Clinical Diagnosis of Congenital Heart Disease M Satpathy, BR Mishra
INDEX
Page numbers followed by f refer to figure, fc refer to flowchart, and t refer to table.
A
Abdomen 45
ultrasonography 143
Abdominal thoracic viscera 52
Abdominal wall defect, midline supraumbilical 48
ABG test 17
Abrachia 82
Abrikossoff's theory 194
Acute respiratory distress syndrome 124, 336
Addison's disease 35
Airway pressure, continuous positive 125, 205
Alagille syndrome 223, 224, 271
Alcohol 16
Alveolar edema 341
Ambrisentan 370
American College of Cardiology 156
American Heart Association 356
American Society of Thoracic Surgeons Classification of VSD 104
Amphetamine 16
Amplatzer duct occluder 135, 166, 384
Amplatzer septal occluder 87, 384
Anacrotic notch 18
Anemia 40
hypochromic microcytic 329
Aneurysm 175, 179
aortic 16
congenital 15, 178, 179
Angina 159, 183, 220, 269
Angiocardiograms 145f
Angiocardiography 60, 85, 110, 134, 153, 158, 159, 161, 192, 197, 210, 225, 232, 268, 282, 286, 292, 355
Angiocytes 3
Angiogram 320
Angiographic classification 123
Angiography 58, 62, 68, 85, 90, 97, 101, 102, 110, 127, 134, 164, 173, 174, 182, 197, 202, 230, 239, 247, 258, 278, 327, 335, 360, 361, 381
Angiotensin-converting enzyme 78
inhibitors 74, 165
Annuloplasty 74
modified 74
Anomalous pulmonary venous connection 47, 100, 334, 347, 356
Anorexia nervosa 35
Anterior diaphragm, deficiency of 48
Aorta 12, 13, 18, 46, 4853, 56, 58, 59, 61, 63, 68, 69, 81, 92, 96, 98, 99, 104, 105, 109, 113, 114, 121, 131, 131f, 133, 133f, 134, 149, 154, 157, 159162, 162f, 164, 167, 170, 177, 182, 183, 186, 189, 195, 200205, 207, 211, 213, 214, 217, 219, 221, 222, 225, 227, 231, 242244, 248, 249, 262, 264, 267, 270, 277, 283, 284, 286, 290, 298, 300, 320, 322f, 323, 324, 332, 333, 340, 349, 365, 368, 377, 381
abdominal 10, 163
aneurysm of 384
ascending 51, 132, 133, 174, 204, 333, 383
descending 9f, 10, 12, 46, 4850, 127, 167, 174, 358, 359, 383
development of 3, 9, 9f
dilatation of 152f
dissection of 16
overriding of 271
severe coarctation of 176, 336
Aortic aneurysms, ruptured 179
Aortic arch 4, 5, 52, 199, 298, 318, 377
anomalies 16, 199, 204, 205
complete interruption of 336, 358
hypoplasia of 317
interruption 40, 305
types of 359f
laterality of 199
left 199, 200, 200f
maldevelopment of 358
normal left 200
obstruction 381
Aortic area 45, 151
Aortic atresia 332
Aortic balloon dilatation 148
Aortic enlargement 38
Aortic isthmus 11
Aortic knuckle 34
Aortic left ventricular tunnel 165
Aortic orifice 271
Aortic regurgitation 17, 26, 36, 66, 110, 113115, 128, 151, 161, 163, 178, 180, 182, 193, 383
congenital 163, 164f
Aortic root 134f
angiocardiogram 183f
dilatation 163
normal 177f
Aortic shadow, absence of 335f
Aortic sinus aneurysm, rupture of 182
Aortic stenosis 38, 111, 148, 150, 388, 389
classification of 150
congenital 40, 148
echocardiogram of 153f
mild 69, 150
moderate 150
severe 16, 32, 151, 151f, 152f
supravalvular 157, 157f, 159, 159f, 187, 224
types of 148f, 154f, 159f
Aortic valve 148, 149, 149f, 155, 159, 165, 182, 206, 228
absent 163
atresia of 331
disease 148, 272
insufficiency 183
lesions 162
normal 149f
repair 115
stenosis 12
Aortico-cameral tunnel 165
Aortico-left ventricular tunnel 163, 165
Aortoarteritis, nonspecific 174
Aortoatrial tunnels 163
Aortogram 110
ascending 134
Aortography 166, 174
Aortopulmonary window 38, 128, 131, 131f, 132
Arch, abnormal 199
Arrhythmias 30, 65, 69, 70, 102, 111, 154, 213, 259, 269, 378
precipitated by 91
sudden cardiac death 193
Arrhythmic episodes 69
Arterial blood gas analysis 355
Arterial fistulam, congenital coronary 189
Arterial partial pressure, normal value of 22
Arterial switch 329
Arteriovenous fistula, congenital pulmonary 353
Artery 185
aberrant
innominate 203
left pulmonary 204
subclavian 9, 201
angiography 343
anomalies, congenital coronary 185
anomalous left coronary 34, 71, 194, 196f, 197f
central pulmonary 42f
common coronary 333
fistula, aortopulmonary 183
hypoplastic nonconfluent pulmonary 294
intercostal 170
left anterior descending 186
reduced number of 364
single great 52
stenosis, innominate 157
Arthritis 86
Asplenia 143
syndrome 40, 99, 237, 240
Atelectasis 282
Athletic appearance 170
Atresia 40, 56
functional pulmonary 207
stenosis 189
Atretic pulmonary valve 289f
perforation of 382
Atretic tricuspid valve 249f
Atria
septation of 3, 6
septum 52
Atrial balloon septostomy 251
Atrial ectopics 86
Atrial enlargement 28
common causes of 31
right 29, 36, 37f, 239f, 342f, 365
Atrial fibrillation 81
Atrial flutter 28, 91, 146
Atrial myxoma 66
Atrial septal defect 7, 16, 26, 28, 37, 38, 64, 74, 79, 82, 84, 8688, 9192, 94, 96, 96f, 98, 102, 106, 127, 136, 151, 209, 212, 237, 243, 244, 254, 271, 281, 289, 312, 324, 332, 333, 339, 344, 349, 353, 365, 368, 369, 377, 380, 384, 388, 389
electrocardiography of 83f, 89f
subcaval 92
types of 79f, 80, 88f
Atrial septal puncture 381
Atrial septation 7
complete failure of 7
Atrial septum 52, 92f, 138
defect of 94f
development of 80f
Atrial switch 329
Atrial systole, pressure during 18
Atrialized right ventricle 254, 255, 258
Atrioventricular block, complete 229
Atrioventricular canal
defect 71, 237
complete 136
right 8
Atrioventricular connection 52, 307, 309, 377
Atrioventricular junction 137, 138f
common 138f, 139f
Atrioventricular nodal rhythms 28
Atrioventricular node branch 186
Atrioventricular septal defect 38, 39, 136, 139f144f, 237, 332, 388
complete 91, 136, 141
partial 88, 141, 145f
unbalanced 140
Atrioventricular septum 104
Atrioventricular valve 52, 138, 138f, 298
common 139f
development of 3, 8, 8f
index 144
regurgitation 21
Augmented vector foot 67
Axillary arterial system 170
Axis deviation, left 28, 30, 250, 251
Azygos vein 6
B
Balloon
aortic
valvotomy 383
valvuloplasty 156, 380
arial septostomy 381, 322, 329, 346, 380, 385
catheter 381f
dilatation 162, 279, 383f
mitral valvuloplasty 99, 380
pulmonary valvuloplasty 206, 215, 380, 382
valvotomy 216
valvuloplasty 56, 154
Barium
esophagography 201
swallow 172
Barlow syndrome 63
Barratt-Boyes classification 100
Bat-wing appearance 41
Bedford sign 35
Beraprost 370
Beta-blockers 296
Biatrial enlargement 57f, 119
Bicuspid aortic valve 148, 149f, 163, 168, 170f, 388
congenital 163
Bidirectional cavopulmonary shunt 251
Bioprosthetic valves 75, 156
Birth, physiological changes after 363
Biventricular hypertrophy 116f, 119, 291
causes of 31f
Blade atrial septostomy 381, 386
Blalock–Taussig procedure, classic 34
Blalock–Taussig shunt 223, 362, 381
classic 172
modified 251
Bland–White–Garland syndrome 194
Bleeding diathesis 368
Blood
flow 25
direction of 12f, 243f, 348f, 351f, 353f
pressure 17, 59, 151, 220, 224, 354
normal 17t
normal 17f
vessels 38
Bone morphogenetic protein 4
Bosentan 370
Brachial blood pressure, measurement of 274
Brachiocephalic veins 6
Bradycardia 17f, 17t, 28
Brain abscess 111, 269, 305, 320, 368
Branch pulmonary artery stenosis 380, 381
Bridging leaflet, superior 138
Bronchiectasis 33
Bronchus
left 35, 200
right 200
B-type natriuretic peptide 125
Bulbar septum 104
Bulboventricular foramen 309, 314
Bulbus cordis 35
Bull and De Leval classification 264
Burchell classification 241
C
Calcium channel blockers 370
Captopril 295
Carbon dioxide 145
Carcinoid syndrome 206, 214
Cardiac anomalies 35, 74, 165
congenital 108
Cardiac apex, localization of 19
Cardiac catheterization 58, 68, 73, 85, 90, 94, 97, 101, 102, 110, 114, 119, 127, 134, 145, 153, 158, 161, 164, 173, 176, 182, 192, 197, 210, 218, 225, 239, 247, 258, 268, 282, 286, 292, 301, 301, 304, 312, 319, 343, 355, 360, 366
