Recent Advances in Pediatrics (Special Volume 16: Pediatric Cardiology) Suraj Gupte
INDEX
A
Amplatzer ASD device 210
Antiarrhythmic agents 330
Antiarrhythmic drugs 332
Anticoagulants 374
Arterial switch operation 142
Atrial septal defects 147
clinical associations 148
clinical profile 148
management
surgical closure 151
transcatheter closure 153
non-invasive investigations 148
cardiac catheterization 150
chest skiagram 148
echocardiography 149
electrocardiogram 148
B
Baloon atrial septostomy 142
Bradyarrhythmia 322
C
Cardiac failure 50, 284
clinical features 50
diagnosis 52
echocardiographic findings 53
electrocardiographic findings 53
radiologic findings 52
etiologic assessment 53
etiology 50, 51
treatment 54
congested state 55
digoxin 55
diuretics 55
general measures 58
nitroglycerin 56
precipitating events 54
sodium nitroprusside 56
vasodilators 56
Cardiac glycoside 41
Cardiac tamponade 229, 231
Cardiomyopathy 1, 21
classification 2
Coagulant factors 373
Coarctation of the aorta 169
anatomical considerations 170
clinical presentation 172
embryology 171
hemodynamics 171
incidence 169
investigations 174
angiography 176
cardiac catheterization 176
echocardiography 175
electrocardiographic features 174
magnetic resonance imaging 174
radiologic features 174
management
medical 177
percutaneous balloon angioplasty 181
surgical 179
physical examination 173
prognosis 181
Congenital cardiac defects 147
Congenital heart disease 105
accuracy of clinical evaluation 109
adult with repaired CHD 122
antenatal diagnosis 120
clinical clues 112
early diagnosis 105
adolescents 109
newborns and young infants 106
genetic aspects 119
incidence and recurrence risk 105
non-invasive studies
chest X-ray 113
ECG 113
echocardiography 117
neonatal ECG 115
pulse oximetry 116
participation in athletic sports 122
preoperative evaluation 118
Congestive heart failure 25
acute failure in children 46
clinical features 29
diagnosis 32
arterial blood gases 34
imaging studies 33
invasive studies 35
lab studies 32
etiology 30
etiopathogenesis 25
staging 36
treatment 36
control of CHF state 39
correction of the underlying cause 37
general measures 37
newer therapies 43
precipitating/aggravating cause 37
prevention of deterioration of cardiac function 38
Constrictive pericarditis 232, 235
clinical features 237
effusive constrictive 241
etiology 236
investigations 238
cardiac catheterization 240
chest X-ray 238
computerized tomography 239
echocardiography 238
magnetic resonance imaging 239
pericardial and endocardial muscle biopsy 240
pathophysiology 236
treatment
medical 240
surgical 241
Cyanosis 81
approach 90
chest X-ray 91
ECG 93
hyperoxia test 93
imaging modalities 92
pulse oximetry 91
use of prostaglandin 95
basic considerations 83
clinical clues 84
diagnosis 99
cardiac catheterization 100
echocardiography 99
differential diagnosis 85
differential cyanosis 89
reverse differential cyanosis 90
difficult situations 97
balancing the circulations 99
diagnostic challenges 97
instituting PGE1 infusion 97
etiology 84
management 100
principles 101
pathophysiology 82
D
Device closure of congenital cardiac defects 209
Amplatzer ASD device 210
complications 214
device related procedures 216
history of device closure 209
indications for closure 212
outcomes 213
surgical ASD closure 209
DiGeorge syndrome 355
clinical findings 356
diagnosis 360
immune system 359
major candidate gene 355
pathophysiology 356
treatment 360
Dilated cardiomyopathy 1
clinical features 5
etiopathogenesis
familial 3
viral 4
investigations 6
angiography 7
cardiac catheterization 7
echocardiography 6
electrocardiogram 6
X-ray 6
management 8
antiarrhythmic agents 11
anticoagulants 10
beta blockers 10
cardiac transplantation 12
diuretics 9
growth hormone 11
immunomodulation 11
immunosuppression 11
intravenous inotropic drugs 8
positive inotropic agents 8
vasodilators 9
pathophysiology 4
prevalence 1
Down syndrome 109
E
Endocardial fibroelastosis 12
F
Failure of impulse propagation 323
Fibrinolysis 373, 376
Frank-Starling mechanism 26
G
Genetic counseling 361
H
Heart block
first degree 323
second degree 323
