This document provides a subject index for information related to adult congenital heart disease. It lists topics, conditions, procedures, and other terms alphabetically and provides the corresponding page numbers for where they are discussed in the referenced material. Some of the main topics covered include arrhythmias, aortopathies, atrial septal defect, Ebstein's anomaly, Fontan procedure, pulmonary hypertension, tetralogy of Fallot, and transposition of the great arteries. The page numbers are often followed by "f" or "t" to indicate they refer to figures or tables, respectively, on those pages.

1. 203Subject Index
A
ACTA2 gene mutations, 149t
ADMA (asymmetrical dimethylarginine), 196
Adult congenital heart disease (ACHD). See also specific conditions
arrhythmias and. See Arrhythmias
care advances in, 1f, 7
growth of population with, 1, 1f, 2f, 3f
heart failure and. See Heart failure
hospitalization rates in, 4, 5f
mortality in, 4, 4f
outcomes of, 4, 4f
pregnancy and. See Pregnancy
specialty care for, 3f, 4, 6–7, 6f
sudden cardiac death in, 4, 4f
Alagille syndrome, 141, 141f
Amplatzer Septal Occluder, 12, 12f
Angiography
in aortic root dilation, 152f
after Fontan procedure, 86f
magnetic resonance, in repaired tetralogy of Fallot, 182, 183f
in pulmonary hypertension, 109–110, 116f, 118f
Angioplasty with stent implantation, for coarctation of the aorta, 31–32
Angiotensin receptor blockers, for aortopathies, 153
Anticoagulation
after Fontan procedure, 87
in pregnancy, 140–141
Aorta, in tetralogy of Fallot, 40, 42, 43f
Aortic aneurysm. See also Aortopathies
coarctation of the aorta and, 33, 34f
conditions associated with, 150, 151f
echocardiography in, 151f
familial, 149t
Aortic arch, right-sided with mirror image branching, 42, 43f
Aortic dilation. See Aortopathies
Aortic pseudoaneurysm, coarctation of the aorta and, 33, 34f
Aortic regurgitation, echocardiography in, 137f, 138f, 145f
Aortic valve prolapse, in ventricular septal defect, 17
Aortic valve replacement
heart failure after, 191f
pregnancy after, 139–141, 139f, 140f
Aortogram, in coarctation of the aorta, 173f, 175f
Aortopathies
bicuspid aortic valve and, 148, 149f, 154t
case explanation, 148
case history, 148
after coarctation of the aorta repair, 152f, 155f
complications of, 154–155
conditions associated with, 149t, 150, 153
D-TGA and, 150, 151f
diagnosis of, 153
epidemiology of, 148–150
etiology of, 150, 152
follow-up for, 154, 154t
medical management of, 153
pathophysiology of, 153
pregnancy in, 138–139, 138f
in repaired tetralogy of Fallot, 49–50, 50f, 150, 151f
after single ventricle repair, 150, 152f
surgical management of, 153–154, 154t
ventricular septal defect and, 150, 150f
Arrhythmias
in adult congenital heart disease
as cause of death, 4, 4f
incidence of, 158
management of, 163, 170
mechanisms of, 165, 169
in atrial septal defect, 12
after atrial switch operation
case explanation, 158
case history, 158
ECG in, 159–160f, 169f, 192f
mechanisms of, 162, 167
MRI of, 158f
pacemaker for, 60, 60f, 61f, 194f
prevalence of, 59
radiofrequency ablation of, 60, 158, 161f, 162f
types of, 59
in atrioventricular septal defect, 23
after CC-TGA repair, 75–76
in D-TGA, 162–163
in Ebstein’s anomaly, 96, 100
after Fontan procedures, 49, 87, 89, 90f, 162–163, 166–168f
in heart failure, 200
Arterial switch operation. See also D-transposition of the great
arteries (D-TGA)
with atrial switch repair, for CC-TGA, 73
diagnostic evaluation of adult following, 59
follow-up for, 59
long-term complications of, 60t, 64, 151f
mortality and survival rates for, 60t
technique for, 57, 57f
Asymmetrical dimethylarginine (ADMA), 196
Atherosclerosis, in coarctation of the aorta, 33
Athletic participation, Ebstein’s anomaly and, 101
Atrial fibrillation
conditions associated with, 12, 169
Ebstein’s anomaly and, 163, 165f, 192f
in repaired tetralogy of Fallot, 49
treatment of, 170
Atrial flutter
atrial septal defect and, 12
after atrial switch operation, 158, 159f, 192f
ECG in, 159f, 160f, 192f
entrainment mapping of, 160f
after Fontan procedure, 166–168f
radiofrequency ablation of, 158, 161f, 162f
in repaired tetralogy of Fallot, 49
in unrepaired single ventricle, 166f
Note: Page numbers followed by f or t indicate figures or tables, respectively.
