Brugada syndrome

Abstract

In 1992, the manuscript " Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome " was published in the Journal of the American College of Cardiology [1]. This publication described eight individuals with a common phenotype: resuscitated from sudden cardiac death (SCD) caused by documented ventricular fibrillation (VF), showing a characteristic ST-segment elevation in the right precordial leads (Fig. 11.1) in a structurally normal heart. Nowadays, this entity is known as Brugada Syndrome (BrS). In the last 20 years, major advances in clinical and mechanistic knowledge have provided very valuable information about the disease, but remaining questions still propel a large research activity on the subject. In this chapter, we review the present knowledge, on clinical, genetic, and molecular features of BrS.