Clinical Diagnosis of Congenital Heart Disease
Clinical Diagnosis of Congenital Heart Disease
Third Edition
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Clinical Diagnosis of Congenital Heart Disease / M Satpathy, BR Mishra
First Edition: 2008
Third Edition: 2020
9789389587982
Printed at
PS Bidwai
A premier Pediatric Cardiologist of India Was a master teacher and a God-like person
and
My late parents and my late wife
—M Satpathy
My parents
Sri Dibakar Mishra and Smt Debalaxmi Mishra
and
My wife Gitanjali and son Gaurav
For their love, support and encouragement
—BR Mishra
- AK Bhattacharyya MD DM
- Professor and Head
- Department of Cardiology
- Guwahati Medical College
- Guwahati, Assam, India
- AK Samal MD DM
- Professor
- Department of Cardiology Hitech Medical College
- Bhubaneswar, Odisha, India
- AN Patnaik MD DM DNB
- Senior Consultant Cardiologist
- Department of Cardiology
- Star Hospitals
- Hyderabad, Telangana, India
- Anita Saxena MD DM
- Professor
- Department of Cardiology
- All India Institute of Medical Sciences
- New Delhi, India
- Arun Srinivas MD DM FACC FESC
- Associate Professor
- Department of Cardiology
- JSS Medical College, JSS Hospital and
- JSS University
- Mysuru, Karnataka, India
- B Dalvi MD DM FACC (USA)
- Consultant Cardiologist
- Department of Cardiology
- Glenmark Cardiac Centre
- Mumbai, Maharashtra, India
- Balu Vaidyanathan MD DNB (Pediatrics)
- Clinical Professor
- Department of Pediatric Cardiology
- Amrita Institute of Medical Sciences Kochi, Kerala, India
- BR Mishra MD DM FCSI FIAE FICC FESC
- Senior Consultant Cardiologist
- Department of Cardiology
- Max Diagnostics
- Cuttack, Odisha, India
- BRJ Kannan MD DM
- Assistant Professor
- Department of Pediatric Cardiology Amrita Institute of Medical Sciences and Research Centre
- Kochi, Kerala, India
- BSJ Nair MD DM
- Assistant Professor, Department of Cardiology, Kottayam Medical College
- Kottayam, Kerala, India
- CD Gupta MD DM
- Former Professor and Head
- Department of Cardiology
- Jammu Medical College
- Jammu, Jammu and Kashmir, India
- CG Bahuleyan MD DM
- Former Professor and Head
- Department of Cardiology
- Government Medical College Thiruvananthapuram, Kerala, India
- G Rajesh MD DM
- Assistant Professor
- Department of Cardiology
- Kottayam Medical College
- Kottayam, Kerala, India
- HN Mishra MD DM
- Former Professor
- Department of Cardiology
- SCB Medical College
- Cuttack, Odisha, India
- HN Mohan Kumar MD DM
- Associate Professor
- Department of Cardiology
- GB Pant Hospital
- New Delhi, India
- HP Guruprasad MD DM
- Assistant Professor
- Department of Cardiology
- JSS Medical College, JSS Hospital and
- JSS University
- Mysuru, Karnataka, India
- IB Vijayalakshmi MD DM FICC FICP
- Head
- Department of Pediatric Cardiology Jayadeva Institute of Cardiology
- Bengaluru, Karnataka, India
- J Jayarajah MD DM
- Professor
- Department of Cardiology
- Sri Ramachandra Hospital Cardiac Care Centre
- Chennai, Tamil Nadu, India
- J Yusuf MD DM
- Associate Professor
- Department of Cardiology
- GB Pant Hospital
- New Delhi, India
- K Sharada MD DM
- Consultant Cardiologist
- Department of Cardiology
- CARE Hospitals
- Hyderabad, Telangana, India
- M Behera MD DM
- Former Professor and Head
- Department of Cardiology
- SCB Medical College
- Cuttack, Odisha, India
- M Satpathy MD DM
- Former Professor of Cardiology
- Haripur Road, Dolamundai
- Cuttack, Odisha, India
- M Shifas Babu MD DM
- Assistant Professor
- Department of Cardiology
- Government Medical College
- Thiruvananthapuram, Kerala, India
- ML Kulkarni MD FIAP FAMS FCPCC (London)
- Professor and Head
- Department of Pediatrics
- JJM Medical College
- Davanagere, Karnataka, India
- N Desai MD DM FACC FICC FAPSIC FESC
- Professor and Head
- Department of Cardiology
- Head Cardiovascular Sciences
- JSS Medical College, JSS Hospital and
- JSS University
- Mysuru, Karnataka, India
