Abstract: | A 42-year-old woman who had a coronary artery fistula, associated with a giant coronary saccular aneurysm was reported. The coronary artery fistula originated from the proximal portion of the right coronary artery drained into the superior vena cava. The chief complaint was heart murmur which was detected at the 2nd intercostal space of the right sternal border. No other symptoms were present. The aneurysm was approximately 5 X 7 cm in size. In the operation using cardiopulmonary bypass, the proximal and distal portions of the coronary artery fistula were ligated successfully without aneurysmorrhaphy. The postoperative conditions was even without any complications. Congenital coronary artery fistulas with a giant saccular aneurysm should be surgically treated as soon as possible because of potential risk of aneurysmal rupture. |