ABSTRACT
The association of sudden unexpected death with coronary artery anomalies has been limited to cases of anomalous origin of the left main coronary artery, either from the right sinus of Valsalva or from the main pulmonary artery. In contrast, anomalies involving the origin of the right coronary artery have been considered to be benign. Postmortem examination in a patient who died suddenly at 23 years of age disclosed that the right coronary artery originated anomalously from the left sinus of Valsalva. The findings in this patient: (1) constitute the first necropsy documentation of premature sudden unexpected death in a patient whose sole pathologic abnormality was anomalous origin of the right coronary artery; (2) have important implications regarding previously proposed mechanisms of sudden death due to anomalous origin of the left main coronary artery; and (3) support recent suggestions that markedly acute angulation of either coronary artery, even when located in the appropriate sinus of Valsalva, may predispose to sudden unexpected death.