Surgical outcome of coronary artery fistulas repair in children.

Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by endocarditis, myocardial infarction, or coronary aneurysms. This article reviews the clinical characteristics and surgical outcome of 10 patients with CAF in Rajae Heart Center. From 1990 to 2000, 10 patients (age, 6 months to 15 years; mean age, 8.5 years) were diagnosed with CAF by echocardiography and cardiac catheterization. Six were female and 4 were male. All patients with isolated CAF (9) were asymptomatic. One patient with associated anomaly (mitral valve prolapse with severe mitral regurgitation) had dyspnea on exertion and palpitation. Five fistulas originated from the right coronary artery-three from the left and two from the left circumflex. Drainage was to the right ventricle (7), right atrium (2), and pulmonary artery (1). The ratio of pulmonary to systemic flow ranged between 1 and 1.6. All patients had surgical ligation. In the symptomatic patient, in addition to ligation, mitral valve replacement was performed. There was no operative or late death. Follow-up evaluation (range, 1-6 years; mean, 4.2 years) showed no evidence of recurrent or residual CAF. Surgical management of CAF is a safe and effective treatment resulting in 100% survival and clousure rate.