The study presents data on cardiovascular malformations in Bavarian li
vebirths, born between 1984 and 1991. Cases have been ascertained retr
ospectively by reviewing hospital records of all children being referr
ed to a children's hospital up to 2 years of age. The classification s
cheme was based on abnormalities in developmental mechanisms. Among 98
4,570 livebirths, 7020 cases with structural congenital heart disease
were identified. The birth prevalence was 7.1 per 1000 livebirths. Bet
ween 1984 and 1991, total prevalence increased from 5.9/10(3) to 8.0/1
0(3). Prevalence in males was 7.3/10(3) and in females 6.9/10(3). 78.1
% of all heart defects were isolated, the remaining 21.9% were associa
ted either with chromosomal abnormalities (9.6%), non-chromosomal synd
romes (1.0%), or noncardiac malformations of other organ systems (11.3
%). Total fatality rate was 12.0%, with two thirds of deaths occurring
within a month of birth or the following month of life. Data were com
pared with those of the Baltimore-Washington Infant Study. This study
presents for the first time regional data on birth prevalences of cong
enital heart defects in Germany. The classification scheme reduces the
wide spectrum of phenotype cardiovascular defects to several pathogen
etic groups. The defects in each group may be related to similar causa
l factors.