[Show abstract][Hide abstract] ABSTRACT: Background: The aim of this study was to determine the clinical course and outcome of coronary artery anomaliesincluding anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnosed by coronary artery angiography (CAA) at our institution. The term coronary artery anomaly refers to a wide range of congenital abnormalities, involving the origin, course and the structure of epicardial coronary arteries. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an extremely rare, but potentially fatal congenital coronary anomaly. Patients and Methods: The review included 21280 patients undergoing CAA at Medical Centers of Shiraz University between years 1997 -2006. The hospital records of all patients with diagnosis of ALCAPA reviewed for analysis. Data were expressed as percentages. Results: There were 149 (0.7 %) cases of coronary anomalies, including 9 (6.04%) proven cases of ALCAPA. In ALCAPA group ,five patients presented with congestive heart failure (CHF) and a non-specific systolic murmur on examination. Isolated mitral valve regurgitation (MR), cerebrovascular accident due to embolization of a mural thrombus, and chest pain were the main presentations in three patients. Two of the patients were under follow up with presumptive diagnosis of coronary artery fistula. The patients aged between 4 months and 35 years. Surgical correction done for all the patients with ALCAPA by direct reimplantation in 8 patients and Takeuchi technique in one. We had one early hospital mortality in our group. Conclusion: ALCAPA should be considered in differential diagnosis of any patient presenting with dilated cardiomyopathy, CHF, MR or chest pain syndrome. Awareness of such pathology can prevent or decrease the morbidity and mortality of a potentially fatal congenital heart disease.