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Table 3 Distribution of the IPCCC codes in the ten categories of the anatomic and clinical classification of congenital heart defects (ACC-CHD) in the EPICARD study

From: Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

Group Number of codes Number of CHD cases %
1. Heterotaxy, including isomerism and mirror-imagery 1
2
3
4
5
6
1
3
7
10
14
2
37
2.7
8.1
18.9
27
37.9
5.4
100.0
2. Anomalies of the venous return 1
2
3
4
5
6
28
3
0
0
0
0
31
90.3
9.7
0.0
0.0
0.0
0.0
100.0
3. Anomalies of the atria and interatrial communications 1
2
3
4
5
6
159
19
4
0
0
0
182
87.4
10.4
2.2
0.0
0.0
0.0
100.0
4. Anomalies of the atrioventricular junctions and valves 1
2
3
4
5
6
158
44
9
1
1
0
213
74.2
20.6
4.2
0.5
0.5
0.0
100.0
5. Complex anomalies of atrioventricular connections 1
2
3
4
5
6
5
3
4
0
1
0
13
38.5
23.1
30.8
0.0
7.7
0.0
100.0
6. Functionally univentricular hearts 1
2
3
4
5
6
100
34
15
3
6
0
158
63.3
21.5
9.5
1.9
3.8
0.0
100.0
7. Ventricular septal defects (VSD) 1
2
3
4
5
6
1337
135
19
1
0
0
1492
89.6
9
1.3
0.1
0.0
0
100.0
8. Anomalies of the ventricular outflow tracts (ventriculo-arterial connections) 1
2
3
4
5
6
404
96
43
17
3
0
563
71.8
17.1
7.6
3.0
0.5
0.0
100.0
9. Anomalies of the extrapericardial arterial trunks 1
2
3
4
5
6
78
68
16
5
2
0
169
46.1
40.2
9.5
3
1.2
0.0
100.0
10. Congenital anomalies of the coronary arteries 1
2
3
4
5
6
9
0
0
0
0
0
9
100.0
0.0
0.0
0.0
0.0
0.0
100.0