third degree 324
Hemostasis 364
Hydralazine 41
Hypertension in children 290
blood pressure measurement 291
clinical manifestations 296
etiopathogenesis 292
pathophysiology 292
essential 296
primary 292
secondary 295
recommendations for athletic participa tion and exercise testing 301
treatment 298
Hypertrophic cardiomyopathy 13
clinical features 14
investigations 15
management
medical therapy 17
non-surgical therapy 17
surgical therapy 18
pathophysiology 14
prevalence 13
I
Infective endocarditis 244
clinical features 248
diagnosis 251
activity 272
antimicrobial therapy 256, 273
blood cultures 252
culture-negative endocarditis 269
diet 271
fungal endocarditis 268
imaging studies 253
inpatient care 272
prosthetic valve and prosthetic material endocarditis 268
prosthetic valve endocarditis 262
radionuclide scans 255
role of anticoagulation 273
role of surgery 270
serologic tests 253
epidemiology 245
pathogenesis 246
pathology 247
prevention 273
prognosis 275
Inherited neonatal thrombosis 373
diagnosis and evaluation 379
risk factors 377
treatment 382
blood component therapy 387
pharmacotherapy 382
surgical thrombectomy 387
thrombolytic therapy 386
Inherited prothrombotic disorders 366
Intraaortic balloon pumping 44
contraindications 45
indications 45
M
Myocarditis 278
clinical manifestations 281
complication 286
etiology 278, 279
investigations 282
pathophysiology 280
treatment 284
N
Neonatal coagulation 373
Nonbacterial thrombotic endocarditis 247
P
Patent ductus arteriosus 161, 216
acyanotic obstructive cardiac lesions 165
cardiac catheterization 162
clinical features 161
echocardiogram 162
management 163
ductus in premature infants 164
surgical closure 163
transcatheter closure of ductus 165
noninvasive evaluation 162
physical findings 161
Pericardial effusion 221
clinical manifestations 223
diagnosis 225
computerised tomography 227
echocardiogram 226
electrocardiography 225
MRI 227
radiographics 226
differential diagnosis 228
etiology 222
management 229
pathophysiology 221
Persistent pulmonary hypertension 60
clinical features 62
conditions associated 61
diagnosis 63
echocardiography 64
hyperventilation tests 64
preductal and postductal PaO2 differences 63
management 65
conventional ventilator therapy 65
extracorporeal membrane oxygen-ation 71
general principles 65
high frequency ventilation 66
inhaled vasodilator therapy 69
inotropic therapy 67
liquid ventilation 72
systemic vasodilator therapy 68
pathogenesis 60
prognosis 72
Pulmonary hypertension 87
associated conditions 188
clinical classification 187
clinical features 193
diagnosis 195
epidemiology 187
pathogenesis 189
prognosis 203
World Health Organization Classification 192
treatment 195
additional pharmacotherapy 201
anticoagulation 196
atrial septostomy 201
calcium channel blockade 197
elastase inhibitors 200
endothelin receptor antagonists 199
gene therapy 200
inhaled prostacyclin analogue 198
nitric oxide 199
oral prostacyclin analogue 198
phosphodiesterase inhibitors 200
prostacyclin analogues 198
prostaglandins 197
subcutaneous prostacyclin analogue 198
transplantation 202
R
Restrictive cardiomyopathy 18
clinical features 19
investigations 19
angiography 20
cardiac catheterization 20
echocardiography 19
management 21
prognosis 21
S
Shock 304
etiology 305
etiopathogenesis 304
management 311
assisted ventilation 313
inotropes 313
role of corticosteroids 315
supportive therapy 315
systemic vasodilators 314
pathogenesis 307
physiologic classification 306
recognition and monitoring 309
T
Tachyarrhythmia 324
accelerated junctional tachycardia 326
atrial flutter 326
chaotic or multifocal atrial tachycardia 325
ectopic 325
supraventricular 324
ventricular 327
Takayasu’s arteritis 338
assessment of disease activity 348
diagnosis 342
angiography 345
aortography 346
laboratory studies 344
radiology 344
sonographic techniques 347
etiology and pathogenesis 339
histopathology 340
mortality 348
signs and symptoms 340
cardiac involvement 341
cutaneous manifestations 342
neurologic findings 342
renal involvement 342
treatment 347
Tetralogy of Fallot 127
clinical manifestations 129
complications
