Editor M Satpathy MD DM
Former Professor of Cardiology Haripur Road, Dolamundai
Cuttack (Orissa) India
Co-editor BR Mishra MD DM
Cardiologist, EKO Imaging Institute Mangalabag,
Cuttack (Orissa) India
Jitendar P Vij
Jaypee Brothers Medical Publishers (P) Ltd
B-3 EMCA House, 23/23B Ansari Road, Daryaganj,New Delhi 110 002, India
Phones: +91-11-23272143, +91-11-23272703, +91-11-23282021, +91-11-23245672
Rel: +91-11-32558559, Fax: +91-11-23276490, +91-11-23245683
e-mail: jaypee@jaypeebrothers.com, Visit our website: www.jaypeebrothers.com
Branches
- 2/B, Akruti Society, Jodhpur Gam Road SatelliteAhmedabad 380 015, Phones: +91-79-26926233, Rel: +91-79-32988717, Fax: +91-79-26927094e-mail: ahmedabad@jaypeebrothers.com
- 202 Batavia Chambers, 8 Kumara Krupa Road, Kumara Park East,Bengaluru 560 001 Phones: +91-80-22285971, +91-80-22382956, Rel: +91-80-32714073Fax: +91-80-22281761, e-mail: bangalore@jaypeebrothers.com
- 282 IIIrd Floor, Khaleel Shirazi Estate, Fountain Plaza, Pantheon RoadChennai 600 008, Phones: +91-44-28193265, +91-44-28194897, Rel: +91-44-32972089Fax: +91-44-28193231, e-mail: chennai@jaypeebrothers.com
- 4-2-1067/1-3, 1st Floor, Balaji Building, Ramkote Cross RoadHyderabad 500 095, Phones: +91-40-66610020, +91-40-24758498, Rel:+91-40-32940929,Fax:+91-40-24758499, e-mail: hyderabad@jaypeebrothers.com
- No. 41/3098, B & B1, Kuruvi Building, St. Vincent RoadKochi 682 018, Kerala, Phones: +91-484-4036109, +91-484-2395739, +91-484-2395740e-mail: kochi@jaypeebrothers.com
- 1-A Indian Mirror Street, Wellington SquareKolkata 700 013, Phones: +91-33-22651926, +91-33-22276404, +91-33-22276415,Rel: +91-33-32901926, Fax: +91-33-22656075,e-mail: kolkata@jaypeebrothers.com
- 106 Amit Industrial Estate, 61 Dr SS Rao Road, Near MGM Hospital, ParelMumbai 400 012, Phones: +91-22-24124863, +91-22-24104532, Rel: +91-22-32926896Fax: +91-22-24160828, e-mail: mumbai@jaypeebrothers.com
- “KAMALPUSHPA” 38, Reshimbag, Opp. Mohota Science College, Umred RoadNagpur 440 009 (MS), Phones: Rel: +91-712-3245220, Fax: +91-712-2704275e-mail: nagpur@jaypeebrothers.com
Clinical Diagnosis of Congenital Heart Disease
© 2008, M Satpathy
All rights reserved. No part of this publication and should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editor and the publisher.
First Edition: 2008
9788184481617
Typeset at JPBMP typesetting unit
Printed at Gopson Papers Ltd, A-14, Section 60, Noida
7Contributors
- AK Bhattacharyya md dm
- Professor and Head of Cardiology
- Guwahati Medical College, Guwahati
- AK Samal md dm
- Department of Cardiology, VSS Medical College
- Burla
- AN Patnaik md dm dnb
- Associate Professor of Cardiology
- Nizam's Institute of Medical Sciences, Hyderabad
- Anita Saxena md dm
- Professor of Cardiology
- All India Institute of Medical Sciences, New Delhi
- B Dalvi md dm facc (USA)
- Consultant Cardiologist, Glenmark Cardiac Centre
- Mumbai
- Balu Vaidyanathan md dm
- Department of Pediatric Cardiology
- Amrita Institute of Medical Sciences and Research Centre Kochi
- BR Mishra md dm
- Cardiologist, EKO Imaging Institute, Mangalabag
- Cuttack (Orissa)
- BRJ Kannan md dm
- Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi
- C Mahadevan
- Department of Cardiology, Ramaiah Heart Centre
- MS Ramaiah Medical College, Bengaluru
- CD Gupta md dm
- Former Professor and Head of Cardiology
- Jammu Medical College, Jammu
- CG Bahuleyan md dm
- Former Professor and Head of Cardiology
- Trivendrum Medical College, Trivendrum
- G Rajesh
- Department of Cardiology, Kottayam Medical College
- Kottayam
- HN Mishra md dm
- Professor of Cardiology, SCB Medical College
- Cuttack
- IB Vijayalakshmi md dm ficc ficp
- Head of Pediatric Cardiology
- Jayadeva Institute of Cardiology
- Bengaluru
- J Yusuf md dm
- Department of Cardiology, GB Pant Hospital
- New Delhi
- K Sharada md dm
- Consultant Cardiologist, Care Hospitals
- Hyderabad
- M Behera md dm
- Professor and Head of Cardiology
- SCB Medical College, Cuttack
- M Jayarajah md dm
- Professor of Cardiology, Sri Ram Chandra Hospital
- Cardiac Care Centre, Chennai
- M Satpathy md dm
- Former Professor of Cardiology, Haripur Road
- Dolamundai, Cuttack (Orissa)
- ML Kulkarni md fiap fams fcpcc (London)
- Professor and Head of Pediatric
- JJAM Medical College, Davangere, Karnataka
- N Desai md dm facc ficc
- Director, Ramaiah Heart Centre
- MS Ramaiah Medical College, Bengaluru
- N Sudhayakumar md dm
- Professor and Head of Cardiology
- Kottayam Medical College, Kottayam
- Naveen Garg md dm mnams faca (USA)
- Department of Cardiology
- Sanjay Gandhi Postgraduate Institute of Medical Science
- Lucknow
- PC Manoria md dm fccp ficp ficn fica (USA)
- Former Professor and Head Department of Cardiology
- Gandhi Medical College, Bhopal
- PK Dash md dm
- Head, Department of Cardiology
- Sri Sathya Sai Higher Institute of Medical Sciences
- Bengaluru
- PK Pati md dm
- Department of Cardiology, CMC, Vellore
- PR Gupta md dm
