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Background Congenital heart disease (CHD) is a lifelong predisposing condition for infective endocarditis (IE). As a consequence of advances in pediatric care, the number of adults with CHD is now exceeding the number of children. The goal of the present study was to determine the cumulative incidence of IE in patients with CHD and detect temporal changes compared with controls. Methods Nationwide registry-based case-control study of patients with CHD born 1930–2017 matched with 10 random controls. Infective endocarditis episodes were linked using the Swedish 10-digit personal identification number. Results In total, 89 541 patients with CHD and 890 470 matched controls were included. In patients with CHD, 1477 IE episodes were registered and 447 episodes in controls. Patients with CHD had 8.5% cumulative incidence of IE at age 87 years, compared with 0.7% in matched controls. Incidence rate of IE per 100 000 person-years was 65.5 (95% confidence interval [CI] 62.2–68.9) and 1.8 (95% CI: 1.7–2.0) in CHD patients and controls, respectively. By age 18 years, patients with CHD had an IE incidence similar to that of 81-year-old controls. Incidence of IE differed by age but not by birth year. Bacterial etiology was registered from 1997 in half of the IE episodes; among CHD IE cases, 43.3% were caused by streptococci and 29.8% by Staphylococcus aureus. Conclusions Infective endocarditis remains an important complication in patients with CHD. Incidence correlate with age and the number of IE episodes are expected to increase as the CHD population grow older.

Cumulative Incidence of Infective Endocarditis in Patients with Congenital Heart Disease: A Nationwide, Case-Control Study Over Nine Decades