Cardiac chambers 189
conventionally position of 54
morphology of 52f
Cardiac computed tomography 85, 95, 239, 258, 268
Cardiac jelly 3
Cardiac lesions 214
major congenital 389f
Cardiac magnetic resonance imaging 85, 109, 212, 258, 268
Cardiac malposition 46, 50, 53
diagnosis of 45
Cardiac multislice computed tomography, techniques of 212
Cardiac silhouette 152, 327f
Cardiac tumors 160
Cardiac ultrasound, role of 375
Cardinal vein 4, 5, 347
left 6f
posterior 6
Cardiomyopathy 32
dilated 36
hypertrophic 64, 74
Cardiovascular system 250
examination of 18
Carina, splaying of 35f
Carotid
arterial pulse, maximum systolic 150
pulse tracing 18, 151f
Carvallo's sign 83, 97
Cat's eye syndrome 271, 339
CATCH 22 syndrome 9
Catheterization 60, 110, 210, 230, 278, 282
Celermajer index 257
Cerebral
abscess 356
hemorrhage 174, 269
infarction 157
necrosis 331
stroke, causes of 69
Cerebrovascular accident 70, 269, 320
Cervical aortic arch 203
Char syndrome 121
CHARGE syndrome 315, 321
Chest
abnormal X-ray of 24
discomfort 63
infection 25
repeated 128
pain 180
radiograph 22
X-ray 15, 46, 67, 133, 166, 176, 214, 219, 261, 269, 300
Cholecystitis 49
Chordae tendineae 55
Chordal rupture 72
Circumflex aortic arch 204
Cleft
mitral valve 305
palate 315, 331
Clinical pressure gradient 383
Coarctation of aorta 16, 32, 35, 40, 56, 149, 150, 159, 167, 167f, 168, 170, 170f, 171, 172f, 173f, 175, 243, 298f, 305, 313, 332, 380, 383, 383f, 388, 389
classification of 168
types of 168, 168f
Coarctation
abdominal 169
common associations of 169
complex 168
extracardiac associations of 169
functional 169
reverse 168
simple 168
Cœur en sabot 37
Collett and Edwards classification 316
Color Doppler 77, 292
echocardiography 122
interrogation 377
Common atrium 91, 237
septum formation in 7f
Compression 35
Computed tomography 101, 109, 134, 192, 197, 199, 301, 302, 335, 343, 366
angiography 360
high resolution 355
scanning 153
Conal septum 104
malalignment of 104
Concordant loop 50
Conduction defects 272, 309
Conduction pathways, abnormality of 255
Conduction system 140
compression of 183
Conduction tissue 254
Congenital aortic sinus aneurysm, unruptured 179
Congenital cardiac lesions 389t
Congenital heart defects
acyanotic 320
cyanotic 320
Congenital heart disease 3, 11, 15, 16, 24, 26f, 27, 28, 33, 38, 4850, 79, 167, 187, 189, 201, 259, 262, 270, 297, 328, 375, 380, 387391
basic embryology of 3
classification of 15, 25
complex 48
prevalence of 387, 388, 388f
prevention of 391
radiological algorithm of 31, 33
sex preponderance of 26t
types of 48, 146, 388
Congestive heart failure 16, 22f, 2325, 56, 65, 72, 78, 86, 91, 102, 105, 115, 123, 141, 151, 166, 169, 180, 188, 193, 195, 208, 218, 219, 224, 238, 244, 249, 251, 255, 266, 269, 272, 281, 291, 301, 317, 325, 334, 341, 356, 360, 365
causes of 23
common causes of 24
differential diagnosis of 251fc
signs of 23
Connective tissue
disease 64
disorders, heritable 64
Conotruncal anomaly 16
Consciousness, transient loss of 273
Continuous wave 126, 153, 173, 210
spectral Doppler 173
Conventional decongestive therapy 166
Cor triatriatum 59, 61, 62
physiologic consequences of 59
subtotal 59
Coronary angiogram
normal
left 186f
right 187f
Coronary angiography 182, 192, 192f
Coronary anomalies 187
classification of 187
major 187
Coronary arterial
anomaly 187, 272
branch 189
fistula, complications of 193
origin 323f
Coronary artery, normal 185
Coronary arteriovenous fistula 128, 183, 189, 189f, 389
echocardiogram of 191f
Coronary artery 52, 158, 163, 165, 166, 185, 186, 189f, 190, 191, 309, 323, 323f, 329, 337
abnormality of 185
anatomy 269
anomalies 188, 189, 272
disease 151
premature 174
left main 186
normal
left 186f
right 186f
proximal right 189f
pulmonary arterial origin of 194
stenosis 159
Coronary atrioventricular fistula 21, 115
Coronary balloon catheter 157
Coronary cameral fistula 263
Coronary fistula 190
Coronary pulmonary shunt 195
Coronary sinus 52, 79, 94, 94f, 185, 190, 238f, 348f, 351
aneurysm 188
anomalies of 188, 351
dilated 55, 59, 349f
unroofed 349f
Coronary stenosis 263
Coronary vascular system 185
Corrective surgery 91
Corrigan's sign 114, 164
Cottage loaf appearance 342
Cough 200
Coxsackie virus 16
Crepitation 296
Crista terminalis 53f
Critical aortic stenosis 155f, 156, 336
Critical pulmonary stenosis 209, 216
Cyanosis 2124, 77, 89, 107, 110, 132, 238, 245, 249, 256, 265, 279, 281, 285, 293, 301, 303, 320, 337, 341
cardiac causes of 22
central 22, 266
mild-to-severe 291
minimal 249
presence of 25
reversed differential 23
Cyanotic spells 23, 273, 279, 285
Cyst, Echinococcus 206
D
Da Costa's syndrome 63
Darling's classification 339
de Musset's sign 114, 164
Dehydration, severe 35
Dextrocardia 16, 33, 49f, 53, 228, 389
isolated 237
Dextroposition 33
Dextroversion 48
Diabetes 16
Diaphoresis 106, 341
Diaphragmatic pericardium 48
Dicrotic notch 18
Dicrotic wave 18
DiGeorge syndrome 9, 16, 271, 288, 321
Digital subtraction angiography 200
Digoxin 115, 135, 175, 198, 240, 261, 295
Dinamap method 17
Disc valve 156
Discrete fibrous membrane 160
Distal aortic segment 168
Distal chamber 59, 60
Diuretics 115, 129, 135, 175, 261
Dizziness 180
Dobutamine 345
Dock's sign 172
Dopamine 345
Doppler
echocardiography, conventional 133
spectrum 68, 126
ultrasound method 17
Double aortic arch 199, 203, 203f
Double chambered right ventricle 213, 215
Double inlet
left ventricle 307
types of 309
right ventricle 307
Double orifice mitral valve 55
Double outlet
left ventricle 289, 298
right ventricle 20, 26, 29, 136, 237, 250, 251, 281, 289, 297, 308, 359, 388
Down's syndrome 16, 33, 80, 103, 141143, 145, 147, 271
Dual sinus origin 323
Duct
aneurysmal dilatation of 129
diameter 123
Ductal arch 377
Ductal closure, mechanism of 122
Ductus arteriosus 1113, 52, 122, 130, 122, 203, 332, 359, 375, 379
aneurysm of 128
aortic attachment of 167
closure of 122
Ductus venosus 13
anomalies of 347, 352
Duroziez's murmur 164
Duroziez's sign 114
Dynamic auscultation 66
Dysphagia 201
lusoria 201
Dysplasia 55
arteriohepatic 187
bronchopulmonary 22
Dysplastic pulmonary valve 213
Dyspnea 23, 106, 151, 159, 163, 200, 220, 274, 275
exertional 114
E
E sign 172
Ebstein's anomaly 16, 22, 28, 31, 35, 37, 66, 71, 228, 229, 253261, 263, 388, 389
echocardiography of 258f
electrocardiogram of 256f
left-sided 260
Ebstein's disease 253
Ebstein's malformation 40, 266
Echocardiogram 93, 153f
Echocardiography 15, 57, 19, 60, 67, 72, 77, 78, 84, 89, 93, 95, 97, 101, 102, 108, 114, 119, 126, 127, 133, 143, 145, 152, 161, 164, 166, 173, 181, 189, 196, 202, 210, 212, 218, 220, 222, 225, 230, 232, 238, 247, 257, 266, 277, 278, 282, 286, 292, 300, 302, 303, 312, 318, 326, 334, 337, 342, 350, 355, 360, 366
Eddies sounds 124
Edema 246
Edward's classification 103, 104, 199, 241, 271, 364
Edward's syndrome 103
Ehlers-Danlos syndrome 64, 224
Eisenmenger's atrial septal defect 366f
Eisenmenger's complex 103, 106, 108, 111, 299, 303, 362, 368f
features of 108
Eisenmenger's disease 362, 364
Eisenmenger's pathology 364
Eisenmenger's physiology 364
Eisenmenger's reaction 362
Eisenmenger's state 364
Eisenmenger's syndrome 17, 35, 40, 91, 102, 135, 142, 218, 219, 306, 310, 318, 349, 362, 365f, 365t, 367f, 369, 369f, 369t, 370
Ejection
click 20, 220
fraction 228
systolic murmur 21, 22, 82, 158, 172, 209, 212, 215, 275, 334
Elastic tissue, abnormal 221
Electrical axis deviation 28, 28f, 28t
