The congenital long-QT syndromes (LQTS) were initially described approximately 50 years ago. The principal events are syncope, seizures, and ventricular tachycardia, characteristically torsade de pointes. Most often, this arrhythmia is self terminating, producing a syncopal episode; however, LQTS is responsible for a significant proportion of sudden cardiac deaths (SCDs) in young people without structural heart disease, estimated to have an incidence of approximately 1 in 2500 and causing thousands of deaths annually.

Long QT:_Who's_at_Risk_for_SCD