Risk stratification and treatment of brugada syndrome

The Brugada syndrome (BS) is a hereditary disease characterized by typical electrocardiographic alterations (elevation of the ST segment in right precordial leads) that affects young individuals without structural heart disease, predisposing them to ventricular arrhythmias and sudden death (SD). Several genetic mutations of different subunits of the sodium, calcium and potassium channel have been involved. The majority of patients with BS remain asymptomatic; however, the most frequent symptoms are syncope and/or SD secondary to polymorphic ventricular tachycardia (PVT) or ventricular fibrillation (VF). Electrocardiographic manifestations of the BS are typically dynamic and occasionally only become apparent after the administration of a sodium channel blocker or with fever. Risk stratification is mainly based on symptoms and the surface electrocardiogram. However, in asymptomatic patients, risk evaluation is still controversial and requires further studies. This review provides an updated summary of the BS from the point of view of genetic, clinical manifestations, risk stratification and management.