Sudden death in young athletes is a rare and devastating event. The most frequent causes of sudden death in this group are hypertrophic cardiomyopathy, anomalous coronary artery, and Marfan syndrome. This article describes the physiology of exercise and the pathophysiology associated with the most frequent causes as well as some of the less common causes of sudden death in young athletes. Identification of youth who may be at risk should be a priority for health care practitioners, and suggestions for recognizing and counseling affected athletes are therefore included

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