A Comprehensive Approach to Congenital Heart Diseases IB Vijayalakshmi, P Syamasundar Rao, Reema Chugh
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1A Comprehensive Approach to CONGENITAL HEART DISEASES2
3A Comprehensive Approach to CONGENITAL HEART DISEASES
Editor-in-Chief IB VijayalakshmiMD, DM, FICC, FIAMS, FIAE, FCSI, FICP, FAMS, DSc Professor of Pediatric Cardiology Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka INDIA Editors P Syamasundar RaoMD, DCH, FAAP, FACC, FSCAI Professor of Pediatrics and Medicine Emeritus Chief of Pediatric Cardiology UT-Houston Medical School Houston, Texas, USA Reema ChughMD, FACC Consultant Cardiology/Adult Congenital Heart Disease and Heart Disease in Pregnancy Kaiser Permanente Medical Center Panorama City, California, USA Foreword Dr Joseph K Perloff
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A Comprehensive Approach to Congenital Heart Diseases
First Edition: 2013
9789350902677
Printed at
5Dedicated to
Maude Abbott
First to publish An Atlas of Congenital Heart Disease
Madam Helen Taussig
Mother of Pediatric Cardiology
Dr Joseph K Perloff
Master teacher of Congenital Heart Diseases
Dr S Padmavati
A doyenne of Pediatric Cardiology in India
who has inspired many like me to become Cardiologists
—Dr IB Vijayalakshmi
My teachers
Dr Lavanya Muhkerjee, Dr Herman W Lipow, Dr Norman J Sissman Dr Jerome Liebman, Dr Leonard M Linde
My parents
Dr PVB Krishna Rao, Dr Patnana Savithramma
My wife and children
Dr Hymavathi
Dr Vijay Kumar, Dr Madhavi, Dr Radhika
—Dr P Syamasundar Rao
Above all
To all the patients
Past, present and future
Who are our best teachers
and
For whom the quest for knowledge continues…
—Dr Reema Chugh6
7Contributors
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9
10
11Foreword
This thousand plus page book is a remarkable achievement that addresses the seemingly impossible task of the spectrum of congenital heart disease from the third week of intrauterine life to late end-of-life issues. Three exceptional Editors were chosen to achieve this goal—IB Vijayalakshmi, P Syamasundar Rao, and Reema Chugh. Together, they represent three generations who have witnessed the major advances since the first blue baby operation at the Johns Hopkins Hospital in 1944.
Survival into adulthood and the issues confronting adults with congenital heart disease have added yet another dimension with Congenital Heart Disease in Adults now a subspecialty in its own right. In the United States, there are currently more adults with congenital heart disease than there are infants and children.
An attractive feature of the book is the seamless continuity from embryo, to neonate, child, adolescent, and adult. Thirteen Sections are written by separate author(s), but the text reads as single-authored.
The first major facility in the English-speaking world dedicated to treating the young was the Hospital for Sick Children in London established in 1852 with the aid of Charles Dickens. The second major facility was the Children's Hospital of Philadelphia founded three years later. The Children's Hospital of Boston opened in 1869. Until the turn of the 20th century, however, these institutions were little more than dim lights of hope in the darkness of pediatric medicine.
Where was congenital heart disease? Osler's Principles and Practice of Medicine devoted a scanty five pages to Congenital Affections of the Heart. Holt's Diseases of Infancy and Childhood devoted seven pages to Congenital Anomalies of the Heart.
In 1929, in Eberswalde near Berlin, Werner Forssman performed the world's first cardiac catheterization on himself. The department chief warned him not to do it, but Forssman ignored him. A nurse tried to stop him, but he tied her to the operating table to keep her out of the way. Into his own antecubital vein, Forssman introduced a cannula through which he passed a 65 cm urethral catheter, and then walked up a flight of stairs to the X-ray department where a photograph showed the catheter tip in his right atrium. The image revolutionized cardiology. Despite later joining the Nazi party, Nobel Laureate Forssman died on June 1, 1979.
In 1896, two Viennese scientists, Edward Haschek and TO Lindenthal, injected liquid calcium carbonate into the hand vessels of a cadaver, producing an image of the vascular system. In the 1930s, George Robb and Israel Steinberg at Bellevue Hospital in New York developed angiography and perfected angiography as a practical technique. Iodine-based contrast materials were injected into the blood vessels of rabbits, and in 1937, into human beings. Castellanos, Pereiras, and Garcia in Havana, visualized the right cardiac chambers in infants and children. The internal structure of the living heart had been revealed for the first time…
In 1956, Cournand, Dickinson and Forssman were awarded the Nobel Prize in Physiology or Medicine for their discoveries concerning heart catheterization and pathological changes in the circulatory system.
Each of the thirteen Sections of the book is subdivided into beautifully illustrated chapters— Embryo to the Neonate, Basics, Defects in Atrioventricular Connections, Shunt Defects, Right and Left Ventricular Obstructive Lesions, Congenital Valvar Lesions, Diseases of the Aorta, Cyanotic Heart Disease, Congenital Cardiomyopathies, Congenital Heart Disease in Adults, Electrophysiological Issues in Children, Miscellaneous, and General Issues.