Electrocardiogram 27, 57, 60, 61, 67, 72, 93, 114, 116, 152, 158, 161, 256, 378
intracardiac 255
Electrocardiographic pattern 83
Electrocardiography 15, 57, 60, 67, 72, 73, 77, 83, 85, 89, 90, 93, 97, 98, 100, 107, 114, 119, 125, 133, 133f, 143, 152, 164, 172, 180, 191, 195, 203, 209, 210, 212, 215, 218, 220, 222, 225, 229, 238, 239, 246, 266, 268, 276, 282, 285, 291, 299, 301303, 310, 313, 318, 320, 326, 342, 348, 349, 355, 360, 365, 369
surface 246
Elfin facies 16
Ellis–Van Creveld syndrome 16, 237
Embolization 384, 385
Embryologic loop, relation of 50
Embryology 45, 79, 103, 122, 148, 189, 194, 203, 206, 262, 282, 288, 297, 307, 358
Enalapril 112
Endarteritis, infective 128, 130, 174, 356
Endocardial cushion defect 7, 71, 136, 239
partial 88
Endocardial cushion, superior 8
Endocardial fibroelastosis, primary 76, 155, 156
Endocarditis 219
infective 69, 70, 111, 115, 135, 154, 174, 183, 193, 206, 219, 250, 320
Endothelin receptor antagonist 370
Endothelium obliterates, cellular necrosis of 12
Endovascular stent 381
Epigastric branch, superior 170
Esophagus 200202
right-sided indentation of 202
European Surveillance of Congenital Anomalies 391
Eustachian valve 352
Extracorporeal membrane oxygenation 283, 345
F
Facies, abnormal 315, 331
Failure to thrive 341
Falcon and Perloff classification 128
Fatigue 220
Feeding difficulty 16
Femoral artery approach 383
Femoral pulse, loss of 383
Fetal
alcohol syndrome 16
arrhythmia, diagnosis of 378
bradycardia 376
cardiac
anomalies, risk factors for 376
function, assessment of 378
interventions 378
circulation 11, 13, 272
concept of 11
circulatory pathway 11
coarctation 168
echocardiography 134, 319, 375, 376f378f
cross-sectional 257
procedure of 376
role of 375, 392
hemoglobin 12
tachycardia 376
Fetus
echocardiographic
anatomy of 375
physiology of 375
Fibroelastosis, endocardial 16, 71, 76, 78f, 156, 335
Fibrosis, endomyocardial 78
Figure-of-eight appearance 342, 344
Figure-of-three sign 176
Fistula
aortobronchial 183
arteriovenous 21
direct visualization of 355
rupture of 193
Flail mitral valve leaflet 64
Flat P waves 29
Floppy mitral valve syndrome 63
Florid congestive heart failure
signs of 155
symptoms of 155
Flow murmurs 25
Flow theory 358
Flush method 17
Fontan operation, modified 251, 251f
Fontan procedure 251, 336
Fontan–Kreutzer operation, modified 251
Foramen ovale 7, 12, 13, 80
G
Gadolinium
contrast-enhanced magnetic resonance angiography 319
enhancement 153
Gastric air 34f, 50f
Gastrointestinal organs 45
Gasul's defect 116
Gasul's phenomenon 106, 111
Gene therapy 370
Gerbode shunt 85, 118, 119, 119f
German measles 16
Gestational age 16
Gianturco coil 295
Glenn anastomosis, classic 251
Glenn procedure, bidirectional 336
Globular heart 334
Goldenhar syndrome 33
Golf-ball appearance 344
Gore-Tex stents 283
Gore-Tex tube grafts 280, 361
Graham-Steell murmur 107, 218, 365
Great arteries 51f, 52, 53, 281, 359
abnormality during development of 8
complete transposition of 39, 322
congenitally corrected transposition of 29, 40, 54, 71, 227, 228
connections 309
corrected transposition of 28, 188
dextro-transposition of 250
D-transposition of 245, 388
formation of 9
level of 51f, 52f, 213f, 214f, 222f
relationship of 51
transposition of 16, 26, 38, 146, 187, 188, 214, 237, 241, 259, 279, 322f, 328, 329, 330, 339, 363, 380, 389
Great Ormond street echo score 257
Great vessels, transposition of 24
Greenwold classification 264
Growth
factor, transforming 4
retardation, intrauterine 16
Guntheroth's theory 274
H
Hackensellner's theory 194
Hammock mitral valve 55, 56
Hand topology, left 53
Headache 354
Heart
block 183
complete 16, 28, 66
boot-shaped 276
defects, congenital 183, 380
disease 20, 204
acyanotic 39, 40
complex 130
congenital acyanotic 79
congenital cyanotic 22
cyanotic 16, 20, 22, 33, 34, 39, 41, 169
ischemic 64
major congenital 22f
distortion of 35
enlargement of 39
failure 24, 127, 255, 259, 305
acute congestive 76
congenital 82
right 24
lesions, acyanotic 21, 25
normal 138f
position of 48
rate 27, 27f, 27t, 378
sound
fourth 220
second 18, 151, 220
single second 20
third 220
syndrome, hypoplastic right 22, 262, 269, 307, 388
tube
formation of 3, 4
subdivisions of primary 4f
univentricular 36
Heart-hand syndrome 121
Helical computed tomography angiography 343
Hematocrit 246, 276
Hemiplegia 269
Hemitruncus 272, 316
Hemodynamic 56, 59, 65, 77, 80, 89, 113, 132, 150, 169, 179, 194, 208, 218, 237, 243, 254, 272, 309, 339, 348, 354, 364
abnormality 190
classification 123
Hemoglobin 246
maternal 12
Hemolysis 384
Hemoptysis 318
Hemorrhage 356
intracranial 22
subarachnoids 174
Hemothorax 355
Heterotaxia syndrome 307, 339
Heterotaxy, abdominal 237
High-pressure chamber 214
Hilar shadows, typical 41f
Hills sign 114, 164
Holt–Oram syndrome 16, 80, 82, 103, 271, 331
Human immunodeficiency virus 369
Hum-shape appearance 230
Hyaline membrane disease 22
Hybrid procedure 336
Hypercalcemia, infantile 224
Hyperkalemia 30
Hyperoxia test 17, 22
Hyperplasia, intimal 364
Hypertension 97, 157, 171, 174
chronic thromboembolic pulmonary 370
heritable pulmonary arterial 367
idiopathic pulmonary arterial 367, 369
Hypertrophy 108
combined ventricular 31
Hypervascularization 77
Hyperviscosity syndrome 22
Hypocalcemia 315, 331
Hypoglycemia 22
Hypokinesia 77
Hypoplasia 55, 56, 61, 104, 262, 317, 331, 332, 378
hepatic ductal 224
isthmal 168, 168f, 298
Hypoplastic left heart syndrome 26, 31, 129, 149, 155, 156, 187, 188, 307, 331, 333, 334f, 335f, 336, 378, 388
echocardiography of 335f
hemodynamic of 333
Hypoplastic tricuspid annulus, severely 264
Hypotension 166, 295
Hypoventilation 145
Hypoxemia 320
severity of 246
Hypoxia 145, 294, 325
I
Incomplete right bundle branch block 213, 342f
Indomethacin 129
Infant, premature 30, 125
Infections, maternal 16
Inferior vena cava 5, 6, 11, 12, 18, 46, 47f, 4850, 92, 100, 238f, 237, 244, 329, 338, 351f, 352f, 375
anomalies of 347
interruption 350
Infundibular chamber 161
Infundibulum 50, 211
Inlet septum 104
Innominate vein, left 340f
Insomnia 66
Intact ventricular septum 22, 23, 31, 40, 262, 268, 323, 329
Interatrial septum 84, 88, 179
lower part of 90f
Intercostal artery, superior 170
Internal thoracic artery 170, 174
International Society for Nomenclature of Paediatric and Congenital Heart Disease 104
Interventricular septum 8, 10
Intrauterine life 141
Invasive procedure 15
Isomerism, bilateral 48
Ivemark syndrome 237
J
Jacobsen syndrome 150
Jatene operation 329
Jugular venous pressure 17, 57, 72, 97, 142, 151, 180, 195, 208, 212, 218, 245, 274, 285, 354, 365
Juxta arterial defect 104
K
Kabuki syndrome 271
Kartagener's syndrome 16, 33, 49
Kartagener's triad 49
Katz-Wachtel phenomenon 108
Kawasaki disease 188
Kerley B lines 41, 60, 72, 342
Kidney 12
Kirklin classification, modified 100
Kommerell diverticulum 202
Konno operation 162
Kothari's theory 274
L
Lanoxin 129
Larry–Eliot classification 41
Laurence–Moon–Biedl syndrome 16
Leaflet, anterosuperior 139
Lecompte maneuver 329
Left atrial
appendage 34, 59, 238
enlargement 29, 35, 35f, 125
hypertrophy 250
pressure 114
thrombus 61
tumor 61
Left atrioventricular
canal 8
valve 231
Left atrium 12, 18, 38, 48, 53, 55, 58, 63, 65, 68, 69, 73, 74, 78, 81, 88, 90, 92, 93, 96, 98, 104, 105, 113, 114, 131, 149, 159161, 164, 165, 170, 174, 189, 207, 217, 221, 242244, 251, 262, 277, 284, 289, 290, 300, 308, 319, 333, 338, 348, 349, 351, 353, 368, 376, 381, 385
morphologic 50
Left bundle branch block 30, 152
