[Congenital heart defects. Frequency at autopsy].

Autopsies were performed on 3,071 stillborns and decreased children up to the age of 16 years at the Institute of Pathology of the Charité from 1969 to 1983. Congenital heart disease (CHD) was found in 814, i.e. 26.5% of the autopsies. Results of re-examination of 642 hearts with CHD are discussed. The most common malformations are ventricular septal defects, d-transpositions of the great vessels, tetralogy of Fallot and aortic coarctations. CHD was more frequently found in boys than in girls (1.5 : 1). The majority of the deaths occurred during the first year of life (78.8%). 20.1% of these took place during the perinatal period and 47.2% within the first 6 months of life. Additional cardiac anomalies were associated with the main defect in 81.8% of cases. The most common such associated defects were atrial and ventricular septal defects, aortic coarctations and other aortic arch anomalies. The frequency of extracardiac malformations in CHD was 7.2%. The most common anomalies were of the central nervous system, the gastrointestinal tract and the urinary system. Malformation syndromes were identified in 5.6% of the CHD cases, including Down's syndrome in 1.4%.