Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.

OBJECTIVES The purpose of this study is to characterize the clinical profile and identify clinical markers that would enable the detection during life of anomalous coronary artery origin from the wrong aortic sinus (with course between the aorta and pulmonary trunk) in young competitive athletes. BACKGROUND Congenital coronary artery anomalies are not uncommonly associated with sudden death in young athletes, the catastrophic event probably provoked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, since anomalous coronary artery origin is amenable to surgical treatment, timely clinical identification is crucial. METHODS Because of the paucity of available data characterizing the clinical profile of wrong sinus coronary artery malformations, we reviewed two large registries comprised of young competitive athletes who died suddenly, assembled consecutively in the U.S. and Italy. RESULTS We reported 27 sudden deaths in young athletes, identified solely at autopsy and due to either left main coronary artery from the right aortic sinus (n = 23) or right coronary artery from the left sinus (n = 4). Each athlete died either during (n = 25) or immediately after (n = 2) intense exertion on the athletic field. Fifteen athletes (55%) had no clinical cardiovascular manifestations or testing during life. However, in the remaining 12 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premonitory symptoms had occurred in 10, including syncope in four (exertional in three and recurrent in two, 3 to 24 months before death) and chest pain in five (exertional in three, all single episodes, < or =24 months before death). All cardiovascular tests were within normal limits, including 12-lead electrocardiogram (ECG) pattern (in 9/9), stress ECG with maximal exercise (in 6/6) and left ventricular wall motion and cardiac dimensions by two-dimensional echocardiography (in 2/2). CONCLUSIONS With regard to congenital coronary artery anomalies of wrong aortic sinus origin in young competitive athletes, 1) standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests in large athletic populations, 2) premonitory cardiac symptoms not uncommonly occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive athletes.