ISKANDAR, E. G., and P. D. THOMPSON. Exercise-Related Sudden Death due to an Unusual Coronary Artery Anomaly. Med. Sci. Sports Exerc., Vol. 36, No. 2, pp. 180-182, 2004. Coronary artery anomalies are, after hypertrophic cardiomyopathy, the second most common cause of exercise-related sudden cardiac death in young American athletes. These anomalies have been associated with myocardial ischemia, arrhythmia, and sudden death during exercise. A 14-yr-old male with no previous abnormal medical history collapsed during soccer practice and was successfully resuscitated without defibrillation. An extensive cardiac workup did not reveal any abnormalities. Two weeks later, he experienced a cardiac arrest while running and could not be resuscitated. Autopsy demonstrated an acute angle take-off of the left main coronary artery and a transverse slit-like opening with a fibrous cushion, which created a kink near its origin. This case report illustrates the difficulty in diagnosing coronary artery anomalies in general, and acute angle take-off and ostial ridges in particular.
Coronary artery anomalies are found in approximately 0.3% of autopsy examinations and can be detected in nearly 1% of patients undergoing coronary angiography (1,2). The most common congenital coronary artery anomaly associated with sudden cardiac death during exercise in young athletes is origin of the artery from a site other than the usual sinus of Valsalva (8). Additional congenital coronary anomalies that have been associated with myocardial ischemia and sudden cardiac death include congenitally small arteries, an intramyocardial course or coronary bridging, an acute angled take-off from the appropriate sinus, and valve-like ridges of the coronary ostium. Acute angle take-off and ostial abnormalities are difficult, if not impossible, to diagnose during life. Coronary artery anomalies are, after hypertrophic cardiomyopathy, the second most common cause of exercise-related cardiac death in young American athletes, account for approximately 14% of such events, and should be included in the differential diagnosis of exercise-related collapse (5,10). The present report describes an athlete who died during exercise due to an acute angle take-off with ostial ridging of his left main coronary artery. This anomaly was not detected despite a premortem cardiac workup for exercise-related syncope and highlights the difficulty of making this diagnosis during life.