hypoxic spells 131
components 127
examination 129
laboratory findings 129
ECG findings 130
echocardiographic findings 130
roentogenographic findings 129
pathophysiology 127
treatment 132
medical 132
postoperative follow up 134
surgical 132
Transposition of great vessels 135
approach to diagnosis 136
cardiac catheterization 141
chest radiograph 140
echocardiography 140
electrocardiogram 140
examination 139
history 138
hyperoxia test 139
pulse oximetry 139
resuscitation 138
management
medical 141
surgical 142
pathophysiology 135
Tubular hypoplasia 170
V
Venous thromboembolism 364
clinical presentations 367
diagnosis
imaging studies 367
etiology 365
incidence 365
treatment 367
low molecular weight heparin 368
oral anticoagulation therapy 369
standard heparin 368
thrombolytic therapy 369
Ventricular septal defects 155, 217
catheterization and angiography 158
clinical presentation 156
echocardiogram 157
electrocardiogram 157
management 159
device closure 160
natural history 158
noninvasive evaluation 157
×
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1RECENT ADVANCES IN PEDIATRICS
Special Volume 16
Pediatric Cardiology
2
3RECENT ADVANCES IN PEDIATRICS
Special Volume 16
Pediatric Cardiology
Editor Suraj Gupte MD, FIAP Director Children's Health Center Professor and Head (Ex) Pediatric Unit Government Medical College and Associated Hospitals Jammu- 180 001 Jammu and Kashmir, India E-mail:surajgupte@rediffmail.com
Honorary Director Pediatric Education Network
Editor The Short Textbook of Pediatrics, Textbooks of Pediatric Emergencies, Neonatal Emergencies and Pediatric Nutrition, Pediatric Yearbook Series, Pediatric Today Monograph Series, Newer Horizons in Tropical Pediatrics, Towards MRCPCH Part II (Theory) Examination
Author Differential Diagnosis in Pediatrics, Instructive Case Studies in Pediatrics, Pediatric Drug Directory, Infant Feeding, Speaking of Child Care: Everything You Wanted to Know, The Baby Book: The Parents’ Guide from Birth to Infancy
Co-editor Asian Journal of Maternity and Child Health (Manila, Philippines)
Section Editor Pediatrics Today (New Delhi, India)
Editorial Adviser Asian Journal of Pediatric Practice (New Delhi, India)
Editorial Advisory Board Member/Reviewer Indian Journal of Pediatrics (New Delhi, India), Indian Pediatrics, (New Delhi, India)
Synopsis: A Current Survey of World Literature in Pediatrics (Detroit, USA), Indian Journal of Pediatric Gastroenterology, Hepatology and Nutrition (Jaipur, India), Maternal and Child Nutrition (Preston, UK)
Examiner National Board of Examinations (NBE) for DNB, New Delhi, All India Institute of Medical Sciences (AIIMS), New Delhi, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sher-i-Kashmir Institute of Medical Sciences (SKIMS) and several universities
Pediatric Faculty Selection Expert All India Institute of Medical Sciences, New Delhi Punjab Public Service Commission
4Published by
Jitendar P Vij
Jaypee Brothers Medical Publishers (P) Ltd
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Branches
Recent Advances in Pediatrics (Special Volume 16: Pediatric Cardiology)
© 2006, Shamma-Bakshi Gupte, Executive Editor
All rights reserved. No part of this publication 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 : 2006
9788180617232
Typeset at JPBMP typesetting unit
Printed at Gopsons
5
To
The fond memory of our late Parents
whose inspiration, blessings and
moral support continue to contribute
a great deal to our academic
endeavors and
Everybody contributing to child health
and welfare for a better future
6
7Contributors 11Preface
With the availability of sophisticated investigative and therapeutic know-how and technological advances in increasing number of centers, pediatric cardiology in India and other developing countries is beginning to gear up for new challenges. As a result, the need to sensitize and involve the pediatricians (both in institutions and field practice) to these advances in relation to specific cardiac disease entities over and above the general principles of congenital and acquired heart disease is being increasingly felt.