A Comprehensive Approach to Congenital Heart Diseases by IBVijayalakshmi, P Syamasundar Rao, and Reema Chugh deals with congenital heart disease from intrauterine life to late end-of-life issues. The book is a tribute to the authors and a rare gift to the reader.
Joseph K Perloff md
Streisand/American Heart Association
Professor of Medicine and Pediatrics Emeritus
Ahmanson/UCLA Adult Congenital Heart Disease Center
University of California –Los Angeles School of Medicine
Los Angeles, California, USA
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13Prologue
I began my formal training in adult cardiology and pediatric cardiology, after completing postgraduate studies in the UK, under the great and foremost pediatric cardiologist, Dr Helen Taussig at the Harriet Lane Home, Johns Hopkins Hospital, Baltimore, USA. There could not have been a more exciting time for the specialty. Dr Robert Gross had ligated a patent ductus arteriosus (PDA) and the first Blalock-Taussig (BT) shunt had been performed by Dr Alfred Blalock at Hopkins a few years later. The clinic at Harriet Lane was full of patients from all over the world. It was one of the best periods of my life.
Since my return to India, I have been dealing mostly with adult cardiology, a large pediatric component of rheumatic heart disease, and with congenital heart disease (CHD) in both children and adults. Looking back over a half century, the developments in this field are breathtaking. At the Johns Hopkins Hospital, in the fifties we depended on auscultation, the electrocardiogram (ECG), X-ray, fluoroscopy (a very large slice) and cardiac catheterization for diagnosis even in small infants. Surgery was at that time limited to closed procedures for CHD (BT or aorto-pulmonary shunts, PDA ligation, coarctation of aorta repair, etc.). Today, the advent of echocardiography has done away (almost totally) with cardiac catheterization in babies. Catheter-based interventions for valve stenosis, device closure of atrial/ventricular septal defects (ASD/VSD) and PDA along with new surgical procedures using the heart-lung machine have revolutionized both diagnosis and treatment. Fetal echocardiography is helpful in the diagnosis of several malformations and is rapidly developing. Correction of some CHDs in the fetal stage has begun in a few places around the world. Genetic and molecular studies are developing in a big way.
The specialty of Pediatric Cardiology is a recognized entity today. However, there is still some pessimism about the advances. For example, despite surgery, many patients with ASD, VSD develop cardiac arrhythmias later in life. Corrected tetralogy of Fallot, transposition of the great arteries and coarctation of aorta almost always require reoperations.
I am sure that this book on congenital heart diseases will be useful for physicians, cardiologists, cardiac surgeons and all students. It will hopefully help in establishing the exact cardiac burden and cost-effective methods for excellence in pediatric heart care. Dr IB Vijayalaksmi, the co-editors and all the authors need to be congratulated for this venture.
S Padmavati frcp (lond), frcpe, fams, facc, faha, fesc, dsc (hon), phd (hon)
Founder-President, All India Heart Foundation
Director and Senior Consultant Cardiologist
National Heart Institute
New Delhi, India
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15Preface
Congenital heart diseases are the most common birth defects among neonates born worldwide. Advancements in pediatrics, congenital heart surgery, anesthesia, internal medicine and obstetric/gynecology have allowed the majority of these infants to survive from childhood into adulthood. Systematic categorization and classifications by embryologists/ pathologists had led to a fundamental understanding of these defects and their associated disorders.
It is, therefore, not surprising that most health providers including the specialists in this field are often overwhelmed by the various congenital heart defects (CHD). For a clinician to understand the entire spectrum of CHD from embryology/ pathology, clinical manifestations, diagnostics tests, management and surgical indications for CHD appears to be an insurmountable task. From making the correct diagnosis to appropriate management requires considerable knowledge and experience. In addition, rapid advances in both catheter-based interventions and surgeries for fetus to grown-up/ adult congenital heart diseases (GUCH/ACHD) needs a deeper understanding of the guidelines and appropriate use criteria (AUC) in order to facilitate proper decision-making by combining the best available scientific evidence with the collective judgment of physicians/surgeons.
Public awareness and patient education are critical to improvements in health care. Fortunately, web-based resources and media are making this knowledge more accessible to all. However, the complexities of CHD often make it difficult even for experts to provide a focused explanation as well as answer all the queries.
Although there are several textbooks on the various aspects of CHD, there are very few that are focused yet comprehensive to address all aspects of the care required for this special population. For medical/postgraduate students and practitioners, quite frequently a lot of time is spent in referring to various resources in order to pull together complete diagnostic and management strategies for one disease!
This book entitled, A Comprehensive Approach to Congenital Heart Diseases is designed to address all the practical aspects that a health provider needs to know to deliver excellent care to the children and adults with CHD. In this book a wholehearted attempt has been made to cover all aspects from embryology, fetal malformations, pathology, clinical approach, diagnostic investigations, management issues, current interventions to the surgery/heart-lung transplantation for CHD. Our ‘A to Z’ approach addresses transition of care into adulthood, long-term issues facing the adults with CHD including pregnancy, contraception and gynecological issues. Multiple distinguished authors from all over India and abroad have made heartfelt contributions to make this book come alive.