Left circumflex
artery 185, 186
coronary artery 186
Left coronary
cusp 149, 323
sinus 177
Left coronary artery 185, 190, 191, 194, 196f, 197f, 323
anomalous origin of 36, 193
origin of 195f
Left heart
failure 24
syndrome 333f
Left parasternal
border 82, 111, 115, 215, 281
heave 220
Left pulmonary artery 12, 18, 34, 99, 200, 201, 203, 204, 225, 251, 282, 316f, 320f, 359
stenosis of 272
Left ventricle 4, 5, 12, 25, 26, 34, 38, 53, 58, 63, 65, 68, 69, 73, 74, 76, 78, 81, 88, 92, 96, 98, 103, 105, 113, 114, 131, 149, 154, 159162, 164, 167, 170, 174, 182, 207, 217, 221, 231t, 238, 239, 242244, 248, 251, 262, 277, 284, 289, 290, 293, 298, 300, 302, 319, 333, 348, 349, 351, 353, 376, 381, 385
Left ventricular
angiocardiography 110
aspect of septum 140f
dilatation 196
dysfunction 255
ejection time index 150
endocardium 53
enlargement 36
causes of 36
hypertrophy 30, 31f, 31t, 108, 108f, 125, 126f, 152f, 167f, 172, 172f, 196, 209
causes of 31f
severe 72
inflow obstruction 55, 59
outflow tract 90, 137, 140, 148, 162, 228, 331, 359, 376
obstruction 323, 324
pattern 308
Leopard's syndrome 207, 214
Lesions
acyanotic 169
cyanotic 21
duct dependent 23, 129, 130
simple 363
Lev and Saphir's classification 315
Levocardia 33, 54, 389
Ligamentum 168
arteriosum 12, 167, 203
Lithium 16
Liver 12, 227, 322, 340, 351
Lower limb hypertension 168
Lown–Ganong–Levine syndrome 29, 310
Lung buds 338
Lutembacher's syndrome 86, 96, 97f, 98, 98f, 99, 285
iatrogenic 96
M
Macaroni syndrome 223
Main pulmonary artery 37, 57, 81, 89, 97, 98f, 108, 122, 131, 143, 204f, 207, 217, 218, 220222, 225, 230f, 238, 246, 247f, 277, 299, 319f, 320f, 335
dilatation of 221
Mammary artery 173
Marfan's syndrome 16, 64, 71, 223
Maternal disease 16
Mechanical valve 75, 156
Meconium aspiration syndrome 22
Mediasternal tumors 224
Mediastinitis 224
Membranous
atresia 242
septa 160
ventricular septum 5
Membranous septum 104
part of 118f
Mental retardation 274
Mesenteric vein, superior 340
Mesocardia 33, 52
Metabolic acidosis 325
Metabolic disorders 34
Methemoglobinemia 22, 356
Mickey Mouse ears appearance 282
Microcardia 35
Mid-diastolic murmur 80, 83, 125
flow 21, 164
Middle aortic syndrome 158
Midsystolic murmur 69
Miller-Edwards catheter 386
Milliken classification 264
Mirror image branching 201
Mitral annulus 53, 258
Mitral arcade 55, 56
Mitral atresia 307
Mitral incompetence 63
Mitral inflow obstructions 40
Mitral leaflets 58f, 68f
anterior 7, 64
level of 68f
posterior 64
Mitral orifice 56f
Mitral regurgitation 19, 26, 36, 63, 69, 71, 7375, 138, 144f, 155, 161, 192, 195, 237, 381
congenital 71
Mitral ring, supravalvar 40
Mitral stenosis 98
congenital 24, 40, 55, 56f
Mitral valve 5, 55, 57f, 73, 90, 98, 160, 244, 308, 332, 378f, 380
anatomy 64
area 97
disease 32
level 55
percutaneous repair of 381
posterior leaflet of 64f
prolapse 16, 63, 63f, 64, 64f, 65f, 69, 71
frequency of 80
syndrome 67
repair 74
replacement 74
stenosis, congenital 57f
M-mode echocardiography 218
Mongoloid facies 16
Monology of Fallot 276
Mood swings 66
Morgan's theory 273
Mori's classification 132
Mosaic flow pattern 214
Müller's maneuver 354, 355
Müller's sign 164
Multi-detector computed tomography 192
angiography 278
Multiple coarctations 169
Multiple noncardiac anomalies 331
Mumps 16
Mural thrombi formation 78
Murmur 20
asymptomatic 21
continuous 21, 22, 275, 354
diastolic 21, 22, 24, 219
duration of 209f
early diastolic 21, 151
functional systolic 111
innocent 20, 102, 111
symptomatic 21
systolic 24, 325, 354
types of 22
Muscular atresia 242
Muscular septum 104
Muscular ventricular septum 5
Myocardial disease, primary 71, 148
Myocardial infarction 72, 191, 193
pattern 198
Myocardial ischemia 71, 183, 190, 266
Myocardial performance index 378
Myocardial perfusion imaging 197
Myocarditis 16, 74, 78
Myocardium 263
Myxedema 30
Myxoid dysplasia 149
Myxoma 61
Myxomatous degeneration, progressive 69
N
Nadas criteria 24t
Nakata index 294
Nasopharyngeal obstruction, chronic 145
Neill's classification 339
Neimberg classification 242
Neonatal circulation 11, 272
Neonatal cyanosis, differential diagnosis of 250fc
Neural theory 171
Newborn, pulmonary hypertension of 367
Nitric oxide 122, 370
Noncardiac anomalies 331
Noncoronary cusp 113, 149, 323
Noncoronary sinus 177
rupture of congenital 183f
Noonan syndrome 16, 38, 80, 99, 167, 207, 211, 213, 214, 224, 331, 382
Norwood procedure 336
Nuclear myocardial perfusion imaging 197
O
Oblique, left anterior 145
Obstructed right ventricle 380
Obstructed systemic venous baffles, postoperative 380
Obstruction 22, 55
congenital 148, 206
fibromuscular tunnel type 160
intrinsic 187
Obstructive cor triatriatum 40
Obstructive emphysema 35
Occlusive fibroelastosis 187
Omphalomesenteric veins 5, 347
Orthodeoxia platypnea 354
Orthopnea 163, 296
Orthotopic heart transplantation 337
Oscillometric method 17
Osteogenesis imperfecta syndrome 64
Ostial stenosis 189
Ostium
atresia of 187
secundum 7, 79
Ostium primum 7
atrial septal defect 144
defect 71, 88
Outflow tract view 376
Outlet chamber 308
Outlet septum 104
Oxygen saturation 11, 18, 18f, 18t, 239
normal 22
Oxygenated blood 11
Oxygenation 14
P
P wave 28
abnormality of 28
types of 29f
absent 29
multiple 29
Pain, anginal 111, 152
Palliative management 295
Palliative procedures 336
Palliative surgery 305, 313
Palmaz-Schatz stent 226
Palpitation 63, 114
Pansystolic murmurs 21, 107, 119, 301
Papillary muscle 63f, 78f
hypoplasia 55
Parachute mitral valve 55, 56, 169
Paradoxical embolism 111, 250, 356
Paradoxical hypertension 175
Paradoxical splitting 20, 152
Parasternal long-axis 67, 159
Parasternal short-axis 127
Paroxysmal nocturnal dyspnea 275
Partial anomalous pulmonary venous
communication 362
connection 20, 86, 99
drainage 94
Patau syndrome 271
Patent ductus arteriosus 16, 26, 29, 38, 39, 81, 106, 115, 121, 121f, 127f, 132, 135, 150, 180, 182, 191, 194, 209, 217, 223, 243, 264, 265, 267, 272, 281, 290, 298, 323, 329, 333, 335, 339, 358, 362, 365, 369, 377, 380, 384, 384f, 388, 389
classification of 123
Patent foramen ovale 79, 243, 272, 324, 332, 339, 381, 385
Peak systolic transvalvular gradient 152
Pediatric cardiac
interventions 380
services 390f, 390t
Pediatric cardiology 392
Pentalogy of Fallot 276
Percussion wave 18
Percutaneous balloon angioplastic procedure 175
Percutaneous transcatheter intervention 129
Pericardium, congenital absence of left 36
Perimembranous 113
Peripheral pulmonary arterial stenosis 150, 223, 224f
classification of 224
Peripheral pulmonary vessels 126
Peripheral pulse 17, 114, 142, 220, 301
examination of 24
Peripheral smear 143
Peripheral vessel 381
Persistent arrhythmias 67
Persistent ductus venosus 347
Persistent fetal circulation syndrome 22
Persistent truncus arteriosus 315
Phenylketonuria 16
Phenytoin 16
Pink tetralogy 275
Pistol shot sound 164
Placenta 12, 377
detachment of 13
Platypnea-orthodeoxia 82
Plethora 125
Plethoric lung 126f
fields 239f
Poland syndrome 33
Polycythemia 269, 354, 356, 365
primary 356
Polysplenia 47, 339
syndromes 99, 339
Pompe's disease 78
Portal vein 340
Post-atrial septal defect 385f
Postnatal life 105
Post-patent ductus arteriosus 385f
Poststenotic dilatation 38
Post-tricuspid shunt 364, 367
left-to-right 36
Precordial pulsation 220
Predominantly cyanosis 325
Pregnancy and pulmonary stenosis 216
Prenatal diagnostic techniques 390
Pressure
normal 18t
tracing 19f
Primitive atrium 46
Primitive bulbus cordis 178
Primitive heart tube formation 3
Primitive ventricle 35
