An accurate, statistically analyzed database is considered to be of fundamental importance in the effort to make our field more quantitative. At present, almost no book on pediatric cardiology, pediatric cardiac surgery, or pediatric cardiac pathology has a database. We initially thought that Abbott’s epochal Atlas of Congenital Cardiac Disease, based on 1000 postmortem cases, was an exception.

However, on further investigation we found that this classic was, to a major degree, a literature review, not a consecutive series of personally studied cases.

Fortunately, however, there are at least four excellent exceptions:

• •
  

  The Report of the New England Regional Infant Cardiac Program (RICP) by Fyler and his associates

  
  
• •
  

  The Baltimore-Washington Infant Study (BWIS) of Ferencz and her colleagues ^,

  
  
• •
  

  The Pathology of Congenital Heart Disease by Bharati and Lev

  
  
• •
  

  Necropsied Cases of Japanese Patients with Congenital Heart Disease by Ando and his coworkers

The publications of Fyler and Ferencz ^, are epidemiologic studies, whereas the books by Bharati and Lev and Ando are pathology studies. To the best of our present knowledge, these are the only published data with which our own findings can be compared statistically.

Although terminology, the understanding of etiology and morphogenesis, and the methods of diagnosis and treatment of congenital heart disease all will change to some extent over time, nonetheless the pathologic anatomy appears to be a virtual constant. Hence, the primary emphasis of the present study is on pathologic anatomic data .

Table 5.1 is a summary of the congenital cardiac pathology database, based on personal study of 3216 consecutive cases. These cases were studied at the Children’s Hospital in Boston, from 1951 to 1997 inclusive, a consecutive 47-year period. We have seven cases between 1944 and 1950, but the consecutive series did not begin until 1951. Cases from 1965 and earlier were studied retrospectively, whereas patients from 1966 on were examined prospectively.

TABLE 5.1

The Congenital Cardiac Pathology Database (n = 3216)

