Sudden Death as a Complication of Anomalous Left Coronary Origin From the Anterior Sinus of Valsalva: A Not-So-Minor Congenital Anomaly

Abstract

Both coronary arteries arising as a single or double vessel from the same sinus of Valsalva has been considered a minor congenital anomaly not affecting longevity. There are rare case reports in the literature of sudden death in young males with this anomaly of coronary origin. We have reviewed 51 such cases from the Armed Forces Institute of Pathology congenital heart disease accessions. There were 33 patients in whom both coronaries arose from the anterior sinus of Valsalva either as a single or double vessel and 18 in whom they arose from the left coronary sinus of Valsalva. Out of the 33 patients in whom the coronaries arose from the anterior sinus of Valsalva, 9 (27.3%) died sudden, unexplained deaths. There were no sudden unexplained deaths among the patients in whom both coronaries arose from the left sinus of Valsalva. It is evident that where the left coronary artery does not pass acutely posterior and leftward between the pulmonary artery and the aorta there is no risk of sudden death. All patients who died suddenly were male. The suggested mechanism for sudden death in these cases is that the acute leftward passage of the coronary artery along the aortic wall causes the entrance into the left coronary svstem to be slit-like. Under circumstances of increased cardiac activity with increased expansion of the pulmonary artery and aorta with exercise, there is stretching of the left coronary artery and a flap-like closure of the orifice of the left coronary with sudden, fatal myocardial ischemia. We also present the first case where this anomaly was recognized in a 14-year-old boy and a surgical attempt was made to correct the problem by creating a non-collapsible funnel-like opening into the left coronary artery.