- Professor and Head, Department of Cardiology
- Institute of Medical Sciences
- Banaras Hindu University, Varanasi
- R Juneja md dm
- Department of Cardiology
- All India Institute of Medical Sciences, New Delhi
- R Kumar
- Department of Cardiology
- Ramaiah Heart Centre
- MS Ramaiah Medical College
- Bengaluru
- Rohit Manojkumar md dm
- Department of Cardiology
- PGI, Chandigarh
- S Guha Dip Card md dm
- Associate Professor, Department of Cardiology
- Kolkata Medical College, Kolkata
- S Shah
- Department of Cardiology
- All India Institute of Medical Sciences, New Delhi
- S Venkatesh dnb dch
- Lecturer, Division of Pediatric Cardiology
- BJ Wadia Hospital for Children, Mumbai
- SK Trivedi md dm
- Professor and Head of Cardiology
- Gandhi Medical College, Bhopal
- SN Rahiman
- Department of Pediatrics
- JJAM Medical College, Davangere, Karnataka
- SN Routray md dm
- Assistant Professor Cardiology
- SCB Medical College, Cuttack
- SR Anil dch md dnb (Ped) dnb (Card)
- Consultant Pediatric Cardiologist
- Apollo Hospital, Hyderabad
- SR Mittal md dm fams ficp fisc (Cardiology)
- Professor and Head of Cardiology
- Jawaharlal Nehru Medical College, Ajmer
- SS Prabhu md dch
- Professor, Division of Pediatric Cardiology
- BJ Wadia Hospital for Children
- Mumbai
- Sunita Maheshwari abp abpc (USA)
- Consultant Pediatric Cardiologist
- Narayana Hrudayalaya, Bengaluru
- UA Kaul md dm
- Former Professor and Director
- Department of Cardiology, GB Pant Hospital
- New Delhi
- V Gouthami md dm dnb
- Department of Cardiology
- Nizam's Institute of Medical Sciences
- Hyderabad
- VS Prakash md dm
- Associate Professor and Chief, Division of Cardiac
- Electrophysiology/Cardiac Pacing
- MS Ramaiah Medical Teaching Hospital
- Bengaluru
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, they 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 need 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.
Being a basically 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 this 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.
11Acknowledgements
I am greatly indebted to my late teachers Dr PL Wahi, Dr PS Bidwai and Dr HN Khattri 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 U Kaul and Dr JP Das. 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 to make this book a reality. My special thanks to Dr BK Mahala of Narayana Hrudayalaya, Bengaluru, Dr Sanat Kumar Sahoo, Cuttack, Dr AN Patnaik of Nizam's Institute of Medical Sciences, Hyderabad and Dr PK Dash, Sri Sathya Sai Institute of Higher Medical Sciences, Bengaluru for their unhesitant help and inspiration from time to time.
I owe a debt to Dr SR Anil, Apollo Hospital, Hyderabad, Dr PK Pati, CMC, Vellore, Dr DB Tonpe, Dr M Behera and Dr SS Mishra, Cuttack for providing their clinical materials for my use. I thank Dr Swarupa Panda for her timely help.
My daughters Sanghamitra and Anuradha and their husbands Bhawani and Debasis deserve special thanks for their constant encouragement for the last three years. I must thank my wife Swachhala and youngest daughter Madhulekha and her husband Rajesh for their full-hearted cooperation and patience all throughout the period of preparing the manuscript.
It is my pleasure to thank our secretarial assistants Mr Jyoti K Nanda and Mr Sanat Nayak, who have taken all pains from beginning to the end to complete this hard task.
My special thanks to my Co-editor Dr BR Mishra 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 wife of Dr Mishra for her whole hearted cooperation.
I am grateful to Shri Jitendar P Vij (CMD), M/s Jaypee Brothers Medical Publishers (P) Ltd., New Delhi. My special thanks to Mr Tarun Duneja (General Manager Publishing), Mr PS Ghuman (Sr Production Manager) and Mrs Samina Khan for their kind cooperation throughout the period. I also thank Mr Sukhdev Prasad (DTP Operator) and Mr Rakesh Verma (Graphic Designer) for their unhesitant help to complete the work in time and finally my sincere thanks to all the associates of the company.
17Abbreviations
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
—Aorto pulmonary 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
CA
—Common atrium
CHB
—Complete heart block
CHD
—Congenital heart disease
CHF
—Congestive heart failure
CoA
—Coarctation of aorta
cTGA/l-TGA
—Congenitally corrected transposition of 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
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
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
SVC
—Superior vena cava
SVT
—Supraventricular tachycardia
T1
—Tricuspid component of first heart sound
TA
—Tricuspid atresia
TAPVC
—Total anomalous pulmonary venous communication
TEE
—Trans esophageal echocardiography
TGA/dTGA
—Complete transposition of great arteries
TS
—Tricuspid stenosis
TTE
—Transthoracic echocardiography
TR
—Tricuspid regurgitation
VSD
—Ventricular septal defect
WPW
—Wolff-Parkinson-White syndrome