Brugada syndrome: a rare cause of sudden death in the young adult with a "normal" heart

BACKGROUND: Brugada syndrome is a rare condition causing sudden death in yo ung adults due to unexpected sudden-onset ventricular fibrillation. Diagnos is is based on the presence of an ST depression in the anteroseptal territo ry and a right branch block. No underlying dysrhythmic condition or arrhyth mogenic heart disease can be detected. CASE REPORT: A 15-year old boy with an uneventful past history experienced cardiocirculatory failure due to ventricular fibrillation during a sports c ompetition. Cardiac arrest developed and resuscitation was initially succes sful but death ensued due to cerebral anoxia. Characteristic electrocardiog raphic signs and the presence of similar anomalies in a brother and an aunt led to the diagnosis of Brugada syndrome. DISCUSSION: Brugada syndrome is a recently discovered hereditary condition with a probably underestimated prevalence. Systematic family studies have d emonstrated autosomal dominant inheritance. The characteristic electrocardi ographic anomalies can be transitory and may be unmasked by sensitivization tests. The only currently effective treatment is the implantable defibrill ator programmed to prevent sudden death by ventricular fibrillation.