A Prospective Study of Sudden Cardiac Death among Children and Young Adults

In 490 cases of sudden cardiac death identified over a 3-year period (annual incidence of 1.3 per 100,000), causes were found in 60% through conventional autopsy, and a clinically relevant cardiac gene mutation was found in 27% of the remaining cases in which genetic testing was performed. Sudden cardiac death among children and young adults is a devastating event for the family and wider community. Coronary artery disease is the predominant cause of sudden cardiac death in older persons, 1 whereas among persons 1 to 35 years of age, sudden cardiac death is more often caused by structural heart disease, including hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, myocarditis, and primary arrhythmogenic disorders (such as the congenital long-QT syndrome, the Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia). 2 – 5 Many of these cardiac causes of sudden cardiac death among children and young adults have an underlying genetic basis. . . .