Objective: Analysis of the proportion of selected types of congenital defects in stillbirth, frequent neonatal and perinatal death rate in the Czech Republic in the years 1994-2002. Design: A retrospective demographic-epidemiological analysis. Setting: Institute for the Care of Mother and Child, Prague. Materials and Methods: The study analyzed individual anonymous data from the Institute of Medical Information and Statistics of the Czech Republic in the period of 1994-2002 (preliminary data only for 2002) from the National Registry of Congential Defects. Fourteen types of defects were selected from the data set. The proportion of children with a certain selected congenital defect which died during the perinatal period were calculated in comparison with the total number of subjects diagnosed for this congential defect in the Czech Republic over the period of observation. Results: A total of 4,436 children died during the perinatal period in the Czech Republic over the period of 1994 – 2002, while 879 of them suffered from one or more congenital defects. The congenital defects represent more than 10% of stillbirth in this period, 32.5% of deceased individuals in early neonatal death rate and almost 20% in perinatal death rate. In 25% of these cases the prenatal diagnosis was positive, whereas prenatal diagnosis was negative or not indicated (a negative screening) in 48% of cases, and in 27% the data were not fi lled out. More than 45% of children deceased with a congenital defect in the perinatal period had two or more congenital defects. Conclusions: Analysis of these results from the registry of congenital defects in the Czech Republic contributed to the information on prenatal deaths in the period of 1994 – 2002. The proportion of congenital defects in perinatal death rate makes up almost 20%, but it also signifi cantly participates in late newborn and post-newborn mortality and morbidity.

        Key words: congenital defects, perinatal mortality, perinatal diagnostics, anencephaly, spina bifi da,