Brugada syndrome

Brugada syndrome (BrS) has originally been described as an autosomal-dominant inherited arrhythmic disorder characterized by ST elevation with successive negative T wave in the right precordial leads without structural cardiac abnormalities. Patients are at risk for sudden cardiac death (SCD) due to ventricular fibrillation (VF). Since 1953, the ECG pattern similar to coved-type ST-segment elevation was reported as a normal variant in the healthy population or related to VF with structural abnormality, but as a distinct disease entity, Brugada and Brugada were the first to report 8 patients with VF, right bundle branch block, and ST-segment elevation on 12-lead ECG. Later, structural changes were reported in an explanted heart and by biopsy specimens in a small number of cases, potentially opening up the discussion of who actually described this disease entity first. Besides this dispute, BrS has been the subject of a number of controversies with regard to its pathophysiology; the prognosis of asymptomatic individuals and the best way to establish their risk; and the causal role of genetic variants, including the role of sodium channel mutations. On the basis of the current knowledge acquired in the past 2 decades, these topics and others related to exciting new treatment options are addressed in this review.