Entity Number	Rank Order	Type of Congenital Heart Disease	Number of Patients	% of Series	95% Confidence Interval
1	1st	Ventricular septal defect, all types	1165	36.23	34.56–37.90
2	2nd	Atrial septal defect, ostium secundum type	793	24.66	23.17–26.15
3	3rd	Patent ductus arteriosus (>2 weeks of age)	565	17.57	16.26–18.88
4	4th	Transposition of the great arteries, all types	488	15.17	13.94–16.40
5	5th	Common atrioventricular canal, all types	481	14.96	13.73–16 .19
6	6th	Tetralogy of Fallot	428	13.31	12.14–14.48
7	7th	Aortic stenosis, all types	398	12.38	11.24–13.52
8	8th	Persistent left/right superior vena cava	415	12.90	11.74–14.06
9	9th	Coarctation of aorta, all types	379	11.78	10.67–12.89
10	10th	Pulmonary stenosis, all types	338	10.51	9.45–11.57
11	11th	Double-outlet right ventricle, all types	265	8.24	7.29–9.19
12	12th	Coronary artery anomaly, all types	261	8.12	7.18–9.06
13	13th	Bicuspid (bicommissural) aortic valve	258	8.02	7.08–8.96
14	14th	Bicuspid (bicommissural) pulmonary valve	248	7.71	6.79–8.63
15	15th	Aortic atresia, valvar	235	7.31	6.41–8.21
16	16th	Right aortic arch in situs solitus or left aortic arch in situs inversus	225	7.00	6.12–7.8
17	17th	Mitral stenosis, congenital	224	6.97	6.09–7.5
18	18th	Mitral regurgitation	216	6.72	5.85–7.59
19	18th	Endocardial fibroelastosis of the left ventricle	216	6.72	5.85–7.59
20	19th	Totally anomalous pulmonary venous connection	203	6.31	5.47–7.15
21	20th	Pulmonary atresia with ventricular septal defect, all types (tetralogy of Fallot excluded)	198	6.16	5.33–6.99
22	21st	Mitral atresia	183	5.59	4.89–6.49
23	22nd	Aberrant right or left subclavian artery	170	5.29	4 .52–6.06
24	23rd	Dextrocardia	120	3.73	3.08–4.38
25	24th	Hypoplasia or absence of the left ventricle	113	3.51	2.87–4.15
26	24th	Tricuspid regurgitation, congenital	113	3.51	2.8 7–4.15
27	25th	Tricuspid atresia	111	3.45	2.82–4.08
28	25th	Truncus arteriosus	111	3.45	2.82–4.08
29	26th	Common atrium	108	3.36	2.74–3.98
30	27th	Asplenia syndrome	94	2.92	2.34–3.50
31	28th	Pulmonary atresia with intact ventricular septum	88	2.74	2.18–3.30
32	29th	Ebstein anomaly	83	2.58	2.03–3.13
33	29th	Tricuspid stenosis, congenital	83	2.58	2.03–3.13
34	30th	Single left ventricle with infundibular outlet chamber	81	2.52	1.98–3.06
35	30th	Right ventricular hypoplasia, primary	81	2.52	1.98–3.06
36	31st	Interrupted aortic arch	67	2.08	1.59–2.57
37	31st	Major aortopulmonary collateral arteries	67	2.08	1.59–2.57
38	32nd	Aortic regurgitation, congenital	64	1.99	1.51–2.47
39	33rd	Unicuspid (unicommissural) aortic valve	63	1.96	1.48–2.44
40	34th	Polyvalvular disease	62	1.93	1.45–2.41
41	35th	Absent ductus arteriosus	59	1.83	1.37–2.29
42	36th	Polysplenia syndrome	57	1.77	1.31–2.23
43	37th	Cleft mitral valve	43	1.34	0.94–1.74
44	38th	Interrupted inferior vena cava	42	1.31	0.92–1.70
45	38th	Partially anomalous pulmonary venous connection	41	1.27	0.88–1.66
46	39th	Straddling tricuspid valve	41	1.27	0.88–1.66
47	40th	Juxtaposition of the atrial appendages, left-sided	38	1.18	0.81–1.55
48	41st	Coronary sinus septal defect	36	1.12	0.76–1.48
49	41st	Premature closure of the foramen ovale	36	1.12	0.76–1.48
50	42nd	Anomalous muscle bundles of the right ventricle	33	1.03	0.68–1.38
51	42nd	Sinusoids	33	1.03	0.68–1.38
52	43rd	Absent pulmonary valve leaflets	31	0.96	0.62–1.30
53	44th	Double-orifice mitral valve	28	0.87	0.55–1.19
54	45th	Hypertrophic cardiomyopathy	26	0.81	0.50–1.12
55	45th	Single right ventricle (left ventricle absent)	26	0.81	0.50–1.12
56	45th	Hypoplastic mitral valve	26	0.81	0.50–1.12
57	46th	Brachiocephalic arteries, anomalies of	25	0.78	0.48–1.08
58	46th	Left ventricular outflow tract obstruction	25	0.78	0.48–1.08
59	47th	Heart block, complete, congenital	24	0.75	0.45–1.05
60	48th	Potentially parachute mitral valve with common atrioventricular canal	23	0.72	0.43–1.01
61	48th	Straddling mitral valve	23	0.72	0.43–1.01
62	48th	Left ventricular inlet and outlet obstruction	23	0.72	0.43–1.01
63	49th	Superoinferior ventricles	22	0.68	0.40–0.96
64	50th	Vascular ring	21	0.65	0.37–0.93
77	55th	Anterolateral muscle of the left ventricle (muscle of Moulaert), prominent	13	0.40	0.18–0.62
