Early repolarization syndrome: is it always benign?

Early repolarization syndrome is a well-recognized idiopathic electrocardiographic phenomenon characterized by prominent J wave and ST-segment elevation predominantly in left precordial leads. The syndrome shares remarkable cellular, ionic, and electrocardiographic similarities with the Brugada syndrome and idiopathic ventricular fibrillation (a variant of the Brugada syndrome with ST-segment elevation in inferior leads). Although early repolarization syndrome is considered a benign entity, its arrhythmogenic potential still remains unknown. We report the case of a 39-year-old male with a family history of sudden death and an electrocardiogram consistent with early repolarization syndrome. Diagnostic dilemmas are discussed.