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2. 204 Subject Index
Atrial hypertrophy, in Ebstein’s anomaly, 96, 97f
Atrial septal defect
anatomy of, 8
case explanation, 8
case history, 8
clinical presentation/history in, 9
complications of, 12
conditions associated with, 8, 12
Ebstein’s anomaly and, 99
ECG in, 8, 9, 9f
echocardiography in, 8, 9f, 10, 10f, 123f
epidemiology of, 8
etiology and pathophysiology of, 8
heart sounds in, 8
MRI in, 10, 11f
pulmonary hypertension in, 111–112, 112f, 123
unrepaired in adult
case history, 8, 177
diagnosis of, 9–10, 9f, 10, 179–180
echocardiography in, 10, 10f, 11f, 178f
follow-up for, 12
management of, 11–12, 12f, 177–180, 179f,
180f, 181f
Atrial switch repair (Mustard procedure). See also D-transposition of
the great arteries (D-TGA)
with arterial switch repair, for CC-TGA, 73
diagnostic evaluation of adult following, 59
long-term complications of, 59, 60t
arrhythmias. See Arrhythmias, after atrial switch operation
baffle leaks, 62, 63f
baffle obstructions, 60, 61f, 62, 62f
heart failure/ventricular dysfunction, 62, 63f, 187, 188f
mortality and survival rates for, 60t
MRI after, 158f
pregnancy following, 133–134, 134f
with Rastelli procedure, for CC-TGA, 74
technique for, 56–57, 57f, 58f
Atrial tachycardia
ECG in, 159f
mechanism in adult congenital heart disease, 165
radiofrequency ablation of, 158, 161f, 162f
Atrioventricular block, 160f
Atrioventricular canal defect. See Atrioventricular septal defect
Atrioventricular reentry, 163
Atrioventricular septal defect
anatomy of, 20–21
balanced vs. unbalanced, 21
cardiac catheterization in, 22
case explanation, 19
case history, 18
chest radiography in, 22
classification of, 20–21
clinical presentation/history in, 22
complications of, 23
conditions associated with, 20
ECG in, 18, 18f, 22
echocardiography in, 19, 19f, 20f, 21f, 22
epidemiology of, 19–20
etiology and pathophysiology of, 21–22
follow-up for, 22–23
management of, 22
in tetralogy of Fallot, 42
Autosomal dominant polycystic kidney disease (ADPKD), 149t
B
Baffle leaks, after atrial switch operation, 62, 63f
Baffle obstruction, after atrial switch operation,
60, 62, 62f
Balloon angioplasty, for coarctation of the aorta, 31, 172–177,
173–176f
Bicuspid aortic valve
aortopathies and, 148, 149f, 154t
in coarctation of the aorta, 24
follow-up for, 154t
heart failure and, 186
infective endocarditis in, 137, 137f
pregnancy and, 137, 137f
Bidirectional Glenn shunt (BDG), 85, 85f
Blalock-Hanlon atrial septectomy, 56
Blalock-Taussig shunt, 83, 83t, 84f
Bosentan, for Eisenmenger syndrome, 129
Bradyarrhythmias, 162. See also Arrhythmias
Brain natriuretic peptide (BNP)
in heart failure, 134, 196
for risk assessment in pregnant women with CHD,
134, 135f
C
Cardiac catheterization
in Alagille syndrome, 141, 141f
in atrial septal defect, 11
in atrioventricular septal defect, 22
in CC-TGA, 71–72, 74f
in D-TGA, 59
in Ebstein’s anomaly, 98–99
in Eisenmenger syndrome, 127–128
in heart failure, 189, 200
interventional. See Balloon angioplasty
in pregnancy, 141, 141f
in pulmonary hypertension, 106–109
in single ventricle anomalies, 82
in tetralogy of Fallot, 45
in ventricular septal defect, 16, 17f
Cardiac output measurement, 108–109
Carpentier technique, tricuspid valve repair, 100–101
CC-TGA. See Congenitally corrected transposition of the great
arteries (CC-TGA)
Celiprolol, for aortopathies, 153
CHD. See Congenital heart disease (CHD)
Chest radiography
in atrioventricular septal defect, 22
in CC-TGA, 69
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3. 205Subject Index
in Ebstein’s anomaly, 98
