[The Brugada syndrome. A predicted sudden juvenile death]

This review deals with the clinical, basic and genetic aspects of recently highlighted form of idiopathic ventricular fibrillation known as the Brugada syndrome. The available data suggest that the Brugada syndrome is a primary electrical disease resulting in abnormal electrophysiological activity in the right ventricular epicardium. Diagnosis is based on the presence of an ST elevation in the anteroseptal territory and a right branch block. No underlying dysarrhythmic condition or arrhythmogenic heart disease can be detected. The available data suggest that less of the action potential dome in the right ventricular epicardium but not endocardium underlies the ST segment elevation seen in the Brugada syndrome and that electrical heterogeneity within right ventricular epicardium leads to the development of closely coupled premature ventricular contractions via a phase 2 reentrant mechanism that then precipitates ventricular tachycardia/ventricular fibrillation. Brugada syndrome is a recently discovered hereditary condition with a probably underestimated prevalence. Systematic family studies have demonstrated an autosomal dominant inheritance. The characteristic electrocardiographic anomalies can be transitory and may be unmasked by sensitization tests. The only currently treatment is the implantable defibrillator programmed to prevent sudden death by ventricular fibrillation and it is indicated in symptomatic patients and should be considered in asymptomatic patients in whom ventricular tachycardia/ventricular fibrillation is inducible by electrophysiologic study.