In the light of this background, the 16th Special volume of the noted international series, Recent Advances in Pediatrics, first launched in 1991 and now concurrently in the 16th Annual and 17th special volumes (RAP 16 and RAP Special Vol 17: Adolescence, respectively), offers deliberations on key topical issues in the field of pediatric cardiology.
The 23 state-of-the-art chapters covered by as many as 31 experts drawn from India and abroad attempt to review the depth and breadth of knowledge in the field of pediatric cardiology with special reference to its applicability in the Indian subcontinent. The objective is to provide authentic knowledge and infomation for the general pediatricians in particular so that they become well equipped to provide good care to their cardiac subjects and take appropriate decisions for referral to the superspecialist in certain unavoidable situations.
Each and every chapter is remarkable by its excellence, providing a stimulating and, not infrequently, even provocative, matured and informed update. Understandably, such an approach is befitting when the goal is not only to inform but also to motivate discussion, interaction, research and innovation.
In keeping with the professed policy of the series, multidisciplinary approach with an eye on future continues to occupy pride of place in this volume too.
We appreciate the distinguished role of the eminent pediatric cardiologist, Dr Anita Saxena, Professor of Cardiology, All India Institute of Medical Sciences, as the Guest Editor as well as contributor of an excellent chapter for this special volume.
We wish to record our indebtedness to our distinguished contributors for providing excellent state-of-the-art chapters in various aspects of pediatric cardiology and related topics. They were also gracious enough to repose confidence in our editorship. Without their understanding and cooperation, this volume wouldn't have seen the light of the day.
Thanks are also due to the Advisory Editorial Board for providing inputs and advice in various editorial matters and peer reviewers for 12not only providing us the benefit of their critical expertise but also for the promptness in responding to our requests for speedy action.
Our daughter, Novy, a final year MBBS student, Government Medical College, Jammu, and son, Manu, an 8th semester student of Bachelor of Engineering, MBS College of Engineering and Technology, Jammu, voluntarily provided all sorts of assistance in taking the project to its logical conclusion.
M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi and their dedicated staff deserve our special gratitudes for the speedy work in completing this project and its skillful production qualities.
Suraj Gupte
Academic Editor
Shamma-Bakshi Gupte
Executive Editor
13Guest Editor's Introductory Note
It is my proud privilege and honor to serve as the Guest Editor for this Special Volume on Pediatric Cardiology of the noted series, Recent Advances in Pediatrics edited by Prof Suraj Gupte.
The subspecialty of pediatric cardiology has witnessed tremendous growth and expansion over the last six decades. Admittedly, it is not possible to cover details of each disease. The main thrust of this book is, therefore, to educate the practising pediatricians regarding the general issues of congenital and acquired heart diseases as relevant in our country and to introduce the advances made recently in this subspecialty.
In this book, congenital heart diseases have been covered in seven chapters, starting with the approach to a newborn with cyanosis to a common clinical scenario faced by the pediatrician. Some of the common congenital heart diseases like tetralogy of Fallot, transposition of great vessels and coarctation of aorta have been discussed in detail.
A large part of the book is dedicated to acquired cardiac disorders in children. The topics covered include myocarditis, cardiomyopathy, infective endocarditis, pericarditis, Takayasu's arteritis, thrombo-embolism, etc. Hypertension in children, a common but often unrecognized problem has also been well discussed in the Chapter on “Childhood Hypertension.” Similarly, another important topic very nicely covered is that of “Cardiac Arrhythmias.” Common rhythm disorders as seen in children are well discussed in details and their management outlined.
The book also deals with common cardiac emergencies like shock and congestive heart failure. There is a fine article covering current thoughts in management of persistent pulmonary hypertension of the newborn.
Although recent advances have been outlined in each chapter, a separate chapter is devoted to the transcatheter treatment for closure of septal defects like atrial septal defect, ventricular septal defect, patent ductus arteriosus, etc. This is a very exciting field for interventional cardiologist as well as the patient, as the defects are closed without resorting to surgery.
In summary, the Recent Advances in Pediatrics (Special Vol 16: Pediatric Cardiology) incorporates topics which have an immense educational value for a practising pediatrician. Hopefully, these discussions would provide a fund of information for better cardiac care of the “little patients” and the well-conceived and well-executed book shall prove a considerable value for the target readership.
Anita Saxena
Professor of Cardiology
All India Institute of Medical Sciences (AIIMS)
New Delhi, India