in Eisenmenger syndrome, 16, 114f, 126, 126f
in heart failure, 189, 193–195f
in tetralogy of Fallot, 43–44, 44f
in ventricular septal defect, 16, 17f
Coarctation of the aorta
anatomy of, 24, 25f
aortogram in, 173f, 175f
case explanation, 24
case history, 24, 172
conditions associated with, 24
echocardiography in, 26–28, 26t, 27f, 28f
epidemiology of, 24
etiology of, 24
follow-up for, 32–33, 32t
heart failure and, 187, 188f
medical management of, 32
MRI in, 28–29, 29f, 30f, 31f
pathophysiology of, 24–25
physical examination in, 25–26
in pregnancy, 33–34
repair of
aneurysms after, 33, 34f, 155f
aortic dilation after, 152f
complications of, 32–34
indications for, 29
re-coarctation after, 33, 33f
transcatheter balloon angioplasty for
outcomes of, 31, 177
technique, 172, 173f, 174, 174–176f
transcatheter stent placement for, 31–32, 32f
Computed tomography (CT)
in CC-TGA, 69, 73f
in Ebstein’s anomaly, 98
in Eisenmenger syndrome, 127
in single ventricle anomalies, 82
in tetralogy of Fallot, 45
Cone reconstruction, tricuspid valve, 101
Congenital heart disease (CHD)
incidence of, 1
shift from children to adults, 2f, 3f. See also Adult congenital heart
disease (ACHD)
Congenitally corrected transposition of the great arteries
(CC-TGA)
anatomy of, 66
cardiac catheterization in, 71–72, 74f
case explanation, 66
case history, 66
chest radiography in, 69
complications of, 75–76
CT in, 69, 73f
differential diagnosis of, 72
ECG in, 69, 70f
echocardiography in, 69, 70f, 71f
epidemiology of, 66
exercise testing in, 72
heart block/arrhythmias in, 75–76
history in, 68–69
medical management of, 72–73
MRI in, 69, 72f, 73f
nuclear scintigraphy in, 71
physical examination in, 69
physiology of, 67–68, 67f
pregnancy in, 76
right ventricular dysfunction in, 76
surgical management of, 73–75, 75t
tricuspid regurgitation in, 76
ventricular septal defect and, 68, 68f
Conotruncal defects, 154t
Coronary artery anomalies
pulmonary hypertension and, 110, 110f
in tetralogy of Fallot, 42, 42f
Creatinine (Cr), in heart failure, 196
D
D-transposition of the great arteries (D-TGA)
anatomy of, 54–55, 55f
aortopathies and, 151f
case explanation, 54
case history, 54, 158
diagnostic evaluation of adult with, 59, 133
epidemiology of, 54
etiology of, 54
medical management of, 56
pathophysiology of, 55–56
surgical management of
arrhythmias following, 162–163, 192f
arterial switch operation for. See Arterial switch operation
atrial switch repair for. See Atrial switch repair (Mustard
procedure)
long-term follow-up after, 59
pregnancy after, 133–134, 134f
Rashkind balloon septostomy for, 56, 56f
Rastelli operation for. See Rastelli operation
transcatheter management of, 56, 56f
Damus-Kaye-Stansel (DKS) anastomosis, 83t, 84, 84f
Danielson repair, tricuspid valve, 100
DiGeorge syndrome, 39
Diuretics
for Ebstein’s anomaly, 100
after Fontan procedure, 87
Double-switch procedure, for CC-TGA, 73
Down syndrome
atrial septal defect in, 8
atrioventricular septal defect in, 20
tetralogy of Fallot in, 39
Doxycycline, for aortopathies, 153
E
Ebstein’s anomaly
athletic participation in, 101
atrioventricular reentry in, 163
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4. 206 Subject Index
Ebstein’s anomaly (Cont.):
cardiac catheterization in, 98–99
case explanation, 94
case history, 94
chest radiography in, 98, 194f
classification of, 95–96
clinical features of, 96
congenital lesions associated with, 68, 96
ECG in, 96, 97f, 164f, 165f, 192f
echocardiography in, 98, 98f
epidemiology of, 94
etiology and pathophysiology of, 94–95, 95f
exercise testing in, 99
long-term complications of, 101
management of, 99–100
