Table 1

Total and live birth prevalence of congenital heart defects: the EPICARD

ACC-CHD categoriesNLBTOPSBPrevalence (per 10 000)
Per centPer centPer centTotal*95% CILive birth††95% CI
1. Heterotaxy, including isomerism and mirror imagery3721.675.72.71.20.8 to 1.60.20.1 to 0.5
2. Anomalies of the venous return3183.916.10.01.00.7 to 1.40.80.5 to 1.2
3. Anomalies of the atria and interatrial communications18295.64.40.05.74.9 to 6.65.54.7 to 6.4
4. Anomalies of the atrioventricular junctions and valves21351.242.76.16.75.8 to 7.73.52.8 to 4.2
5. Complex anomalies of atrioventricular connections1353.846.20.00.40.2 to 0.70.20.1 to 0.5
6. Functionally univentricular hearts15830.462.76.95.04.2 to 5.81.51.1 to 2.0
7. VSDs149193.65.70.747.044.6 to 49.444.442.1 to 46.8
8. Anomalies of the ventricular outflow tracts56379.418.52.117.716.3 to 19.314.212.9 to 15.6
9. Anomalies of the extrapericardial arterial trunks17073.523.53.05.34.6 to 6.24.03.3 to 4.7
10. Congenital anomalies of the coronary arteries9100.00.00.00.30.1 to 0.50.30.1 to 0.5
All286781.916.31.890.387.0 to 93.674.871.8 to 77.8
All, excluding cases associated with chromosomal anomalies247189.29.81.077.874.8 to 80.968.467.3 to 73.2
All, excluding cases associated with chromosomal or other anomalies203692.86.40.864.161.4 to 67.060.257.5 to 62.9
All, excluding cases associated with chromosomal or other anomalies and IVSD93084.214.01.829.327.4 to 31.224.923.2 to 26.7
  • *Total number of births (denominator)=317 538.

  • †Total number of live births (denominator)=314 022.

  • CHD, congenital heart disease; IVSD, isolated ventricular septal defects; LB, live births; SB, stillbirths; TOP, termination of pregnancy; VSD, ventricular septal defect.