Delayed depolarization and histologic abnormalities underlie the Brugada syndrome

Pacing Clin Electrophysiol. 2023 Feb;46(2):172-181. doi: 10.1111/pace.14650. Epub 2023 Jan 10.

Abstract

Brugada syndrome (BrS) is a controversial disease whose pathophysiology is still far from being fully understood. Unlike other cardiological disorders, a definite etiology has not yet been established so that it could be summarized under two main chapters: "functional" or "organic", "repolarization" or "depolarization" disorder. Despite initial descriptions leaned towards the organic substrate and delayed depolarization features, functional and repolarization theories have attracted most of the Cardiological attention for many years. Data from electrocardiography, endocavitary tracings, electroanatomic mapping and histopathology, however, demonstrated that BrS is mainly characterized by structural myocardial changes mostly at the right ventricular outflow tract (RVOT), but also at the right ventricle (RV) and by delayed conduction at the same sites. Conduction disorders at different levels may also be present and identify patients at high risk for major arrhythmic events. The aim of the present review is to provide the current state of art of the pathophysiology of BrS, focusing on electro-vectorcardiography and electrophysiological features, histopathology, echocardiography, and cardiac magnetic resonance imaging (CMRI).

Keywords: Brugada syndrome; SCN5A; organic substrate; sudden cardiac death; ventricular fibrillation.

Publication types

  • Review

MeSH terms

  • Brugada Syndrome* / diagnosis
  • Cardiac Conduction System Disease
  • Electrocardiography / methods
  • Heart
  • Humans
  • Myocardium / pathology