Prostaglandin 269, 295
infusion 337
Prostanoids 370
Proximal chamber 59, 60
Pseudocoarctation 168
Pseudoxanthoma elasticum 64
Pulmonary angiography, selective 356f
Pulmonary arterial
aneurysm 353
blood 181f
enlargement 37, 37f, 217f
fistula 190
hypertension 29, 31, 41, 57, 77, 81, 97, 103, 141, 220, 224, 305, 341, 365, 369, 389
severe 42f
impulse 325
markings 38, 38f
pressure 109, 364
shadow 292f
Pulmonary arterioles, muscularity of small 364
Pulmonary arteriovenous
aneurysm 353
fistula 41, 353, 355f
multiple 356f
malformations 353
Pulmonary artery 8f, 1113, 18, 34, 36, 51, 51f, 52f, 53, 56, 57f, 59, 61, 72, 81f, 96, 99, 105, 119, 121f, 127f, 131, 131f, 133, 133f, 157, 170, 177, 186, 189, 192f, 193, 194, 195f197f, 205, 211, 211f, 215, 219, 222f, 227, 227f, 231, 239f, 244, 248, 262f, 264, 271, 288, 301, 312f, 320, 322f, 328, 340, 353f, 360, 362, 369, 377, 381
absence of 204
anastomosis 34
anomalies of 204
anomalous origin of 199
banding 147, 283
branch stenosis 16, 21
conduits 380
constriction of 223
development of 8f
hypertension 362
idiopathic dilatation of 38, 220, 221, 221f, 222f
origin of 327f
overriding of 324
part of main 223
sinuses adjacent to 323f
stenosis 159, 214
wedge pressure 364
Pulmonary atresia 2023, 31, 39, 40, 129, 187, 242, 242f, 243f, 245, 262, 262f, 263, 264, 267f, 276, 288, 289f, 290, 292f, 316, 388
echocardiography of 267f, 293f
electrocardiography of 292f
pathways in 264f
Pulmonary atrioventricular fistula 21
Pulmonary blood flow 25, 39, 141, 299, 336
normal 38, 40
Pulmonary capillary pressure 56
Pulmonary circulation 13f, 228
Pulmonary collaterals 288
Pulmonary edema 21, 41, 41f, 97
Pulmonary ejection click 142, 222
Pulmonary flow 39, 243
murmur 20, 341
normal 26
Pulmonary hypertension 26, 37, 39, 57, 59, 64, 86, 111, 125, 128, 135, 232
develops 84
radiological signs of 41
severe 107, 280f
Pulmonary infarction 365
Pulmonary oligemia 40, 247f
Pulmonary plethora 38, 38f
Pulmonary pressure 362
Pulmonary regurgitation 83, 90, 117f, 165, 210f, 211f, 217, 218, 220, 222, 242f, 275, 283, 365
causes of 220f, 220t
Pulmonary stenosis 16, 26, 31, 38, 102, 110, 111, 116, 117f, 154, 210f, 211f, 212, 215, 216, 242, 242f, 243f, 245, 284, 284f, 285, 298, 298f, 300f, 302f304f, 324, 363, 382
absence of 222f
classification of 211f, 211t
echocardiography of 211f
hemodynamic classification of 208
mild 69, 209
severe 129, 142, 209f, 210f, 212f, 212t, 268
single ventricle with 311f
types of 207f
Pulmonary thrombosis 365
Pulmonary trunk 263
idiopathic dilatation of 221
Pulmonary valve 96, 188, 206, 215, 217, 277, 286, 368, 381f
balloon dilatation 381f
stenosis 12, 206
syndrome, absent 281, 282f, 283f
absence of 217
normal 207
thick dysplastic 213f
Pulmonary valvotomy 215
Pulmonary vascular
disease 111, 125, 128, 141, 245, 303, 324, 362
obstructive disease 363
Heath-Edward classification of 364
resistance 19, 19f, 80, 105, 123, 145, 245, 247, 255, 309, 340, 362
Pulmonary vascularity 319f
Pulmonary vasodilators 112
Pulmonary vaso-occlusive disease 56
Pulmonary vein 18, 34, 52, 56, 59, 61f, 99, 238f, 243, 244, 264, 290, 293, 324, 333, 343, 353f
atresia, common 40
complete transposition of 338
left 99f
ostia 143
right upper 92f
selective 343
stenosis 40, 55, 62
Pulmonary veno-occlusive disease 61, 328, 367
Pulmonary venous
blood 339
confluence 338, 344f
flow 211
hypertension 39, 41, 41f, 56, 57f, 196f
obstruction 334, 341
stenosis 62, 381
systolic flow 68
Pulmonic stenosis 329, 388, 389
severe 208
Pulsatile precordium precordial prominence 25
Pulse 59, 151, 170, 224, 354
abnormal 21
arterial 17, 274
low volume 151
oximetry 295, 355, 360
pressure 151
low 151
wave 68
Pure infundibular stenosis 117
Q
Q wave 29
abnormality of 29
implication of 29f
QRS
complex 29
duration 30
QT interval 30
Quadricuspid aortic valve 163
Quadricuspid valve 166
Quincke's sign 114
Quinckes pulse 164
R
Raghib's complex 349, 352
Rashkind procedure 329
Rastelli classification 139
Recurrent coarctation 169
Recurrent paroxysmal supraventricular tachycardia 146
Recurrent respiratory infection 21
Recurrent stenosis 175
Regional wall motion abnormality 197
Regurgitation, congenital pulmonary 217, 219f
Reid syndrome 63
Renal artery stenosis 157
Renin-angiotensin aldosterone system 171
Residual coarctation 169
Respiration 17
Respiratory distress 17, 281, 334
with stridor 205
Respiratory infection 240
repeated 16, 320
Respiratory rate 17, 17t
Respiratory system examination 21
Restrictive cardiomyopathy 66
Retrocristal septum 104
Retroesophageal diverticulum 202
Retrograde aortography 182
Rhabdomyoma 160
Rheumatic fever 86, 165
Rheumatic heart disease 64, 96, 206
Rheumatic mitral
regurgitation 91
stenosis 61, 86
Rheumatic multivalvular heart lesions 259
Rheumatic origin 96f
Rhythm 28, 146
Riemenschneider and Moss classification 118
Right aortic
arch 132, 199, 201, 202, 202f, 203, 278f, 311f
sinus 182f
Right atrium 12, 18, 34, 35f, 46, 5961, 80, 88, 90, 92, 92f, 9698, 103105, 109, 113, 118, 121, 131, 144, 157, 160, 170, 177, 182, 207, 214, 221, 244, 248, 249, 254, 258, 262, 264, 267, 270, 284, 286, 289, 308, 312, 322, 324, 333, 343, 344, 352, 365, 368, 381, 385
morphologic 49, 50
Right bundle branch block 20, 30, 83, 143, 152, 172, 220, 239f, 251, 369
Right coronary
angiography 197f
artery 183, 185187, 190, 194, 195f, 197f, 323
cusp 111, 149, 323
sinus 177
aneurysm of 182f
Right pulmonary
artery 18, 34, 38, 98f, 99, 134, 200, 203, 204, 204f, 205f, 225, 225f, 251f, 277, 316f, 320f, 359, 367f
angiography 101f
veins 99f
Right ventricle 13, 19, 25, 30, 34, 38, 52, 53, 5961, 77, 80, 84, 88, 94, 96, 103, 105, 109, 113, 114, 118, 121, 131, 144, 149, 157, 160, 167, 170, 177, 207, 211, 220, 231t, 238, 243, 244, 248251, 254, 258, 264, 267, 270, 277, 316, 324, 332, 340, 343, 344, 349, 368, 369, 381
dominance 25, 26
enlargement 35f
Right ventricular
angiocardiography 258
angiography 212f
blood 11
enlargement 36, 37f
hypertrophy 30, 31f, 31t, 108, 172, 208, 212, 215, 220, 225, 257, 270272, 276f, 282, 326, 334, 360, 365, 366, 369
severe 29
outflow tract 51, 112, 113, 116, 187, 190, 206, 229, 254, 277, 281, 289
outflow tract
obstruction 183, 271
stenosis, residual 380
thrombus 206
Roentgenography 88
Roger's disease 103, 106, 107
Roger's murmur 107
Rubella 16
Rubella syndrome 16, 150, 167, 207, 224
congenital 16, 223
Rupture, acute 180, 181
S
Sakakibara and Konno classification 178
Sakakibara classification, modified 179
San Luis Valley syndrome 271
Sandergard's coarctation 208
Sano procedure 336
Scimitar sign 101, 101f
Scimitar syndrome 33, 100, 101
Scoliosis 34
Septal defect
aortopulmonary 128, 131
interatrial 284
Septal notch 41
Septal perforation 105
Septal puncture 386
Septum primum 7, 80f
defect 79, 88
Septum secundum 7, 52, 80f
Sex hormone 16
Shock, features of 21
Shone's complex 58, 169, 358
Shunt
lesions 25
left-to-right 25
right-to-left 25
murmurs 25
pre-tricuspid 362, 364
pretricuspid left-to-right 39
reversal of 212t
right-to-left 25, 286f
Sick infants, management of 295
Single atrium 71, 237
Single ventricle 29, 187, 188, 307, 312, 314, 388
Sinoatrial node branch 186
Sinus
bradycardia 28
rhythm 60, 83f, 303, 326f, 378f
rupture of right 177f
Sinus of Valsalva 119, 166, 177, 177f, 178, 188, 272, 278
aneurysms 128, 177, 178, 180, 181, 182f, 183, 184
classification system for 178
congenital 178, 179
dilatation of 181
rupture of 21, 3638, 85, 115, 128, 178180, 181f, 182
types of 178f
congenital aneurysm of 163
rupture 21, 120, 128, 179, 181, 389
