Effective Long-Term Surgical Management of Congenital Coronary Artery Fistulas

Congenital coronary artery fistula (CAF) is a rare anomaly involving communication between the coronary artery and any cardiac chamber. We retrospectively studied 23 patients with CAF who were surgically treated at 2 institutes over the past 38 years. All patients had continuous murmur and were diagnosed using echocardiography and cardiac catheterization. Eighteen patients had no other heart anomalies, and 5 had an associated anomaly. Fifteen patients were treated using cardiopulmonary bypass via differential surgical approaches (6 patients, CAF orifice closure through cardiac chamber; 6, coronary arteriotomy for CAF orifice closure; and 3, CAF ligation). Eight patients underwent CAF ligation without cardiopulmonary bypass. CAF originated from the right coronary artery in 11 patients; the left coronary artery in 10, and both arteries in 2. Drainage occurred at the following sites: the right ventricle (10 patients), right atrium (6), left ventricle (4), left atrium (2), and pulmonary artery (1). All surgeries were performed through a median sternotomy. There were no mortalities during or after the hospital stay. Aortic root replacement was performed in 1 patient 30 years after the CAF surgery. Three of the 6 patients who underwent coronary arteriotomy for CAF orifice closure showed coronary artery occlusion at the distal coronary arteriotomy site with long-term collateral formation. Surgical management of CAF was thus effective, resulting in 100% long-term survival and closure rates. Dilated CAF-associated vessels have been normalized for the long term. CAF should be therefore considered even in asymptomatic patients because of the risk of future complications.