Although a high mumber of patients with congenital heart disease(CHD) undergo surgical palliation or definite correction up to adolescence, adult congenital heart disease(ACHD) may remain a potential lifelong risk factor for infective endocarditis(IE) in patients growing up with congenital heart disease(GUCH). Methods: In a retrospective case study of a tertiary care center long-term clinical course and complications of patients with IE and GUCH were analysed. Results: Data of 52 patients with CHD, who fulfilled the Saiman criteria for infective endocarditis and were treated between April 1986 and March 2001, were identified: Risk factors for infective endocarditis were previous cardiovascular operation(51.9% ), use of foreign material(38.5% ), dental or other surgical procedures without recommended antibiotic prophylaxis(25.0% ), or cardiac catheterization(5.8% ). Staphylococcal(38.9% ) or streptococcal species(35.2% ) were cultivated in most cases as causative microorganisms. Complications were: recurrence of IE(7.7% ), septic embolisms(30.8% ) leading to central nervous complications(7.7% ), embolism of pulmonary arteries(7.7% ), renal arteries(1.9% ), arteries of the extremities(9.6% ), or infarction of spleen(1.9% ). Other cardiac(23.1% ) or extracardiac(13.5% ) complications were frequent. The need of re-operations during or after IE was high(67.3% ). The hospital mortality was 1.9% , late mortality was 7.7% . Conclusions: Patients with IE and CHD show a broad clinical spectrum of cardiac and extracardiac complications. They may lead to a complicative short-and long-term course with the potential risk of death and a high number of re-operation. Efforts have to be made to improve long-term outcome of patients with ACHD by an interdisciplinary cooperation. Although a high mumber of patients with congenital heart disease (CHD) undergo surgical palliation or definite correction up to adolescence, adult congenital heart disease (ACHD) may remain a potential lifelong risk factor for infective endocarditis (IE) in patients growing up with congenital heart disease (GUCH). Methods: In a retrospective case study of a tertiary care center long-term clinical course and complications of patients with IE and GUCH were analysed. Results: Data of 52 patients with CHD, who fulfilled the Saiman criteria for infective endocarditis and were treated between April 1986 and March 2001, were identified: Risk factors for infective endocarditis were previous cardiovascular operations (51.9%), use of foreign material (38.5%), dental or other surgical procedures without recommended antibiotic prophylaxis (25.0%), or cardiac catheterization (5.8%). Staphylococcal (38.9%) or streptococcal species (35.2%) were cultivated in most cases as causative microorganisms. Complic ations were: recurrence of IE (7.7%), septic embolisms (30.8%) leading to central nervous complications (7.7%), embolism of pulmonary arteries (7.7%), renal arteries (1.9%), arteries of the extremities The need of re-operations during or after IE was high (67.3%). The hospital mortality was 1.9%. Other cardiac (23.1%) or extracardiac , late mortality was 7.7%. Conclusions: Patients with IE and CHD show a broad clinical spectrum of cardiac and extracardiac complications. They may lead to a complicative short-and long-term course with the potential risk of death and a high number of re -operation. Efforts have to be made to improve long-term outcome of patients with ACHD by an interdisciplinary cooperation.