aneurysm of 184
Sinus venosus 46, 6f
anomalies of valve of 347, 352
defect 92
type 79, 92
Sinusitis 33
Situs 33, 46
ambiguous 47, 47f
dextrocardia irrespective of 45
Situs inversus 33, 46
dextrocardia 49, 49f, 50f
levocardia 34f, 49, 50f
Situs solitus 33, 46, 50, 228
dextrocardia 48, 48f
levocardia 46f, 48
Skeletal abnormalities 33
Skodaic theory 168
Smith's classification 339
Smith–Lemli–Optiz syndrome 331
Snowman appearance 342
Snowman's cardiac contour 344
Soto's classification, modified 104
Sounds, multiple 124
Spinal artery 170f
Spiral computed tomography 225
Spleen 46, 4850, 227, 322
position of 54
Splenic vein 340
Splitting, normal 20
Squatting 274
Staghorn sign 60
Stenosis 331
infundibular 278f
mild 150
moderate 150
pulmonary
congenital 206
dysplastic 213f
infundibular 213, 214f, 215
isolated 206
severe 150
Stenotic aortic valve 149f
Stenotic lesions, incidence of 26
Stenotic mitral valve 96f
Sternal defect, lower 48
Stomach 227, 322
Straight back syndrome 64, 69
Stridor 200
Stroke 356
Stromal endometriosis 206
Subaortic discrete membrane 161f
Subaortic obstruction 298
Subaortic stenosis 162, 242, 305, 324
types of 160
Subcardinal veins 5
Subclavian artery
left 167f, 170, 174f, 359f
retroesophageal left 202
right 170, 199, 203, 359
Subendocardial injury 76
Subpulmonary stenosis 325, 328
Subvalvular aortic stenosis 56, 160, 160f
Subvalvular apparatus defect 71
Sudden cardiac death 69, 183, 269
Superior vena cava 5, 6, 11, 12, 18, 34, 46, 47, 49, 92, 92f, 93f, 94, 100, 110, 140, 237, 238f, 244, 251f, 272, 338, 348, 349, 349f, 351, 351f, 375
absent normal right-sided 347
anomalies of 347
left 81, 94, 188, 242, 333, 348f350f
obstruction 173
persistent left 55, 237, 347, 348
Supracardinal veins 5
Supravalvular membrane 55, 62
Supravalvular mitral
membrane 58
stenosing ring 58
Supravalvular pulmonary stenosis 223
Supravalvular stenosing ring 55
Supraventricular arrhythmias 67, 69, 86, 91
Supraventricular rhythm 30
Surgery, role of 371
Surgical reconstruction, staged 336
Swiss-Cheese defect 111
Syncopal attack 152
Syncope 159, 180, 220
Systemic blood flow 39
Systemic collateral artery 39, 289
Systemic hypertension 175
Systemic vascular resistance 12, 14, 19, 105, 362
Systemic veins 52
Systemic venous stenosis 381
Systolic click syndrome 63
T
Tachyarrhythmias 255
Tachycardia 17f, 17t, 23, 28, 295, 296
atrial 256
persistent supraventricular 146
Tachypnea 23, 170, 296, 334, 341
Tadalafil 370
Takayasu's arteritis 168, 224
Takeuchi repair 198
Tandon and Edward classification 242
Taussig-Bing anomaly 39, 298f, 299, 301, 306, 328
Teardrop appearance 301
Teeter-Totter appearance 259
Telangiectasia
hemorrhagic 353
hereditary 353
Tetralogy of Fallot 8, 9, 16, 34, 99, 111, 116, 140, 187, 188, 201, 207, 212, 212t, 217, 223, 246, 250, 270, 270f, 271, 276f, 278, 281, 285t, 288, 299, 300f, 310, 348, 369, 378, 388, 389
acyanotic 117, 213
echocardiogram of 277f
echocardiography of 277f
postoperative 380
severe 268
Thoracic aorta 132
Thoracic viscera 48, 49
Thorax 45
Three-dimensional echocardiography 181
Thrombosis, intravascular 250
Thymic hypoplasia 315
Thyrotoxicosis 40
Tidal wave 18
Tissue ribonucleoprotein antigen, soluble 76
Todaro's tendon 243
Toronto classification 123
Total anomalous pulmonary venous connection 20, 21, 26, 31, 35, 38, 59, 128, 143, 146, 328, 338, 339, 341, 343, 347, 352, 362, 388, 389
types of 339f
Trabecular septum 104
Trachea 35, 46, 4850, 200202
indentation of 53f
right-sided 202
Transcatheter aortic valve replacement 381
Transcatheter delivered device closure 135
Transcatheter interventions 380
general principles of 381
Transcatheter occlusion 384
Transesophageal echocardiography 73, 85, 95, 97, 134, 145, 153, 161, 181, 239, 247, 300
Transient ischemic attack 69, 356
Transient systolic murmur 20
Transthoracic echocardiography 72, 93, 161, 303
Transverse aortic arch, hypoplasia of 378
Traube's sign 114
Tricuspid atresia 16, 31, 36, 39, 129, 241, 242f244f, 245, 246f, 247, 249, 268, 294, 307, 328, 388, 389
echocardiogram of 248f
Tricuspid flow murmurs 341
Tricuspid regurgitation 18, 38, 83, 97, 180, 209, 220, 253, 263, 332, 365
Tricuspid valve 5, 53, 90, 182, 214, 227, 228, 244, 253, 262, 263, 265, 308, 380
abnormal 254
anomalies 324
atresia 242
Ebstein's anomaly of 37
Ebstein's malformation of 40
leaflets 253
normal 149f
septal leaflet of 254f, 258f
Trilogy of Fallot 276, 285t, 286f
Trimethadione 16
Trisomy
13 103, 271
18 103, 271
21 16, 103, 271
Trivial stenosis 150
Truncal valve 317
regurgitation 219
Truncus arteriosus 45, 9, 20, 35, 39, 135, 187, 188, 219, 315317, 318f, 320, 321, 328, 388
common 315
communis 315
echocardiography of 319f, 320f
incidence of 315
types of 316f
Tubular hypoplasia 168
Tumors 206
Turner's phenotype 170
Turner's syndrome 16, 99, 150, 167, 331
Twin-to-twin transfusion syndrome 376
Two-dimensional
echocardiogram 57, 191, 300
echocardiography 97, 153, 180
U
Uhl's anomaly 261, 266
Ultrasound, use of intravascular 383
Umbilical artery 12
constriction of 12
Umbilical cord 12
Umbilical vein 46, 12, 13, 347
anomalies of 347
Unicuspid valve 149f
aortic 155f
Upper limb 23
hypertension 171
V
Valsalva aneurysm 183f
acquired sinus of 178
Valsalva maneuver 82
Valve
area, normal 150
replacement 70
Valvotomy, surgical 56
Valvular aortic stenosis 148, 152f, 156, 383
associated lesions of 150
Valvular atresia 242
Valvular pulmonary stenosis 208, 212f, 382
mild 86
severe 287
Valvuloplasty 115, 380
Van Praagh classification 309, 316
Vascular endothelium 13
Vascular obstructions, dilatation of 380
Vascular ring 40, 199
malformations 200
Vascular sling 205
Vasodilators 295
Vein
anomalous right pulmonary 101f
anterior cardinal 5, 6, 6f
bridging 348
common cardinal 46
hepatic 12, 351
innominate 94, 348, 349
stenosis, congenital pulmonary 61, 61f
Velocardiofacial syndrome 9, 271
Venous system
bilateral 4f
development of 5
Ventricular aneurysm 66
Ventricular arrhythmia 171
Ventricular chambers, morphology of main 308
Ventricular fibrillation 154
Ventricular hypertrophy 30
Ventricular origin, arrhythmias of 30
Ventricular premature contraction 74
Ventricular septal crest 139
Ventricular septal defect 16, 26, 29, 38, 39, 56, 90, 103, 105, 113, 114, 116, 118, 127, 132, 136, 146, 150, 154, 168, 178, 192, 193, 194, 201, 207, 217, 223, 228, 242, 242f, 243f, 244, 250, 251, 254, 270, 271, 281, 288, 289f, 290, 290f, 292f, 297, 302, 308, 315, 322, 324, 329, 358, 362, 363, 365, 369, 381, 385, 388, 389
echocardiogram of 368f
isolated 9
large 32, 107, 111, 116, 135, 304f
subaortic 270f, 304f
spontaneous closure of 107
subaortic 277f, 298f, 300f, 303f
types of 105f
Ventricular septum 132, 138
development of 5f
rupture of 105
Ventricular sinus septum 104
Ventricular systole, pressure during 18
Ventricular tachycardia 28, 69, 154
Ventricular topology 52, 53
Ventriculoarterial connection 377
Ventriculoarterial discordance 50
Ventriculocoronary arterial communications 263
Vertebral artery 170f
Vessel thrombosis 364
Vibratory systolic murmur 20
Viscera
abdominal 48, 49
normal position of 46, 54
Visceral position, abnormal 45
Visceral situs 311
Vitelline vein 46, 347
W
Waterfall appearance 311
Water-Hammer pulse 164
Waterston shunt 362
Waterston-Cooley shunt 251
Weakness 220
Wheezing 200
Williams syndrome 16, 158, 207, 223, 224
Williams-Beuren syndrome 157, 167
Windsock appearance 181
Wolff–Parkinson–White syndrome 20, 28, 30, 188, 229, 310, 334
X
X-ray chest 302
Y
Young's theory 274
Z
Z-scores 267
×
Chapter Notes

