Background: Anomalies of the coronary arteries are uncommon, usually discovered incidentally during diagnostic cardiac catheterization or cardiac computed tomography (CCT). Coronary artery fistula (CAF) is one of the varieties of coronary termination anomalies. Some of CAF patients have symptoms of myocardial ischemia. Methods: In King Abdulaziz university hospital (KAUH) over six years period, five patients presented with acute coronary syndrome (ACS) and were diagnosed to have CAF by cardiac catheterization during the diagnostic workup of ischemic heart disease (IHD), four males and one female, mean age 57.4 ± 6.6 years; four patients had CAF with associated coronary atherosclerotic disease (CAD); one patient had large complex CAF with no significant CAD; all patients with CAD underwent closure/excision of the CAF with surgical revascularization; the patient with no CAD preferred surgical excision over endovascular intervention. Results: All patients had surgery with uneventful postoperative course and they were doing well with 3 to 7 years outpatient follow up. Conclusion: Surgical management is safe in patients with coronary artery anomalies presenting with ACS.
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