arrhythmia ablation for, 100
medical, 100
surgical, 100–101
MRI in, 98, 99f, 164f
physical examination in, 96
pregnancy and, 101
prognosis for, 101
ECG. See Electrocardiography (ECG)
Echocardiography
in aortic regurgitation, 137f, 138f, 145f
in atrial septal defect, 8, 8f, 9f, 10, 10f, 178f
in atrioventricular septal defect, 19f, 20f, 21f, 22
in CC-TGA, 69, 70f, 71f
in coarctation of the aorta, 26–28, 26t, 27f, 28f
in dilated cardiomyopathy, 195f
in Ebstein’s anomaly, 98, 98f
in Eisenmenger syndrome, 114f, 126–127, 127f
fetal, 135–136, 136f
in heart failure, 189
in pregnancy, 135t, 145f
in pulmonary hypertension, 105–106, 105f, 106t, 107f, 108f
in single ventricle anomalies, 82
in tetralogy of Fallot, 44, 46f
in ventricular septal defect, 14f, 15f, 16
for women with CHD considering pregnancy, 134, 134f
Edward syndrome, tetralogy of Fallot in, 39
Ehlers-Danlos syndrome, 149t
Eisenmenger syndrome
atrial septal defects in, 123, 123f
cardiac catheterization in, 127–128
case explanation, 121
case history, 121, 122f
chest radiography in, 16, 114f, 126, 126f
CT in, 127
ECG in, 125–126, 125f, 193f
echocardiography in, 121, 121f, 122f, 126–127, 127f
epidemiology of, 121–122
etiology and pathophysiology of, 22, 122–123
history in, 124
iatrogenic, 123, 124f
laboratory studies in, 129
long-term outcomes of, 130–131
medical management of, 129–130
MRI in, 127, 128f
patent ductus arteriosus in, 123, 124f
physical examination in, 124–125
prognosis for, 115, 131
pulmonary hypertension in, 113–115, 114f, 115f, 116f, 123
surgical management of, 130
thrombosis in, 115
in women, 130
Electrocardiography (ECG)
in acute prosthetic valve insufficiency, 191f
in atrial fibrillation, 165f, 192f
in atrial flutter, 159f, 160f, 192f
in atrial septal defect, 8, 8f
in atrial tachycardia, 159f
in atrioventricular septal defect, 18, 18f, 22
in CC-TGA, 69, 70f
in Ebstein’s anomaly, 96, 97f, 164f, 165f
in Eisenmenger syndrome, 125–126, 125f, 193f
in heart failure, 189, 193–195f
in hypertrophic cardiomyopathy, 190f, 191f
in left ventricular noncompaction, 190f
in supraventricular arrhythmias, 162–163
in tetralogy of Fallot, 44f
in ventricular septal defect, 13, 13f, 16, 193f
Endocardial cushion defect. See Atrioventricular septal defect
Endothelin receptor antagonists, for Eisenmenger syndrome, 129
Entrainment mapping, 160f
Erythrocytosis, in Eisenmenger syndrome, 129–130
Exercise testing
in CC-TGA, 72
in coarctation of the aorta, 33
in Ebstein’s anomaly, 99
in Eisenmenger syndrome, 128–129
in heart failure, 200
in single ventricle anomalies, 82
in women with CHD considering pregnancy, 134
F
Fetus, echocardiographic screening in, 135–136, 136f
Fick principle, 108–109
Fontan procedures
angiography after, 86f
aortopulmonary connections in, 85, 86f
case explanation, 80
case history, 78–80, 78f, 79f, 80f
complications of
arrhythmias, 87, 89, 90f, 162–163, 166–168f
decreased exercise tolerance, 87
diagnosis of, 82–83
growth failure, 78, 79f, 87
liver failure, 89, 91f
neurologic, 92
plastic bronchitis, 89, 90f
protein losing enteropathy, 89
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5. 207Subject Index
psychiatric, 92
surgical management of. See Single ventricle anomalies, surgical
management of
thromboembolic, 92, 92f
ventricular dysfunction, 89, 91f, 92
lateral tunnel, 79f, 85, 169f, 197f
MRI after, 86f
total cavopulmonary connection, 85, 87, 88f
G
Growth failure, after Fontan procedure, 78, 79f, 87
H
Heart block, after CC-TGA repair, 75–76
Heart failure
in adult congenital heart disease
biomarkers of, 189, 196–197
cardiac catheterization in, 189
case explanation, 186
case history, 186
