anomalous Left Coronary Artery from the Pulmonary Artery
xii  Cases in Adult Congenital Heart Disease
Foreword
We live in an era of rapid advances in medicine, including questions about what they might expect from life with their
cardiology and cardiac surgery. Because of that, life expectancy defect, my first answer is always, “I don’t know.” Each person’s
of patients born with congenital heart disease has increased journey is dependent on the unique specifics of his or her
steadily in the past four decades. The result of this is a growing anatomy, history of care, coexisting medical conditions, and the
population of adults living with congenital heart disease. Every wild card of luck. The person best equipped to help answer
day, teens and young adults with congenital heart disease are these questions is the patient’s own adult congenital heart spe-
transitioning from the care of dedicated pediatric cardiologists cialist.
into the uncertain world of adulthood. All over the world, spe- Drs. Gatzoulis, Webb, Broberg and Uemura have been at the
cialized medical centers and groups are being formed to provide forefront of advances in care of adult congenital heart disease
care appropriate for their complex and ongoing medical for many years and have edited or contributed to numerous
needs. textbooks for medical professionals. This new book, however,
The most common reason patients join the Adult Congenital is the first to focus on specific cases. It includes 85 cases from
Heart Association is to find others living with the same condi- first presentation to the latest follow-up, and discusses the
tion in the hope of finding others with these rare diagnoses who specific challenges in each patient. By choosing to focus on
have shared their particular experiences and struggles. For most individual cases, the editors fully explore all aspects of the
of us, survival was dependent on whatever treatments were congenital heart picture and illustrate the array of trajectories
available at the time and place of our birth. As medicine that comprise life with congenital heart disease. By creating
advanced, treatments were constantly evolving. Over time, all Cases in Adult Congenital Heart Disease, the editors have created
the stents, patches, valves, and pacemakers have changed the a unique and essential tool to help ensure that these patients
structure of our hearts, further affected by the process of aging find the care they seek. They guide the medical professional to
and the specifics of our life and health. a much richer understanding of the complexity one must master
What does it mean to be an adult living with congenital heart to help ensure that each of us, patient by patient, gets the life
disease? As president of the Adult Congenital Heart Associa- and health care we deserve.
tion, I am asked this question every day. A patient facing
surgery asks, “Am I likely to feel better?” A newlywed asks, Amy Verstappen
“Do you think I should have children?” A parent of an infant President and CEO
asks, “Can my child live a normal life?” When patients ask me Adult Congenital Heart Association
Cases in Adult Congenital Heart Disease xiii