Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare congenital anomaly, which presents with myocardial ischemia and infarction in children. If left untreated, it carries a high mortality rate in the first year of life. In patients who survive to the adulthood, the coronary steal phenomenon and retrograde left-sided coronary flow provide a substrate for chronic sub-endocardial ischemia, which may lead to left ventricular dysfunction, ischemic mitral

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... tion, malignant ventricular arrhythmias, and sudden cardiac death. We report a case of an 8 years old Saudi female, who was referred to our cardiac center as a case of coronary artery fistula from RCA to RV and referred for possible transcatheter closure and was found to have the anomalous origin of the left coronary artery from the pulmonary artery that was subsequently surgically corrected. The Patient was medically free, asymptomatic apart from on and off palpitations during asthma exacerbations especially after taking Ventolin, she has no history of chest pain, her ECG showed no signs of ischemia, echocardiography showed normal ventricular systolic function which is unusual for ALCAPA cases due to multiple coronary collaterals. Citation: Hawaelrasoul BA, Alsahari A, Elwy A. Late presentation of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was confused with coronary artery fistula. Ann Cardiovasc Thorac Surg 2018;1(3):76-80.