as cause of death, 4, 4f
chest radiography in, 189, 193–195f
diagnosis of, 189
ECG in, 189, 190–193f
echocardiography in, 189, 195f
epidemiology of, 186
etiology and pathophysiology of, 187
follow-up for, 199–200
medical management of, 197
MRI in, 189
probability by age and type of defect, 188f
surgical management of, 197, 199
after aortic valve replacement, 191f
in atrioventricular septal defect, 21
classification of, 187
in hypertrophic cardiomyopathy, 190f, 191f
in left ventricular noncompaction, 190f
in repaired tetralogy of Fallot, 195f
after surgical repair of D-TGA, 62, 133–134, 134f
Heart-lung transplantation, for Eisenmenger syndrome, 130
Heart sounds
in atrial septal defect, 8
in atrioventricular septal defect, 18
in CC-TGA, 69
in Ebstein’s anomaly, 96
in tetralogy of Fallot, 43
in ventricular septal defect, 13, 16
Heterotaxy syndrome, 20, 81t
Holt-Oram syndrome, 8
Hypertension, in coarctation of the aorta, 24–25, 32
Hypertrophic cardiomyopathy, 190f, 191f
Hypoplastic left heart syndrome, 81t
I
Implantable cardioverter-defibrillators, for ventricular
tachyarrhythmias, 170
Indicator dilution method, cardiac output measurement, 108
Infective endocarditis
in pregnant woman with CHD, 137, 137f
ventricular septal defect and, 17
Intra-atrial reentrant tachycardia
vs. atrial flutter, 165
ECG in, 167, 169f
risk factors for, 167
treatment of, 167–169
K
Kawashima procedure, 87, 89f
L
L-TGA (ventricular inversion), 66, 67f, 68. See also Congenitally
corrected transposition of the great arteries (CC-TGA)
Left ventricular dysfunction, in repaired tetralogy of Fallot, 48–49
Left ventricular noncompaction
case history, 186
ECG in, 190f
heart failure and, 187, 190f
MRI in, 196f
pathophysiology of, 187, 188f
Lithium, in pregnancy, 94
Liver failure, after Fontan procedures, 89, 91f
Loeys-Dietz syndrome
aortopathies in, 149t, 154t
follow-up for, 154t
incidence of, 149t
Low-molecular-weight heparin (LMWH), in pregnancy, 140
M
Magnetic resonance angiography (MRA), in repaired tetralogy of
Fallot, 182, 183f
Magnetic resonance imaging (MRI)
in aortic root dilation, 151f, 152f
in atrial septal defect, 10, 11f
after atrial switch operation, 158f
in CC-TGA, 69, 72f, 73f
in coarctation of the aorta, 28–29, 29f, 30f, 31f
in Ebstein’s anomaly, 98, 99f, 164f
in Eisenmenger syndrome, 127, 128f
after Fontan procedure, 86f
in heart failure, 189
in left ventricular noncompaction, 196f
in single ventricle anomalies, 82
in tetralogy of Fallot, 44–45, 197–199f
in truncus arteriosus, 135f
Marfan syndrome
aortopathies in, 149t, 154t
follow-up for, 154t
incidence of, 149t
pregnancy in, 138–139, 138f
Matrix metalloproteinases (MMP), in aortic aneurysm
formation, 153
Melody valve, 183–184, 184f
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6. 208 Subject Index
Mitral valve replacement, pregnancy after, 139–141, 139f, 140f
Murmurs
in atrioventricular septal defect, 18, 19, 22
in CC-TGA, 69
in Ebstein’s anomaly, 96
in ventricular septal defect, 16
Mustard procedure. See Atrial switch repair (Mustard procedure)
MYH11 gene mutations, 149t
N
Neurofibromatosis, 149t
Nuclear scintigraphy, in CC-TGA, 71
O
Ostium primum defect, 8. See also Atrial septal defect
Ostium secundum defect, 8, 10. See also Atrial septal defect
Oxygen therapy, for Eisenmenger syndrome, 129
P
Pacemaker therapy
for CC-TGA, 75–76
indications for, 163
Patau syndrome, tetralogy of Fallot in, 39
Patent ductus arteriosus
coarctation of the aorta and, 24
in Eisenmenger syndrome, 123, 124f
Patent foramen ovale, Ebstein’s anomaly and, 96
Phosphodiesterase inhibitors, for Eisenmenger syndrome, 129