Just like the saying goes “It takes a village to raise a child”, it takes a global effort to care for an individual born with CHD from “womb to tomb”. This book hopes to reach a wide global audience comprising but not limited to medical/ postgraduate students, nurse specialists, general practitioners, pediatricians, pediatric and adult cardiologists, as well as cardiac surgeons.
This book has three editors representing the three generations who have witnessed the major advancements in this field since the first “blue baby” operation. We hope to blend the global literature, new technology and our Indian and international work experience to bring the best to our readers.
As the Editor-in-Chief, I (Dr IB Vijayalakshmi) have enjoyed collaborating with Dr P Syamasundar Rao who brings his vast experience to this book, and Dr Reema Chugh who is a specialist in adult congenital heart disease/heart disease in pregnancy in the United States. My special thanks to my associate Dr Chitra Narasimhan for her dedicated and diligent support. I am extremely grateful to her for working beyond the call of her duties. I am grateful to Dr Pradeep Vaideeswar, a pathologist par excellence, for writing the pathology section and sharing excellent pictures of pathological specimens gathered during his distinguished career, and to Dr Prasanna Simha for writing on the surgical management of various CHDs, and to Mr P Madhusudan for drawing explicit diagrams. We express our sincere gratitude to living legends, Dr S Padmavati for writing the prologue and Dr Joseph K Perloff for writing the foreword.
As a co-editor, I (Dr P Syamasundar Rao) thank DrVijayalakshmi for inviting me to co-edit the book with her and for asking me to contribute several chapters for this book. I have immensely enjoyed these tasks and feel delighted that I was able to pass on the knowledge that I acquired from my teachers, Drs Lavanya Muhkerjee (Andhra Medical College, Visakhapatnam, India), Herman W Lipow (Good Samaritan Hospital, Phoenix, Arizona, USA), Norman J Sissman (Stanford University, Palo Alto, California, USA), Jerome Liebman (Case-Western Reserve University, Cleveland, Ohio, USA), and Leonard M Linde (UCLA Medical Center, Los Angeles, California, USA) as well as from many pediatric cardiology and cardiovascular surgery 16colleagues that I, over the years, had the opportunity to interact with at Medical College of Georgia, Augusta, Georgia, USA; King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia; University of Wisconsin Medical School, Madison, Wisconsin, USA; Saint Louis University School of Medicine, St Louis, Missouri, USA and University of Texas-Houston Medical School, Houston, Texas, USA.
As a co-editor and contributor to the section on CHD in adults and genetic syndromes, I (Dr Reema Chugh) sincerely thank Dr IB Vijayalaksmi for giving me the opportunity to share the learning and experience that I have gathered from all my teachers in this field—Dr SK Khanna (GB Pant Hospital, New Delhi, India), Drs Hillel Laks and Alon S Aharon (UCLA Medical Center, USA) who introduced me to pathology/surgery in CHD; Dr Jane Somerville (Royal Brompton K Hospital, London, UK), Dr Philip A Ludbrook, Kathryn J Junge (Washington University in St Louis, USA), Dr John S Child, Dr Joseph Perloff, Mary Canobbio and Pamela Miner (UCLA Medical Center, Los Angeles, USA) for teaching me everything I know about taking care of the adults with CHD. My deepest gratitude for the inspiration and support received over the years from all the medical directors, chiefs of medicine, cardiologists, cardiac surgeons, staff, and patients at the Kaiser Permanente Medical Centers (in Panorama City and Los Angeles), USA. Many thanks to Gloria Tongson, NP, and the sonographers at the Echo Lab—Paul Junkel, Sarah Phillips, Terri McAnallen, Albert Amoranto, and Janae Johnson—who join me every day in taking care of the adults with CHD. I am grateful to our librarians, Ms Winnie Wong and Ms Hovey Lee for their diligent efforts to fulfill my literature searches. My special thanks to Drs Harold Bass, Terry Talkin, Indubala Vardhan, Sami Azzam, Kevin Landa for their editorial guidance, and to Mr Rober B Reber for his expertise as an audio-visual engineer. My sincere gratitude to my beloved teachers, Sister Michael (Carmel Convent, Udhampur, India) and Mrs Arzoo Baker (La Martiniere's Girls School, Lucknow, India) for instilling in me a lifelong love for learning. Above all, I am indebted to my parents (Colonel Prem and Sneh Chugh), my sister Gayatri, Dr Rajesh Behl, John and especially my daughter Tanisha, for their unconditional love and support in this ongoing journey.
We sincerely thank the President of CSI, Ashok Seth and President Elect, Dr PK Deb for getting it officially released by Dr His Excellency, the Vice President of India Shri Hamid Ansari, during the 64th Annual Conference of Cardiological Society of India at New Delhi.
My sincere thanks are due to Jaypee Brothers Medical Publishers (P) Ltd, especially Shri Jitendar P Vij (Group Chairman) and Mr Ankit Vij (Managing Director) who extended full cooperation to prepare this prestigious book and published it expeditiously.
IB Vijayalakshmi
P Syamasundar Rao
Reema Chugh