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

OBJECTIVES Sudden Death The purpose of this study is to characterize the cl inical profile and identify Clinical markers that would enable the detectio n during life of anomalous coronary artery origin from the wrong aortic sin us (with course between the aorta and pulmonary trunk) in young competitive athletes. BACKGROUND Congenital coronary artery anomalies are not uncommonly associat ed with sudden death in young athletes, the catastrophic event probably pro voked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, si nce anomalous coronary artery origin is amenable to surgical treatment, tim ely clinical identification is crucial. METHODS Because of the paucity of available data characterizing the clinica l profile of wrong sinus coronary artery malformations, we reviewed two lar ge registries comprised of young competitive athletes who died suddenly, as sembled consecutively in the U.S. and Italy. RESULTS We reported 27 sudden deaths in young athletes, identified solely a t 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 ex ertion on the athletic field. Fifteen athletes (55%) had no clinical cardio vascular manifestations or testing during life. However, in the remaining 1 2 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premo nitory symptoms had occurred in 10, including syncope in four (exertional i n three and recurrent in two, 3 to 24 months before death) and chest pain i n five (exertional in three, all single episodes, less than or equal to 24 months before death). All cardiovascular tests were within normal limits, i ncluding 12-lead electrocardiogram (ECG) pattern tin 9/9), stress ECG with maximal exercise tin 6/6) and left Ventricular wall motion and cardiac dime nsions by two-dimensional echocardiography (in 2/2). CONCLUSIONS With regard to congenital coronary artery anomalies of wrong ao rtic sinus origin in young competitive athletes, 1) standard testing with E CG under resting or exercise conditions is unlikely to provide clinical evi dence of myocardial ischemia and would not be reliable as screening tests i n large athletic populations, 2) premonitory cardiac symptoms not uncommonl y occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncop e or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive a thletes. (C) 2000 by the American College of Cardiology.