Plastic bronchitis, after Fontan procedures, 89, 90f
PR prolongation, in atrioventricular septal defect, 19
Pregnancy
anticoagulation management during, 140–141
atrial septal defect in, 8
after atrial switch surgery, 133–134
cardiac catheterization during, 141, 141f
cardiac surgery during, 142
in CC-TGA, 76
coarctation of the aorta and, 33–34
Ebstein’s anomaly and, 101
in Eisenmenger syndrome, 130
infective endocarditis in, 137, 137f
in Marfan syndrome, 138–139, 138f
physiologic changes in, 142
in repaired tetralogy of Fallot, 50
in truncus arteriosus, 135–136, 135f
in women with CHD
cardiac risk assessment for, 134, 134f, 135t, 142, 143t, 144t
cardiac surgery during, 142
echocardiography in, 134, 134f, 145f
epidemiology of, 142
fetal outcomes, 144
hemodynamics of, 142, 144f
maternal outcomes, 142
with mechanical valve replacements, 139–141, 139f, 140f
Pro-B-type natriuretic peptide, in heart failure, 196
Prostacyclins, for Eisenmenger syndrome, 129
Prostaglandin E1, for D-TGA, 56
Protein-losing enteropathy, after Fontan procedures, 82, 89
Pulmonary artery, in tetralogy of Fallot, 41f
Pulmonary atresia
incidence of, 81t
with tetralogy of Fallot, 40
Pulmonary hypertension
angiography in, 109–110
cardiac catheterization in, 106–109
diagnosis of, 104–105
echocardiography in, 105–106, 105f, 106t, 107f, 108f
in Eisenmenger syndrome, 113–115, 114f, 115f, 116f, 123
elevated distal pressure and, 110–111, 110f
elevated flow and, 111–112, 111f, 112f, 113f, 113t
patent ductus arteriosus and, 123, 124f
physiology of, 104, 104f
in pulmonary vascular disease, 118, 118f, 119f
after shunt repair, 115–118, 116f, 117f
Pulmonary regurgitation, in repaired tetralogy of Fallot, 45, 46f,
47f, 182
Pulmonary valve, in tetralogy of Fallot, 40, 41f
Pulmonary valve replacement, in repaired tetralogy of Fallot
MRI in, 47–48, 48f
timing of, 47, 47f
transcatheter, 48, 182–184, 183f, 184f
Pulmonary vascular remodeling, after repair of shunt lesions,
116, 118, 118f
Pulmonary vasodilators
for Eisenmenger syndrome, 129
after Fontan procedure, 87
Pulmonary vein stenosis, 118, 119f
Pulmonary wedge angiography, 109
R
Radiofrequency ablation, for atrial tachycardia, 158, 161f, 162f
Rashkind procedure, 56, 56f
Rastelli operation. See also D-transposition of the great arteries
(D-TGA)
with atrial switch repair, for CC-TGA, 74
long-term complications of, 60t, 64
mortality and survival rates for, 60t, 64
technique for, 57, 58f
Right atrial enlargement, in Ebstein’s anomaly, 96, 97f
Right bundle branch block
in atrial septal defect, 8, 9f
in tetralogy of Fallot, 44f
in ventricular septal defect, 15
Right ventricular dysfunction
after CC-TGA repair, 76
after D-TGA repair, 62, 63f
in Eisenmenger syndrome, 113, 114f
after tetralogy of Fallot repair, 45, 47, 47f
Right ventricular outflow tract acceleration time,
106, 107f, 108f
Right ventricular outlet flow obstruction, in tetralogy of Fallot,
40, 41f, 43
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7. 209Subject Index
S
Sano conduit, 83, 83t, 84, 84f
Senning procedure, 56, 187
Sildenafil
for Eisenmenger syndrome, 129
after Fontan procedure, 87
Single ventricle anomalies
cardiac catheterization in, 82
diagnosis of, 81–82
echocardiography in, 82
epidemiology of, 80–81
etiology of, 81, 81t
exercise testing in, 82
after Fontan palliation. See Fontan procedures
heart failure and, 187, 188f
laboratory evaluation in, 82–83
medical management of, 87
MRI/CT in, 82
pathophysiology of, 81
surgical management of
bidirectional Glenn shunt for, 85, 85f
Kawashima procedure for, 87, 89f
procedures, 83t
for pulmonary blood flow, 83–84, 84f
for pulmonary vascular protection, 85
