BACKGROUND: Infective endocarditis is a rare disease in the pediatric population. We sought to define patient characteristics and outcomes of surgical therapy for endocarditis in children. METHODS: We performed a retrospective review of all patients with infective endocarditis who received surgical therapy between January 1, 1990, and March 1. 2011. We were interested in their congenital heart defect, prior surgical procedures, and outcome of the operation. RESULTS: We identified 35 cases of endocarditis in 34 patients requiring surgical intervention. Mean age was 10.7 +/- 8.8 years. There was a bimodal age distribution at presentation: 11 (31%) were younger than 1 year and 15 (43%) were 10 to 21 years. Of the 34 patients, 22 (63%) had no history of prior cardiac operation. The infective organism was identified in 30 (86%), with Staphylococcus aureus (n = 8) and Streptococcus viridans (n = 6) predominating. Valve replacement was performed in 22 patients and valve repair in 10. All patients received 6 weeks of postoperative intravenous antimicrobial therapy. Operative mortality was 15% (5 of 34). The 5 deaths occurred in infants who were a mean age of 2.5 months, and 3 of the 5 infants (60%) were premature. Of 4 patients with fungal infection, 3 patients died. The Ross operation was performed successfully in 5 patients with severe aortic valve disease. Reoperations (n = 10 [28%]) included valve replacement in 5 and conduit replacement in 3, all but 1 due to somatic growth resulting in functional stenosis. CONCLUSIONS: The outcome of surgical therapy for endocarditis in children was similar to that reported for adults, with an overall mortality of 15%. The Ross operation was very effective in patients with aortic valve endocarditis. There is a significant incidence of late reoperation for valve and conduit replacement due to somatic growth. Age younger than 1 year, prematurity, and fungal organisms appear to be risk factors for death. Patients surviving to discharge had good outcomes, with no episodes of recurrent endocarditis.