Brugada syndrome: Clinical and genetic aspects

After its recognition in 19921 as a distinct clinical entity, Brugada syndrome has increasingly been recognized worldwide as an important cause of sudden cardiac death (SCD) at a young age, in the absence of structural cardiac abnormalities. Patients affected with Brugada syndrome are at risk for SCD from fast polymorphic ventricular tachycardia (VT)/ventricular fibrillation (VF), especially at rest.2 Brugada syndrome is characterized by a typical electrocardiographic (ECG) pattern consisting of ST segment elevation in the right precordial leads and in leads positioned in the upper intercostal spaces,3-5 (Figure 32-1). The large number of case reports and clinical/experimental studies lately published about Brugada syndrome indicate its increasing weight and interest for its still not completely known aspects, such as the underlying pathophysiological mechanism, its genetic background, and its prognosis and treatment.