for systemic blood flow, 84, 84f
total cavopulmonary connection for, 85, 87, 88f
Sinus venosus defect, 8, 10. See also Atrial septal defect
SMAD3 gene mutations, 149t
Stents, for coarctation of the aorta, 31–32, 32f
Sudden cardiac death
in adult congenital heart disease, 4, 4f
in Ebstein’s anomaly, 101
in repaired tetralogy of Fallot, 49
Supraventricular arrhythmias
in Ebstein’s anomaly, 96, 97f
ECG in, 96, 97f, 162–163
electrophysiology studies of, 163
epidemiology of, 162
in repaired tetralogy of Fallot, 49
T
Tetralogy of Fallot
anatomy of, 40–42, 40f, 41f, 42f, 43f
aortopathies and, 150, 150f
with atrioventricular septal defect, 42
case explanation, 39
case history, 39
chest radiography in, 43–44, 44f
conditions associated with, 39
coronary artery abnormalities in, 42, 42f
ECG in, 43, 44f
echocardiography in, 44
epidemiology of, 39
etiology of, 39
MRI in, 44–45
pathophysiology of, 42–43
physical examination in, 43
with pulmonary atresia, 40, 42
repair of
activity restrictions after, 50–51
aortopathies after, 49–50, 50f, 150, 151f
arrhythmias after, 49, 162–163
echocardiography after, 46f
heart failure/left ventricular dysfunction after, 48–49,
187, 188f, 195f, 198f, 199f
pregnancy after, 50, 139–141, 140f, 141f
pulmonary regurgitation after, 45, 46f
pulmonary valve replacement after, 47–48, 48f, 182–184,
183f, 184f
right ventricular dilatation after, 45, 47, 47f, 198f
techniques for, 45, 46f
right-sided aortic arch with mirror image branching in, 42, 43f
survival rate in, 45
unrepaired, mortality from, 45
TGFBRI/II gene mutations, 149t
Total cavopulmonary connection, 85, 87, 88f
Transforming growth factor beta signaling, 153
Transposition of the great arteries. See Congenitally corrected
transposition of the great arteries (CC-TGA);
D-transposition of the great arteries (D-TGA)
Tricuspid atresia, 81t
Tricuspid regurgitation, after CC-TGA repair, 76
Tricuspid valve anomaly. See Ebstein’s anomaly
Tricuspid valve replacement, for CC-TGA, 74, 76
Trisomy 21. See Down syndrome
Trisomy 13, tetralogy of Fallot in, 39
Trisomy 18, tetralogy of Fallot in, 39
Truncus arteriosus
in fetus, 136f
pregnancy in, 135–136, 135f, 136f
Turner syndrome
aortopathies in, 148, 149t, 154t
coarctation of the aorta in, 24
follow-up for, 154t
incidence of, 149t
Twin AV nodes, 163
U
Unfractionated heparin (UFH), in pregnancy, 140–141
V
Ventricular inversion (L-TGA), 66, 67f, 68. See also Congenitally
corrected transposition of the great arteries (CC-TGA)
Ventricular septal defect
anatomy of, 16
aortopathies and, 150, 150f
cardiac catheterization in, 16, 17f
case explanation, 15
case history, 13
CC-TGA and, 68, 68f
chest radiography in, 16, 17f
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8. 210 Subject Index
Ventricular septal defect (Cont.):
clinical presentation/history in, 16
complications of, 17
conditions associated with, 16
ECG in, 13, 13f, 16, 191f
echocardiography in, 14f, 15f, 16
epidemiology of, 15–16
etiology and pathophysiology of, 16
follow-up for, 17
heart failure in, 191f
heart sounds in, 13
management of, 16–17
pulmonary hypertension in, 123
in tetralogy of Fallot, 40, 40f, 41f
Ventricular systolic dysfunction, after Fontan procedures, 89, 91f, 92
Ventricular tachyarrhythmias
conditions associated with, 170
risk stratification for, 170
sudden cardiac death and, 170
in tetralogy of Fallot, 49
treatment of, 170
Ventriculogram, in ventricular septal defect, 17f
W
Warfarin, in pregnancy, 140
Wedge angiography, pulmonary, 109, 116f
Wolff-Parkinson-White pattern, in Ebstein’s anomaly, 96, 164f
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