
OBJECTIVE Reports describing surgery for infective endocarditis (IE) in children and adults with congenital heart disease (CHD) are limited. This study aimed to determine the patient characteristics, preoperative conditions, surgical outcomes, and risk factors in this cohort. METHODS All children and adults with CHD who underwent surgical treatment for IE between 2001 and 2024 were reviewed. Patient characteristics and surgical procedures were evaluated; survival, recurrent IE, and risk factors were analyzed. RESULTS A total of 96 patients underwent surgical treatment for IE, including 52 children and 44 adults with CHD. Underlying CHD included ventricular septal defect in 31 patients, aortic stenosis/insufficiency in 26, and tetralogy of Fallot in 14. The most frequently infected lesion was the aortic valve in 40 patients, and the most frequently detected organism was Staphylococcus species in 34 patients. Vegetation was observed in 65 patients. Median age at surgery was 17.2 (IQR: 11.7-29.4) years. In-hospital mortality was 6.3%, and survival at 5, 10, and 20 years was 90.6, 88.1, and 80.5%, respectively. Freedom from recurrent IE was 96.4% at 20 years. Number of previous operations (hazard ratio (HR): 1.585, p=0.005), gram-negative bacteria (HR: 11.475, p<0.001), emergency operations (HR: 17.988, p=0.001), and longer aortic cross-clamp time (HR: 1.011, p=0.043) were risk factors for mortality. CONCLUSIONS In children and adults with CHD, mortality following surgery for IE was low, and recurrent IE was rare. Individualized management based on the patient's preoperative condition and the causative organism appears to be important for improving survival.