- N Sudhayakumar MD DM
- Professor and Head
- Department of Cardiology
- Kottayam Medical College
- Kottayam, Kerala, India
- Naveen Garg MD (Medicine), DM (Cardiology), DNB (Cardiology)
- Professor
- Department of Cardiology
- Sanjay Gandhi Postgraduate Institute of Medical Sciences
- Lucknow, Uttar Pradesh, India
- PC Manoria MD DM FCCP FICP FICN FICA (USA)
- Former Professor and Head
- Department of Cardiology
- Gandhi Medical College
- Bhopal, Madhya Pradesh, India
- PK Dash MD DM
- Head, Department of Cardiology
- Sri Sathya Sai Higher Institute of Medical Sciences
- Bengaluru, Karnataka, India
- PK Pati MD DM
- Assistant Professor
- Department of Cardiology
- Christian Medical College
- Vellore, Tamil Nadu, India
- PR Gupta MD DM
- Professor and Head
- Department of Cardiology
- Institute of Medical Sciences
- Banaras Hindu University
- Varanasi, Uttar Pradesh, India
- R Juneja MD DM
- Associate Professor
- Department of Cardiology
- All India Institute of Medical Sciences
- New Delhi, India
- Rohit Manojkumar MD DM
- Associate Professor
- Department of Cardiology
- Postgraduate Institute of Medical Sciences and Research (PGIMER)
- Chandigarh, India
- S Guha Dip Card MD DM
- Associate Professor
- Department of Cardiology
- Kolkata Medical College
- Kolkata, West Bengal, India
- S Shah MD DM
- Department of Cardiology
- All India Institute of Medical Sciences
- New Delhi, India
- Sumitra Venkatesh DNB DCH
- Associate Professor
- Division of Pediatric Cardiology
- BJ Wadia Hospital for Children
- Mumbai, Maharashtra, India
- SK Trivedi MD DM
- Professor and Head
- Department of Cardiology
- Gandhi Medical College
- Bhopal, Madhya Pradesh, India
- SN Rahiman MD
- Associate Professor
- Department of Pediatrics
- JJM Medical College
- Davanagere, Karnataka, India
- SN Routray MD DM
- Professor and Head
- Department of Cardiology
- SCB Medical College
- Cuttack, Odisha, India
- SR Anil DCH MD DNB (Ped) DNB (Card)
- Consultant Pediatric Cardiologist
- Department of Pediatric Cardiology
- Apollo Hospital
- Hyderabad, Telangana, India
- SR Mittal MD DM FAMS FICP FISC
- Professor and Head
- Department of Cardiology
- Jawaharlal Nehru Medical College
- Ajmer, Rajasthan, India
- SS Prabhu MD DCH
- Professor and Head
- Division of Pediatric Cardiology
- BJ Wadia Hospital for Children
- Mumbai, Maharashtra, India
- Sunita Maheshwari ABP ABPC (USA)
- Consultant Pediatric Cardiologist
- Narayana Health (formerly Narayana Hrudayalaya)
- Bengaluru, Karnataka, India
- Sunil Kumar MD DM
- Assistant Professor
- Department of Cardiology
- JSS Medical College, JSS Hospital and
- JSS University
- Mysuru, Karnataka, India
- UA Kaul MD DM
- Former Professor and Director Department of Cardiology
- GB Pant Hospital
- New Delhi, India
- V Gouthami MD DM DNB
- Former Associate Professor
- Department of Cardiology
- Nizam's Institute of Medical Sciences
- Hyderabad, Telangana, India
It is a matter of immense pleasure that the third edition of the book Clinical Diagnosis of Congenital Heart Disease is being published. The previous editions have found to be useful for medical students, interns, residents, and fellows who deal with congenital heart diseases. Utility of the book prompted for another revised edition.
Rapid advances in medical science put a challenge to constantly keep one updated in diagnostic and therapeutic strategies which greatly impact the outcome particularly in babies with congenital heart diseases. In this revised edition we made an endeavor to update each chapter keeping in view the original intent to provide a simplified overview of various congenital heart diseases for an easy understanding of the subject. Original format of each chapter remained the same. All effort has been made carefully to keep the book comprehensive and to safeguard the need of the students and common practitioners so that they make an accurate diagnosis leading to better patient outcome.
