BACKGROUND AND OBJECTIVES: The relative proportion of infective endocarditis (IE) with congenital heart disease (CHD) has increased because of improved survival with CHD. This may affect the current profile of IE with CHD. The aim of this study is to assess the changing profiles of IE with CHD. SUBJECTS AND METHODS: All cases diagnosed from January 1985 to May 2006 were retrospectively reviewed and were analyzed according to the diagnosed period (period I-prior to 1995, period II-after 1996). Duke criteria were used for the definition of diagnosis. RESULTS: As a whole, 121 episodes occurred (63 episodes in period I and 58 episodes in period II). Although the mean age was similar for patients diagnosed in the two periods, the proportion of younger age patients was larger during period II. During period II, the number of non-surgical ventricular septal defect (VSD) cases decreased and the number of Rastelli procedure cases increased. Negative blood cultures were similar in patients for both periods. S. viridans was the most common causative organism in patients during both periods. Post-surgical cases and IE after a dental procedure increased during period II. The in-hospital mortality rate, total complication rate, and proportion of IE requiring early surgery were not different for patients in either period. However, the interval to early surgery was shorter for patients during period II (period I 35+/-21.1 days, period II 22+/-16.5 days, p=0.041). CONCLUSION: Our study indicates that IE in CHD is still a major problem with similar clinical features exhibited during the two different periods. The presence of non-surgical VSD as a major underlying defect indicates that one needs to pay more attention to the education of the population at risk.