78	55th	Marfan syndrome	13	0.40	0.18–0.62
79	55th	Absence of a pulmonary artery branch	13	0.40	0.18–0.62
80	55th	Scimitar syndrome	13	0.40	0.18–0.62
81	56th	Cor triatriatum (sinistrum)	12	0.37	0.16–0.58
82	57th	Double aortic arch	11	0.34	0.14–0.54
83	58th	Sinus venosus defect	10	0.31	0.12–0.50
84	58th	Quadricuspid aortic valve	10	0.31	0.12–0.50
85	58th	Supramitral stenosing ring	10	0.31	0.12–0.50
86	58th	William syndrome	10	0.31	0.12–0.50
87	58th	Crisscross hearts	10	0.31	0.12–0.50
88	58th	Aneurysms of sinus venosus	10	0.31	0.12–0.50
89	59th	Aneurysm of the left ventricle, congenital	9	0.28	0.10–0.46
90	60th	Bicuspid truncal valve	9	0.28	0.10–0.46
91	60th	Ectopia cordis	9	0.28	0.10–0.46
92	60th	Isolated levocardia	9	0.28	0.10–0.46
93	60th	Lung, absence of	9	0.28	0.10–0.46
94	60th	Uhl disease of the right ventricle	9	0.28	0.10–0.46
95	61st	Left ventricular dysplasia	8	0.25	0.08–0.42
96	61st	Pulmonary artery sling	8	0.25	0.08–0.42
97	62nd	Blood cyst, prominent	7	0.22	0.06–0.38
98	62nd	Typical congenital mitral stenosis with left ventricular outflow tract obstruction	7	0.22	0.06–0.38
99	62nd	Quadricuspid pulmonary valve	7	0.22	0.06–0.38
100	62nd	Absence or atresia of the right superior vena cava	7	0.22	0.06 to .38
101	63rd	Common-inlet right ventricle	6	0.19	0.04–0.34
102	63rd	Cantrell syndrome	6	0.19	0.04–0.34
103	63rd	Left ventricular to right atrial shunt	6	0.19	0.04–0.34
104	63rd	Myxomatous mitral valve	6	0.19	0.04–0.34
105	63rd	Absence or hypoplasia of left ventricular papillary muscle, without parachute mitral valve	6	0.19	0.04–0.34
106	63rd	{S,L,S}, i.e., isolated ventricular inversion	6	0.19	0.04–0.34
107	64th	Anatomically corrected malposition {S,D,L}	5	0.16	∗
108	64th	Anomalous muscle bundles of the left ventricle	5	0.16
109	64th	Double-outlet infundibular outlet chamber	5	0.16
110	64th	Discontinuous pulmonary artery branches	5	0.16
111	64th	{I,D,S}, i.e., situs inversus of atria, D-loop ventricles, solitus normally related great arteries	5	0.16
112	64th	Rhabdomyoma	5	0.16
113	65th	Absence of main pulmonary artery	4	0.12
114	65th	Obstructive bulboventricular foramen	4	0.12
115	65th	Chiari network, prominent	4	0.12
116	65th	Displaced septum primum	4	0.12
117	65th	Isolation of the left atrial appendages	4	0.12
118	65th	Retroaortic innominate vein	4	0.12
119	65th	Left ventricular diverticulum, apical	4	0.12
120	65th	Absence or defect of pericardium	4	0.12
121	65th	Pulmonary artery branch from ascending aorta (“hemitruncus”)	4	0.12
122	65th	Functionally single right ventricle, i.e., diminutive left ventricle present	4	0.12
123	66th	Congenitally unguarded tricuspid orifice, i.e., absent tricuspid leaflets	3	0.09
124	66th	Diverticulum of Kommerell	3	0.09
125	66th	Double-outlet right atrium, i.e., one atrioventricular valve opening into each ventricle	3	0.09
126	66th	Intussusception of the left atrial appendage (inside-out appendage)	3	0.09
127	66th	Isolated left subclavian artery	3	0.09
128	66th	Right atrial outlet atresia, i.e., right-sided “mitral” atresia with large left ventricle	3	0.09
129	66th	Raghib syndrome	3	0.09
130	66th	{S,L,I}, i.e., solitus atria, L-loop ventricles, with inverted normally related great arteries	3	0.09
131	67th	Aneurysm of sinus of Valsalva	2	0.06
132	67th	Annuloaortic ectasia	2	0.06
133	67th	Adherent mitral valve (plastered against left ventricular septal surface)	2	0.06
134	67th	Isolated ostium primum type of atrial septal defect (without cleft mitral valve or ventricular septal defect of atrioventricular canal type)	2	0.06
135	67th	{A(S),L,I} i.e., situs ambiguus of the viscera, solitus atria, L-loop ventricles, and inverted normally related great arteries	2	0.06
136	67th	Ostial stenosis of pulmonary arterial branch	2	0.06
137	67th	Atrioventricular situs discordance with atrioventricular alignment concordance	2	0.06
138	67th	Common-inlet left ventricle	2	0.06
139	67th	Crossed pulmonary arteries	2	0.06
140	67th	Common ventricle (ventricular septum absent)	2	0.06
141	67th	Endocardial fibroelastosis of the left ventricle, familial	2	0.06
