Objectives—To present population-based data on prevalence, surgery and mortality for infants and children up to 5 years of age with congenital heart disease (CHD).

Design—Data from the EUROCAT Registry of Congenital Malformations for Funen County, Denmark, 1986-1998.

Results—Five hundred and seventy-three infants and children were diagnosed with a CHD and livebirth prevalence was 7.9 per 1000 births. Thirty-two per cent of all infants and children had an intervention (surgery or catheter treatment) performed. Eighteen per cent died within the first 5 years with the majority of deaths within the first year of life. For 74% of all deaths, surgery had not been performed. There was a decline in mortality for 1994-1998 compared to 1986-1993 both as a percentage of all cases (p < 0.05) and as deaths per 1000 births (p = 0.13), and deaths within the first 28 d after surgery almost disappeared during the study period.

Conclusion—Mortality and morbidity for infants and children with CHD is rather high although surgical mortality has improved considerably. Survival may be improved further for the small group of severely ill newborns dying before surgery. In newborns with multiple malformations, however, survival might not be possible or desirable.