Congenital atresia of the orifice of the left coronary artery and its main stem.

A 13-year-old black male who died suddenly as a result of atresia of the left coronary ostium and left main coronary artery is presented. An association between this anomaly and supravalvar aortic stenosis is observed. The literature is reviewed and 14 similar cases are compared. Presentation of this anomaly is variable, as is survival and may be attributed to the development of an adequate collateral circulation.