142	67th	{I,L,I}, i.e., situs inversus of viscera and atria, L-loop ventricles, and inverted normally related great arteries	2	0.06
143	67th	Inferior vena cava, narrowing of at level of liver	2	0.06
144	67th	Moderator band of left ventricle	2	0.06
145	67th	Innominate vein anterior to thymus	2	0.06
146	67th	Malincorporation of common pulmonary vein	2	0.06
147	67th	Right atrium, idiopathic dilatation of	2	0.06
148	67th	Right ventricular dysplasia syndrome	2	0.06
149	67th	Right ventricle, small chambered and thick walled (without pulmonary atresia and intact ventricular septum)	2	0.06
150	67th	Straddling of both tricuspid valve and mitral valve	2	0.06
151	67th	{S,D,I}, i.e., isolated infundibuloarterial inversion	2	0.06
152	67th	Subclavian artery as first branch of aortic arch	2	0.06
153	67th	Right superior vena cava draining into left atrium	2	0.06
154	67th	Triple-orifice mitral valve	2	0.06
155	67th	Triple-orifice tricuspid valve	2	0.06
156	67th	Muscular tricuspid valve	2	0.06
157	68th	Acardia (in twin)	1	0.03
158	68th	Aneurysm, coronary sinus	1	0.03
159	68th	Acommissural aortic valve	1	0.03
160	68th	Absence of aortic valve leaflets	1	0.03
161	68th	{A(I),L,I}, i.e., situs ambiguus of the viscera, inverted atria, L- loop ventricles and inverted normally related great arteries	1	0.03
162	68th	{A(I),L,S}, i.e., isolated infundibuloarterial noninversion	1	0.03
163	68th	Absence of left atrial appendage	1	0.03
164	68th	{A,L,I}, i.e., situs ambiguus of viscera and atria, L-loop ventricles, and inverted normally related great arteries	1	0.03
165	68th	Alagille syndrome, i.e., peripheral pulmonary stenosis, cholestasis, and odd facies	1	0.03
166	68th	Cervical aortic arch	1	0.03
167	68th	Cor triatriatum dextrum	1	0.03
168	68th	Double-inlet and double-outlet right ventricle	1	0.03
169	68th	Double-outlet left atrium, i.e., one atrioventricular valve to each ventricle	1	0.03
170	68th	Fifth aortic arch, persistent	1	0.03
171	68th	Glycogen storage disease (Pompe disease)	1	0.03
172	68th	Infundibular atresia, isolated	1	0.03
173	68th	Carotid artery, isolated	1	0.03
174	68th	{I,D,I}, i.e., isolated ventricular noninversion	1	0.03
175	68th	Left innominate artery, isolated	1	0.03
176	68th	{IS,D,S} with polysplenia, i.e., situs inversus of the viscera, situs solitus of the atria, D-loop ventricles, and solitus normally related great arteries	1	0.03
177	68th	Interseptovalvular space, prominent	1	0.03
178	68th	Inferior vena cava, right-to-left switching of (at liver)	1	0.03
179	68th	Inferior vena cava draining into left atrium via coronary sinus septal defect	1	0.03
180	68th	Levoatrial cardinal vein	1	0.03
181	68th	Left atrium, absence of	1	0.03
182	68th	Left subclavian artery, ostial stenosis of	1	0.03
183	68th	Microcardia	1	0.03
184	68th	Multiple orifice common atrioventricular valve	1	0.03
185	68th	Multiple orifice tricuspid valve	1	0.03
186	68th	Congenitally unguarded mitral orifice, i.e., absence of mitral leaflets	1	0.03
187	68th	Mitral valve causing left ventricular outflow tract obstruction	1	0.03
188	68th	Mitral valve, partly muscular	1	0.03
189	68th	Normally related great arteries with bilateral conus	1	0.03
190	68th	Origin of the right and left pulmonary artery branches from ascending aorta	1	0.03
191	68th	Overriding tricuspid valve	1	0.03
192	68th	Pseudocoarctation of the aorta	1	0.03
193	68th	Premature closure of the ductus arteriosus	1	0.33
194	68th	Patent ductus venosus	1	0.33
195	68th	Premature narrowing of the foramen ovale	1	0.03
196	68th	Rendu-Osler-Weber disease, i.e., pulmonary arteriovenous fistulae and familial hemorrhagic telangiectasia	1	0.03
197	68th	Solitary aorta arising above the right ventricle with absence of the main pulmonary artery	1	0.03
198	68th	Supramitral band, congenital	1	0.03
199	68th	Submitral network	1	0.03
200	68th	Senger syndrome, i.e., nonobstructive hypertrophic cardiomyopathy with cataracts	1	0.03
201	68th	Teratoma, intrapericardial	1	0.03
202	68th	Umbilical vein running anteriorly and superiorly to the liver	1	0.03
203	68th	Vascular vice with totally anomalous pulmonary venous connection	1	0.03

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