Coronary artery fistula: Single-center experience spanning 17 years

Background: Coronary artery fistula (CAF) is an uncommon form of congenital heart disease. It is often diagnosed incidentally during angiograms. We have reported on clinical characteristics, diagnosis, and management of CAF. Methods: Retrospective review of a tertiary referral institution's database identified 30 patients with CAF between 1987 and 2004. Mean follow-up was 31.61 ± 48.03 months. Results: Mean age was 60 ± 12.7 years. Most common site of CAF origin was the left anterior descending artery (LAD) (14, 46.7%). The most common site of drainage was the main pulmonary artery (15, 50%). Therapeutic strategies were based on symptoms and shunt size. Conservative management was the option in 17 patients (56.7%) with small shunts and mild or no symptoms. Patients with moderate/severe symptoms and/or large shunts were treated with either percutaneous embolization (6, 20%) or surgical ligation (7, 23.3%). Four patients (13.3%) died during follow-up. No deaths were reported in the embolization group, two patients died of cancer in the conservative management group, and two patients died in the surgical group due to cardiac tamponade and cancer, respectively. Conclusions: Origin of CAF was predominantly from the left system. Clinical presentations were variable depending on type, size of fistula, and the presence of other cardiac conditions. Management of CAF is still controversial and treatment of adult asymptomatic patients with nonsignificant shunting is still a matter of debate. Newer imaging modalities may enhance noninvasive diagnosis. A national registry is necessary for further insights into optimal treatment for large fistulae and the natural history of smaller fistulae.