Background: Coronary arteriovenous fistulas (CAVFs) are direct connections from one or more coronary arteries to cardiac chambers or a large vessel. They are mostly of congenital origin. The aim of this study was to describe clinical presentation and also delineate the course and management of CAVF. 

Methods: Clinical data, chest x-rays, echocardiographic and angiographic evaluation of 40 patients with congenital CAVF during 1990 to 2008 were reviewed retrospectively. 

Results: Seventeen patients were ≤ 20 years old (42.5%) were mostly asymptomatic, and twenty tree cases were older than 20 years old (57.5%), mostly symptomatic (P<0.05). Twenty one (52.5%) patients had pure CAVF and nineteen (47.5%) patients with associated intarcardiac congenital heart disease (15%) or acquired valvular and coronary arteries diseases (32.5%). CAVFs mostly originated from left anterior descending artery (LAD) (42.5%) and mostly drained into the main pulmonary artery (MPA) (35 %). Twenty-four patients underwent CAVF surgical ligation. From twenty-one patients with pure CAVF, eight (38%) patients were complicated by congestive heart failure and aneurism formation of fistula.

Conclusion: Unlike some previous reports, in our study, the most prevalent origin site for CAVFs was the left anterior descending (LAD). Most patients with CAVFs especially those who went first diagnosed before 20 years old were asymptomatic. On the other hand, as the continuous murmur is not always detected in children or infants, consequently, cases of spontaneous closure may remain undetected. All symptomatic and asymptomatic patients with moderate to severe shunting should be operated on and minimal morbidity and good surgical results could be expected.