The strength of this book has been its lucid description of basics with emphasis on clinical aspect. Major changes has been done in echocardiography and approach to management with new illustrations and figures with an effort to be concise in providing scientific information relevant in day-to-day management aiding in clinical diagnosis, investigation, and treatment. One chapter each has been provided for intervention in congenital heart disease, basic understanding of fetal echo, and the problem of congenital heart diseases in India. As in other editions references are deliberately omitted not only to reduce the volume but also for the fact that ready-references are available in this age of information technology.
We hope the third edition will continue to serve the students, residents, and practitioners alike and fulfil the expectations of the contributors and editors, who have given a tremendous effort in bringing out this updated edition.
We extend our appreciation to all the contributors and to M/S Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India and its staff for their sincere and hard effort.
M Satpathy
BR Mishra
Preface to the First Edition
Congenital heart disease is generally thought to be a difficult and challenging subject. Due to limitations of diagnostic facilities and suitable treating hospitals, many souls have departed their bodies prematurely, undiagnosed and untreated. Physicians, pediatricians and cardiologists give less attention to congenital heart diseases, as this branch of cardiovascular science is less alluring and rewarding.
As a teacher, when I aimed at the postgraduate students in medicine, I found there is a definite place for a book on congenital heart disease which should be comprehensive and also with strong clinical bias because it is essential on the part of treating physicians caring for the newborn, the infant and the children to have early diagnosis with accuracy so as to prevent high morbidity and mortality. Recently, many pediatric cardiology units have come-up and tremendous advances in percutaneous interventions and surgical techniques have been made, so the outlook for congenital heart disease looks brighter today. As a matter of fact, without basic knowledge of anatomy and pathophysiology, it is difficult to develop a clinical acumen to make early accurate diagnosis. Further, correct interpretation of recent investigatory procedures will be more difficult. Early diagnosis, initiation of treatment with existing facilities and decision for referral to a standard cardiac center at the right time can definitely save many precious lives.
Although there are many books available on this subject, there are referral books of very high standard and are expensive for individual possession. Keeping this in mind, I felt the need of a book on congenital heart disease, which can be affordable and cater to the basic needs of postgraduate students, medical teachers, pediatricians and practicing cardiologists.
Prominent cardiologists across the country have contributed many chapters from their vast experience and knowledge. I have attempted to describe all aspects of these congenital heart diseases in a uniform pattern. Elaborate description has been made on common diseases. Illustrations with simple hand-drawn sketches are given, wherever necessary depicting the abnormal anatomy and pathophysiological consequences for clear understanding of the anomaly. Controversial points and details of advanced imaging modalities are deliberately omitted. I have made a sincere effort to use simple teaching language and clear description of the basic clinical findings. Wherever necessary, the mechanisms are explained using short bracketed notes. I have tried to keep a link between pre-echo era and present-day investigatory tools as regards clinical diagnosis is concerned.
Basically, being a clinical book, management of diseases are described in brief (elaborate management schedules are beyond the scope of this book).
References are deliberately omitted to reduce the bulk of the book, as ready references are available in these days of advance information technology. A separate chapter is included on the incidence of congenital heart disease in India and the present status of management facilities available across the country.
I have put my hard and sincere efforts in giving a definite shape to the book. My dream and vision will be fulfilled, if this book becomes useful for the students of medicine, pediatrics and physicians dealing with cardiovascular science related to congenital heart disease.
M Satpathy
Acknowledgments
I am greatly indebted to my late teachers Dr PL Wahi, Dr PS Bidwai and Dr HN Khattri of Postgraduate Institute of Medical Sciences and Research (PGIMER), Chandigarh, India, whose art of teaching convinced the students that reaching at a correct bedside diagnosis in congenital heart disease is not that difficult. I owe my gratitude to all my teachers, particularly to Dr JP Das and Dr Upendra Kaul. Their master teaching on the subject gave me the inspiration for writing a clinical book for the students of medicine.
I am grateful to all the contributors for their benevolent and valuable contribution in spite of their busy schedule without which this book could not have become a reality.
My daughters Sanghamitra, Anuradha and Madhulekha deserve special thanks who remained a source of continuous inspiration for me while preparing the manuscript.
