Methods: Between January 2008 and December 2012, 10 pediatric patients with CAVF were diagnosed in our institution. Demographic characteristics, symptoms and clinical findings, electrocardiographic, echocardiographic, angiographic findings, clinical courses after treatment were evaluated retrospectively.

Results: Congenital coronary arteriovenous fistulas were originated from the left coronary artery in six patients, right coronary artery in three patients, and the left circumflex artery in one patient. The drainage site of most CAVFs was the right ventricle, as expected and drainage to pulmonary artery (2) and right atrium (1) were other common anatomical locations, respectively. We followed the patients for the degree of shunt and for ongoing enlargement of coronary arteries for six months. Proper intervention for surgery or transcatheter occlusion was based on individual basis. Surgical ligation was the preferred management in three patients, as the anatomic features of fistulas were not appropriate for a catheter-based intervention. A catheterbased attempt for interventional closure was unsuccessful in a patient and underwent surgical ligation.

Conclusion: Our study results suggest that it is important to diagnose CAVF in childhood due to the high risk of complications seen in adulthood, particularly, such as heart failure, myocardial ischemia, infective endocarditis and arrhythmias.