Cardiac disease and risk of sudden death in the young: the burden of the phenomenon.

Sudden cardiovascular death is a rare but catastrophic event in young men and women throughout the world. Sudden death is difficult to study. Factors that need elucidation are (1) the definition of sudden death; (2) diagnosis of the cause of sudden death; (3) the true incidence of sudden death, and (4) age and gender of individuals being studied. The "burden" of sudden death is far-reaching and involves medical, emotional, and economic burdens on the family members at risk, the entire family of the victim, and society in general. The pathologist trying to evaluate a case of sudden death also has a burden to make the correct diagnosis, especially since the cause of the sudden death may determine risk to the victim's family members. Sudden death is difficult to prevent since it may be the first and last manifestation of the cardiovascular disease. Also, paradoxically, the greatest number of deaths occurs in "low-risk" groups. The most common causes of cardiovascular deaths in the young are cardiomyopathy, coronary anomaly, obstructive coronary artery disease, myocarditis, valvular disease, channelopathy, and aortic disease leading to dissection or rupture. Many sudden deaths in the young occur during or shortly after exercise. Appropriate pre-participation screening of competitive athletes can reduce the incidence of sudden cardiovascular death in the young. Which measures to try to prevent these rare deaths are indicated and/or cost effective is a matter of discussion and controversy.