My special thanks to my other editor Dr BR Mishra, for his dedicated hard work, overall supervision, and particularly for preparing schematic diagrams depicting diseases for better and easier understanding of students. It is a rare combination that not only, he is a cardiologist but also an excellent artist. I also thank Dr Gitanjali Kar (w/o Dr BR Mishra) for her wholehearted cooperation.
I am grateful to Shri Jitendar P Vij and Mr Ankit Vij, M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India. My special thanks to Mr MS Mani, Dr Richa Saxena and Dr Nidhi Sood for their kind cooperation in helping us to publish this edition in time. Finally, my sincere thanks to all the associates of the company with special mention to Mr KK Raman and Mr Sunil Dogra.
Abbreviations
A2
Aortic component of second heart sound
AA
Aortic arch
AF
Atrial fibrillation
ALCAPA
Anomalous left coronary artery from pulmonary artery
AMI
Acute myocardial infarction
Ao
Aorta
AP window
Aortopulmonary window
AR
Aortic regurgitation
AS
Aortic stenosis
ASD
Atrial septal defect
AV
Aortic valve
AV valve
Atrioventricular valve
AVSD
Atrioventricular septal defect
BP
Blood pressure
BDCPA
Bidirectional cavopulmonary anastomosis
BT
Blalock Taussig
CA
Common atrium
CHB
Complete heart block
CHD
Congenital heart disease
CHF
Congestive heart failure
CoA
Coarctation of aorta
CS
Coronary sinus
cTGA/L-TGA
Congenitally corrected transposition of the great arteries/levo-transposition of the great arteries
DOLV
Double outlet left ventricle
DORV
Double outlet right ventricle
DTGA
Dextro-transposition of great arteries
ECG
Electrocardiogram
ECD
Endocardial cushion defect
EDM
Early diastolic murmur
ESM
Ejection systolic murmur
FiO2
Fractional inspired oxygen
HCM
Hypertrophic cardiomyopathy
HLHS
Hypoplastic left heart syndrome
ICU
Intensive care unit
IVC
Inferior vena cava
JVP
Jugular venous pulse
LA
Left atrium
LAD
Left anterior descending (artery)
LAE
Left atrial enlargement
LBBB
Left bundle branch block
LCA
Left coronary artery
LCX
Left circumflex (artery)
LPA
Left pulmonary artery
LPSB
Left parasternal border
LSB
Left sternal border
LSVC
Left superior vena cava
LV
Left ventricle
LVF
Left ventricular failure
LVH
Left ventricular hypertrophy
LVIO
Left ventricular inflow obstruction
LVOT
Left ventricular outflow tract
M1
Mitral component of first heart sound
MPA
Main pulmonary artery
MR
Mitral regurgitation
MRI
Magnetic resonance imaging
MS
Mitral stenosis
MVP
Mitral valve prolapse
MVR
Mitral valve replacement
OP
Ostium primum
OS
Ostium secundum
P2
Pulmonary component of second heart sound
PA
Pulmonary artery
PA
Pulmonary atresia
PAH
Pulmonary arterial hypertension
PAPVC
Partial anomalous pulmonary venous communication
PDA
Patent ductus arteriosus
PFO
Patent foramen ovale
PND
Paroxysmal nocturnal dyspnea
PPH
Primary pulmonary hypertension (arterial)
PR
Pulmonary regurgitation
PS
Pulmonary stenosis
PSM
Pansystolic murmur
PV
Pulmonary vein
PVH
Pulmonary venous hypertension
RA
Right atrium
RAE
Right atrial enlargement
RBBB
Right bundle branch block
RCA
Right coronary artery
RHD
Rheumatic heart disease
RPA
Right pulmonary artery
RSOV
Rupture of sinus of Valsalva aneurysm
RV
Right ventricle
RVF
Right ventricular failure
RVH
Right ventricular hypertrophy
RVOT
Right ventricular outflow tract
S1
First heart sound
S2
Second heart sound
S3
Third heart sound
S4
Fourth heart sound
SV
Single ventricle
SVC
Superior vena cava
SVT
Supraventricular tachycardia
T1
Tricuspid component of first heart sound
TA
Tricuspid atresia
TAPVC
Total anomalous pulmonary venous communication
TEE
Transesophageal echocardiography
TGA/dTGA
Complete transposition of great arteries
TS
Tricuspid stenosis
TTE
Transthoracic echocardiography
TR
Tricuspid regurgitation
VSD
Ventricular septal defect
WPW syndrome
Wolff–Parkinson–White syndrome