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1BASIC
  1. Basic Embryology of Congenital Heart Diseases
    ML Kulkarni, SN Rahiman
  2. Fetal and Neonatal Circulation
    BR Mishra, AK Samal
  3. Bedside Diagnosis and Classification of Congenital Heart Diseases
    IB Vijayalakshmi, M Satpathy
  4. Electrocardiogram: Clinically Relevant to Congenital Heart Diseases
    ML Kulkarni, M Satpathy
  5. Radiological Diagnosis of Congenital Heart Diseases
    K Sharada, M Satpathy2

Basic Embryology of Congenital Heart DiseasesCHAPTER 1

ML Kulkarni,
SN Rahiman
It is essential to have a sound knowledge of embryology of the heart. It helps us to understand better the anatomical defects and their implications on circulatory physiology.
Recent years has brightened the scope of understanding the underneath factors leading to congenital heart diseases (CHDs). Microscopy, experimental models and various advanced genetic techniques have revealed more facts in the last few decades.
In this chapter, an attempt has been made to understand some basic principles underlying the development of heart, which have been described under the following subheadings (though it must be realized that such a compartmentation is only for convenience of understanding). It should not overrule the complexity of the subject.
  • Primitive heart tube formation
  • Looping
  • Wedging
  • Septation of the atria
  • Development of the atrioventricular (AV) valves.
  • Development of the aortic and pulmonary trunks.
  • Development of aorta and its branches.
Cardiovascular development is an early requirement of the growing embryo. It occurs from day 18 to 12 weeks of intrauterine life.
 
FROM FERTILIZATION TO PRIMITIVE HEART TUBE FORMATION
The entire cardiovascular system develops from the mesoderm. On 18 days of life, diffusion alone is insufficient to meet the nutritional demands of the rapidly developing embryo. Hence the anterolateral plate of mesoderm present over the yolk sac, the connecting stalk and the body of the embryo differentiates to form angioblasts. Angioblasts form endothelial cell clusters called angiocytes. These spread in a cephalic direction and unite anteriorly to form a horseshoe shaped plexus of small blood vessels.
The two lateral groups then fuse ventrally in the midline to form the primary or the primitive heart tube (Fig. 1.1). It has an inner endocardium and an outer myocardium separated by the cardiac jelly. This primordial myocardium previously formed from the splanchnic mesoderm.
Between the 21st and 24th day, the primitive heart tube subdivides from below upward into right and left horns of sinus venosus (SV), SV proper, primitive atrium (PA), primitive ventricle (PV) and bulbus cordis (BC) (Fig. 1.2).
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FIG. 1.1: Formation of primitive heart tube.
4
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FIG. 1.2: Subdivisions of the primary heart tube.
Into each horn of SV, three bilateral venous systems drain. From lateral to medial they are the cardinal vein (CV), the umbilical vein (UV) and the vitelline vein (VV) (Fig. 1.3).
 
Abnormal Development during the Formation of Heart Tube
Abnormality at this stage almost always results in embryonic death because of the critical nature of the early circulation to the further growth and development.
 
FROM PRIMITIVE HEART TUBE THROUGH LOOPING
The heart is the first organ to break the bilateral symmetry of the early embryo. A differential growth begins, which allows a posterior leftward slow growth and anterior rightward fast growth. This leads to rightward or dextro-looping due to which the future right ventricle (RV) comes to the front and right, of future left ventricle (LV) (Fig. 1.3). Further disproportionate growth occurs resulting in bending of the heart tube at AV junction.
Eventually, the inflow tract and future LV are posterior and to the left. The outflow tract and future RV remain anterior and to the right (Fig. 1.4).
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FIG. 1.3: The three paired bilateral venous systems. The curved arrows point toward the direction of looping.
 
Abnormal Development during Looping
Mirror image reversal of the normal left-right asymmetry is often associated with normal organ development, i.e., dextrocardia with situs inversus totalis infrequently have associated complex defects. Discordance of thoracic and visceral asymmetry universally results in defective organogenesis. For example, Heterotaxy syndromes, which often have complex cardiac anomalies.
Abnormality of looping alone, i.e., levo-looping with normal development of outflow tract results in ventricular inversion, i.e., RV receives from left atrium (LA) and LV receives from right atrium (RA).
Abnormality of looping as well as outflow tract development may lead to corrected transposition of the great vessels. Here exists both ventricular inversion as well as transposition of the great vessels. This is a physiological correction where the systemic venous blood reaches the lungs through RA, morphological LV, and the pulmonary artery. Oxygenated blood returns to the systemic circulation through the LA, morphologic RV, and aorta. Without other defects, hemodynamics is normal.
 
FROM LOOPING TO WEDGING
The PV grows centrifugally from the greater curvature of cardiac loop.5
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FIG. 1.4: Dorsal view of looping. The arrows are showing toward the direction of looping.
At this point, the inflow tract has access only to the LV; whereas RV has access only to the outflow tract. Two very important processes now begin to occur.
  1. Convergence: The inflow constriction (future AV canal) and BC converge more toward each other. Simultaneously, the atria are expanding centrifugally and AV canal septation is taking place.
  2. Wedging: The septated AV canal moves to the right and each orifice lies over its future respective ventricle. The BC shifts leftward such that a part of the outlet sits over the LV and rest sits over the RV.
After convergence and wedging, each ventricle has gained access to both inflow and outflow tracts.
Now, the ventricular septum development begins. The interventricular septum grows upward from the floor of the bulboventricular cavity. The bulbar septum grows downward to partially reach the interventricular septum. Still, a gap remains between the two which is eventually filled by growth of AV cushions (Fig. 1.5).
 
Abnormal Development during Wedging
Abnormalities of looping and convergence can result in inflow malalignment: Double inlet LV or outflow malalignment—double outlet RV.
Only abnormal wedging results in double outlet RV.
Failure of ventricular septation and trabeculations results at single ventricle or hypoplasia of one or both ventricles. Ventricular septal defects originate during this stage.
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FIG. 1.5: Development of the ventricular septum.
 
Development of Venous System
Primitive veins develop as bilaterally symmetrical pairs of:
  • Vitelline veins or omphalomesenteric veins
  • Umbilical veins
  • Cardinal veins
Cardinal veins (CVs) consist of anterior and posterior veins on each side which unite to form common cardinal veins (CCVs) which in turn open to the right and left horns of SV in their respective sides in about 20-somite stage. Anterior cardinal veins (ACVs) drain from the upper part of the body including upper limbs whereas the posterior cardinal veins (PCVs) drain from the lower part of the body and lower limbs. Subsequently, interconnections occur between these veins with prominence of certain parts and regression of other parts. Gradually the right CVs become more prominent. The extra-pericardial part of superior vena cava (SVC) develops from the right ACV and the intra-pericardial part develops from right CCVs. The right sinus horn is incorporated into RA whereas the left CV and VV gradually regress. The left horn is separated from LA. The transverse portion of SV and the proximal left sinus horn become the coronary sinus. The distal left sinus horn and left CCVs ultimately become ligamentous structures (ligament of Marshall). The PCVs receiving blood from lower part of body gradually regress with development of other paired veins like subcardinal and supracardinal veins. Inferior vena cava (IVC) develops from five different embryonic venous channels. They are: (1) posterior cardinals, (2) supra-cardinals, (3) sub-cardinals, (4) communication of right subcardinal with hepatic vein and (5) from part of hepatic vein which develops from right VV. Ultimately the IVC is incorporated into RA.
Persistent left superior vena cava (LSVC) develops when there is failure of obliteration of the left ACV during embryonic6 development. It is a common venous anomaly present in about 0.5% of normal population and associated in about 3–5% of cases of CHD. The LSVC originates from junction of left innominate and left jugular vein and descends in front of aortic arch and subsequently drains to coronary sinus. Very rarely the right SVC may be absent, and then all the venous blood from upper part of body drains to enlarged coronary sinus through the LSVC. When both left and right SVC are present, there is usually a communication between both of them by left innominate vein also known as the bridging vein (Refer to Chapter 36, Fig. 36.1). The caliber of the bridging vein varies from atretic to large size.
 
