Anomalous origin of the left coronary artery from the proper sinus with acute angulation and an intramural segment.

This is the first report of a coronary artery with an anomalous origin from the proper sinus resulting in ischemic events in a child. Transthoracic echocardiogram, computed tomogram, and coronary angiogram revealed that, although the left main coronary trunk originated from the left sinus, its ostium was displaced horizontally and was located near the commissure between the left and noncoronary valve cusps. Moreover, it was associated with an acute take off angle and an intramural segment, which are known contributing features for ischemia in cases of anomalous origin of a coronary artery from the wrong sinus. Surgical intervention, involving the unroofing procedure, was employed successfully to eliminate the ischemic events. At the latest follow up, no chest pain was reported and the transthoracic echocardiogram showed no stenosis of the neo-ostium. Even in a coronary artery that originates from the proper sinus, an abnormal ostial location could be associated with an acute takeoff angle and an intramural segment. This finding is extremely rare but entails the risk of ischemia and sudden death.