BACKGROUND: In Europe, sudden cardiac death (SCD) is one of the most common 
causes of death. Although sudden cardiac death usually happens in older people, 
5% to 10% of the affected individuals are young and apparently healthy. Sudden 
death in infants, children, and young adults is relatively rare, with an 
incidence of 1 to 5 per 100 000 persons per year. Nonetheless, up to 7000 
asymptomatic children die in the USA each year, almost half of them without any 
warning signs or symptoms.
METHOD: Selective literature review.
RESULTS: Although structural cardiovascular abnormalities explain most cases of 
sudden cardiac death in young people, the cause of death remains unexplained 
after autopsy in 10% to 30% of cases. Potentially lethal ion channel disorders 
(channelopathies) such as the long QT syndromes (LQTS), catecholaminergic 
polymorphic ventricular tachycardia (CPVT), and the Brugada syndrome (BrS) may 
account for at least one-third of these unexplained cases. Most of these 
diseases are hereditary with autosomal-dominant transmission, i.e., there is a 
50% chance that the children of affected individuals will be affected 
themselves.
CONCLUSIONS: Post-mortem genetic screening for sequence variations in cardiac 
ion channel genes has become an important forensic tool for elucidating the 
cause of sudden cardiac death. Moreover, it allows the identification of other 
family members bearing the previously undiagnosed gene defect, who can then 
undergo a cardiological evaluation if indicated by their clinical history.