Objective:The objective of the work was to make a differential analysis of the incidence and survival of children, born with selected types of birth defects,during the first year of life,based on individual and cohort data. Type of study:Retrospective demographic-epidemiological analysis. Material:The investigation is based on individual data on born infants,diagnosis of birth defects and children who died within the first year in the CR during the period from 1994 -1998.In the cohort 475 834 born infants were followed up,incl.1520 stillborn ones.The number born with a congenital defect was 11 528 of those 33 were stillborn.The number of infants who died during the first year of life was 3037,incl.972 with an birth defect.In the first part of the investigation prenatally diagnosed defects were selected.The authors investigated the mortality rate of infants born with selected birth defects during different periods in the first year of life. Results:During the first year of life in the investigated group of 3037 children died,incl.one half before the age of 10 days,and 75 %before the age of 64 days.In the group of born infants 99.4%survived one year,in the sub-group born with an birth defect just under 92%which is consistent with the values of infant mortality quotients (0.64 and 8.46 per 100 liveborn infants).The lowest survival intensity was confirmed as expected in infants with anencephaly.Patau ’s syndrome (100%lethal by the first year of life),in left heart hypoplasia syndrome and Edward ’s syndrome.Conversely in a high percentage of surviving infants Turner ’s syndrome was present,limbs, Fallot ’s tetralogy,agenesis of the kidneys,and renal hypoplasia (unilateral affection).Conclusion:The success of prenatal diagnosis of these lethal and serious birth defects presented in the submitted paper has a high standard comparable with advanced countries.Despite these advances however birth defects still occur also in born infants and represent a high percentage of the infant mortality and morbidity.In both instances an important complicating factor is that many of these infants are born premature and to the problem of possible solution of an birth defect possible complications ensuing from necessary neonatal intensive care of immature neonates with an birth defect(s)are added.

        Key words: birth defect,incidence,Czech Republic,prenatal diagnosis,stillbirths,perinatal mortality rate, neonatal mortality rate,infant mortality