SEPTATION OF THE ATRIA
Before atrial septation begins, two events must take place:
  1. Transfer of the original three paired bilaterally systemic venous inflow systems to the right side of the heart.
  2. Incorporation of pulmonary venous inflow to LV.
Initially two anastomotic channels develop to transfer blood from left side of the body to right. Brachiocephalic vein in the head, which transfers all blood from left cardinal to right CV and ductus venosus in the body, transfers blood from left UV to the right VV and eventually to SV. Simultaneously, there occurs regression of both distal vitelline systems; both proximal UV, proximal left VV and distal right UV (Figs. 1.6A to D).
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FIGS. 1.6A TO D: (A) Veins draining into sinus venosus as viewed from behind. (B) Anastomosis between anterior cardinal vein. (C) Involution of left cardinal vein and incorporation of sinus venosus into right atrium. (D) Major veins join sinus venosus.
The sinoatrial junction shifts rightward to open into right half of common atrium. This results in regression of left sinus horn, which eventually becomes the coronary sinus.
The pulmonary venous system develops from the left posterior wall of the LA, initially as a single primary pulmonary vein. It then extends toward the developing lung buds. The lung buds are posterior to the heart and derive blood from the splanchnic plexus. The pulmonary vein grows posteriorly and superiorly toward the intrapulmonary venous plexus and once contact occurs, blood flows into LA. The earlier connection with the splanchnic plexus is then lost. The 7LA grows by incorporating the primary pulmonary vein itself back into the posterior wall. Eventually, four pulmonary veins draining into LA are formed.
Now, septation of atria follows (Figs. 1.7A to D). At the end of 4th week of development, a thin crescent-shaped septum goes from the cranial aspect of PA and it represents the first portion of septum primum (SP). The opening between the lower rim of the SP and endocardial cushion (EC) is called the ostium primum (OP). This OP gradually gets obliterated when the growing SP fuses with EC. Before the SP fuses with EC, several perforations develop in its midportion that enlarge and coalesce to form a single large opening the ostium secundum (OS).
Another septum, the septum secundum (SS) then develops along the cranial and posterior wall of the RA. This septum which also has a crescent shaped leading edge extends midway along SP and its crescent shaped lower margin form the foramen ovale (FO). Usually the SS covers the OS. The septum formation is completed in 34 days.
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FIGS. 1.7A TO D: Septum formation in the common atrium.
 
Abnormal Development during Atrial Septation
Complete failure of atrial septation results in development of a common atrium often with both systemic and pulmonary veins appearing bilaterally.
Failure of development of upper connecting brachiocephalic vein results in bilateral SVC or persistence of left SVC draining into the coronary sinus.
Failure of development of SP results in large OS defects.
Failure of fusion of SP with the EC results in OP atrial septal defect (ASD).
Failure of fusion of SS with the SV results in the development of a SV ASD, with or without anomalous pulmonary venous drainage.
Failure of connection between the primitive pulmonary vein and the intrapulmonary venous complex results in various forms of total or partial anomalous pulmonary venous return.
8Defective incorporation of the primitive pulmonary vein into the left atrial wall results in cor triatriatum.
 
DEVELOPMENT OF AV VALVES
Particulates known as adherons accumulate in the cardiac jelly in the region of AV and conotruncal valve formation. These adherons stimulate the endocardial cells to transform cardiac jelly into mesenchyme. This mesenchyme forms dorsal and ventral outpouches. It further differentiates to form fibrous connective tissue which finally forms the valves (Figs. 1.8A and B).
Simultaneously cavitation and septation of ventricular chambers are occurring. Papillary muscles develop and attach to the valve leaflets through chordae tendineae.
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FIGS. 1.8A AND B: Stages of development of atrioventricular valves.
 
Abnormalities during Development of AV Valves
  • Atresia of one of the valves (tricuspid or mitral atresia) or displacement of tricuspid valve (Ebstein's anomaly).
  • Atrioventricular septal defects (AV canal anomaly).
  • Abnormalities of papillary muscular attachment.
 
DEVELOPMENT OF AORTIC AND PULMONARY TRUNKS
The conotruncus, which is the common outflow tract, has to separate into aortic and pulmonary outflow tracts. Swellings appear from right and left sides and not from dorsal and ventral wall. Similar transformation, as in development of AV valves follows and results in the formation of separate valves.
Neural crest cells migrate to the site of formation of outflow septum. The septum develops from distal to proximal and meets the EC below to separate the two semilunar valves. The septum is formed in a spiral manner as shown in the Figures 1.9A to C to provide the final anatomy of the great arteries.
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FIGS. 1.9A TO C: Sequential development of pulmonary artery and aorta. (A) Conotruncus is divided by a spiral septum. (B) Aorta and pulmonary artery develop with a spiral relation. (C) Final shape of aorta (Ao), and pulmonary artery (PA).
 
Abnormalities during Development of Great Arteries
  • Complete absence of neural crest migration results in persistence of truncus arteriosus (TA).
  • Partial absence of neural crest migration produces double outlet RV, tetralogy of Fallot (TOF) and double inlet LV.
  • 9Asymmetrical division of the outflow tract orifice may result in stenosis of one of the valves.
In fact, all cardiac tissues contain neural crest cells except the conducting system.
 
DEVELOPMENT OF AORTA AND ITS BRANCHES
Early in development, there are two paired lateral dorsal aortae which fuse to form the descending aorta. Simultaneously, the pharyngeal arches are growing and each arch has a pair of aortic arch arteries. The first, second, and fifth arches disappear. The third, fourth, and sixth arches form the major components of central cardiovascular system (Figs. 1.10A and B).
 
Abnormal Development during Formation of Great Arteries
Failure of migration of neural crest cells into the aortic arch arteries result in a wide variety of phenotypes which are as follows:
  • DiGeorge syndrome
  • Velocardiofacial syndrome
  • Catch 22 syndrome
  • Interrupted aortic arch
  • Truncus arteriosus
  • Tetralogy of Fallot
  • Isolated ventricular septal defects
  • Aberrant subclavian artery and other subtle arch anomalies are the result of third or fourth aortic arch defects.
  • Interrupted aortic arch results from fourth arch defect.
  • Persistent DA and proximal pulmonary atresia results from sixth arch artery defect.
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FIGS. 1.10A AND B: Development of aorta and its branches. (A) Four paired arches (I, II, III and IV) join to form descending aorta. (B) Paired I, II and V arches involute, paired III arches persist as common carotid arteries, right IV arch contribute to formation of right subclavian artery, left IV arch forms aortic arch, pulmonary arteries develop from VI arch, ductus arteriosus (DA) form from the proximal left VI arch.
Our understanding of cardiac embryology and pathology has expanded dramatically with genetic investigations across species from flies to human beings. Hence, classifications based on anatomy alone are increasingly found wanting. The clinician must be open to new associations and potential deviations of seemingly unrelated congenital anomalies.
 
SALIENT FEATURES
  • During 3rd week of embryonic life, clusters of blood and vessel forming capillaries appear in cardiogenic area known as angioblastic islands.
  • During this 3rd week (18th day) the primitive heart tube is formed by fusion of two endothelial tubes. It has inner endocardium and outer myocardium which develop from cardiac jelly.
  • Between 21st to 24th day, the primitive heart tube is formed consisting of right and left horns of SV, SV proper, PA, PV, BC, and TA from below upward.
  • The ventricular myocardium starts beating by 4th week of embryonic life. Consequently atrial myocardium develops which starts beating at a faster rate.
  • At the end of 4th week, a downward growth from the cephalic wall, known as SP divides the PA into right and 10left. LA is derived from left portion and RA is derived from right portion of the PA. The SP fuses with EC below. SS then develops on the right side of the SP to take part in atrial septation that is completed by 34 days. FO is formed due to overlapping of these two septa that allows blood to flow only from right to left (RA to LA). Coronary sinus is formed by left horn and body of the left SV.
  • The EC divides the common AV canal by proliferation of connective tissue into right and left side from which MV and TV take origin.
  • During 6th week of intra-uterine life, the AV node and its bundle develop from dorsal EC of AV canal. Sinoatrial (SA) node develops from SV, which starts beating at faster rate and acts as a pacemaker. By the end of fetal life its place is almost fixed which is close to the orifice of SVC.
  • Interventricular septum (IVS) develops from three sources: (1) ventricular septum, (2) the proximal bulbar septum, and (3) septum intermedium. The septum intermedium is responsible for the membranous part of the IVS and proximal bulbar septum give rise to muscular part. The muscular part gradually fuses with the downward growing membranous septum completing the IVS by 38th to 45th days of gestation. The trabecular cord like attachment in the ventricular wall subsequently develops as chordae tendineae and papillary muscles.
  • The outflow portion develops from right side of BC (conus) that is incorporated to primitive RV, which connects RV to pulmonary artery. Inflow portion of RV develops from right side of primitive RV. The inflow (rough portion) of LV is formed from the left side of the PV. The outflow (smooth or aortic vestibule) develops from dorsal portion of the BC that connects with aorta maintaining aortomitral continuity.
  • The conotruncus, which is the common outflow tract, is separated by a spiral septum into aorta and pulmonary trunk by the end of 9th week of gestation. The aortic and pulmonary orifice is formed from dorsal and ventral portion of BC respectively. The semilunar valves develop from swellings appearing from right and left side of conotruncus which ultimately give rise to aortic and pulmonary valves.
  • The TA forms the aortic sac, which gives rise to six pairs of aortic arches. The first, second, and fifth arches disappear. The third arch forms common carotid and gives rise to internal and external carotids. Right fourth arch forms right subclavian but the left subclavian artery is formed from left seventh intercostal artery. Aortic arch is formed by left fourth and left dorsal arch during 4th to 5th week of gestation. Descending aorta and abdominal aorta is formed by fusion of both dorsal aortae. Pulmonary artery is formed from ventral part of each sixth aortic arch. The dorsal part of left sixth arch gives rise to DA.
  • Right CCV becomes a part of SVC at its opening and RVV gives rise to the terminal part of the IVC.
  • Cardiogenesis which starts about 18th day of gestation is completed by 49th day, thereafter maturation process goes on, even after birth.