Symptomatic coronary artery fistulas (CAF) are associated with significant morbidity and mortality. With the advent of high-resolution 2-dimensional and color Doppler echocardiography, the detection rate of clinically silent CAF has increased, but their clinical significance and outcome have not been defined. The clinical, echocardiographic, electrocardiographic, and angiographic findings and documented follow-up of 31 patients with an echocardiographic finding of a clinically silent coronary artery fistula from 1986 to 1997 were analyzed. Mean age at diagnosis was 7.2+/-8.4 years. Indications for echocardiography were murmur (n = 23), congenital heart disease (n = 2), cardiomegaly (n = 2), chest pain (n = 1), stridor (n = 1), syncope (n = 1), and chest trauma (n = 1). CAF were detected with color Doppler flow mapping in all patients. The origin of the fistula was from the left coronary artery system (n = 27), right coronary artery system (n = 3), and bilateral (n = 1). The exit sites were the pulmonary artery (n = 18), right ventricle (n = 8), right atrium (n = 2), and left ventricle (n = 3). Global and regional left ventricular function were normal in all patients at presentation and follow-up. Spontaneous closure of the fistula was documented in 7 patients (23%) at mean follow-up of 2.6+/-2.0 years. In 23 patients the fistula persisted without intervention. All patients remained asymptomatic, without adverse clinical events or evidence of ischemia at a mean age at follow-up of 9.3+/-9.1 years (range 4 months to 42.0). Based on this experience, there is no evidence that clinically silent CAF diagnosed incidentally by color Doppler echocardiography are associated with adverse clinical outcome in childhood and adolescence. Conservative management with continued follow-up of these patients appears to be appropriate.