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PURPOSE: To investigate the pathologic substrates of sudden death in young competitive athletes. PATIENTS AND METHODS: Twenty-two cases of sudden death in young competitive athletes occurring in the Veneto region (northern Italy) in the period January 1979 to December 1989 were studied by postmortem examination. The athletes included 19 males and three females, ranging in age from 11 to 35 years (mean, 23 years). RESULTS: In 18 cases, sudden death occurred during (16 cases) or immediately after (two cases) a competitive sport activity. In 10 subjects, sudden death was apparently the first sign of disease. Postmortem examination disclosed that this fatality was due to arrhythmic cardiac arrest in 17 cases; among these, right ventricular cardiomyopathy, also known as 'right ventricular dysplasia,' was the most frequently encountered cardiovascular disease (six cases), followed by atherosclerotic coronary artery disease (four cases), conduction system pathology (three cases), anomalous origin of right coronary artery from the wrong aortic sinus (two cases), and mitral valve prolapse (two cases). In two athletes, the abrupt lethal complication was 'mechanical' and consisted of pulmonary embolism and rupture of the aorta; in three athletes, death was due to a cerebral cause. All athletes with right ventricular cardiomyopathy died during effort, and most had a history of palpitations and/or syncope. Whenever available, electrocardiographic (ECG) tracings showed inverted T waves in precordial leads and/or left bundle branch block ventricular arrhythmias. CONCLUSIONS: Clinicopathologic correlations indicate that in the Veneto region of Italy, right ventricular cardiomyopathy is not so rare among the cardiovascular diseases associated with the risk of arrhythmic cardiac arrest, and seems to account for the majority of cases of sudden death in young athletes; this disorder can be suspected during life on the basis of prodromal symptoms and ECG signs., The pathologic conditions leading to sudden death in young athletes were assessed by reviewing 22 cases. The deaths in occurred in 19 males and 3 females between January 1979 and December 1989. The ages of the athletes ranged from 11 to 35 years old. Sudden death occurred during an athletic activity in 16 cases, after the sports activity in two cases, and was the first sign of disease in 10 cases. Death resulted from cardiac arrest due to arrhythmia in 17 cases. These included six cases of right ventricular dysplasia, a heart condition characterized by abnormalities of the right heart ventricle; four cases of atherosclerotic coronary artery disease, characterized by fatty degeneration and thickening of the walls of the coronary arteries; three cases of abnormal conduction in the heart; two cases of defects in the physical structure of the right coronary artery; and two cases of mitral valve prolapse. The development of a blood clot in the lungs and rupture of the aorta occurred in two cases. Death was due to disorders affecting the brain in three athletes. All athletes with heart disease involving the right ventricle died during physical exertion and had a history of palpitations and/or fainting. These findings show that disease of the right ventricle is a common cause of sudden death among young athletes. This heart disorder is characterized by abnormalities in heart electrical activity and early symptoms, such as palpitations and fainting. (Consumer Summary produced by Reliance Medical Information, Inc.)