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Thirteen new patients 174 patients previously reported with coronary arteriovenous fistula (CAVF) were reviewed to delineate the course management of CAVF to clarify the role of surgical ligation in the young asymptomatic patient.-Patients were grouped according to age: 99 patients (four new 95 reported) were ≤ 20 years old 88 (nine new 79 reported) were 2 20 years old. Of those under 20 years of age, 19% had preoperative symptoms or CAVF-related complications, including congestive heart failure (CHF) in 6%, subacute bacterial endocarditis in 3% death in one patient. Seventy-six patients younger than 20 years old had CAVF ligation with only one significant complication. In contrast, 63% of the older group all of our nine older patients had preoperative symptoms or complications, including CHF in 19%, SBE in 4%, myocardial infarction (MI) in 9%, death in 14% fistula rupture in one patient. Of the 43 ligated older patients, 23% had surgical complications, including MI in three death in three. Meanpulmonic-to-systemic flow in the entire group was 1.6: 1 did not differ significantly in those with or without symptoms or complications. One of our patients one previously reported had spontaneous CAVF closure. In summary, early elective ligation of CAVF is indicated in all patients because of the high incidence of late symptoms complications the increased morbidity mortality associated with ligation in older patients.