Structural Heart Alterations in Brugada Syndrome: Is it Really a Channelopathy? A Systematic Review

J Clin Med. 2022 Jul 28;11(15):4406. doi: 10.3390/jcm11154406.

Abstract

Brugada syndrome (BrS) is classified as an inherited cardiac channelopathy attributed to dysfunctional ion channels and/or associated proteins in cardiomyocytes rather than to structural heart alterations. However, hearts of some BrS patients exhibit slight histologic abnormalities, suggesting that BrS could be a phenotypic variant of arrhythmogenic cardiomyopathy. We performed a systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses Statement (PRISMA) criteria. Our comprehensive analysis of structural findings did not reveal enough definitive evidence for reclassification of BrS as a cardiomyopathy. The collection and comprehensive analysis of new cases with a definitive BrS diagnosis are needed to clarify whether some of these structural features may have key roles in the pathophysiological pathways associated with malignant arrhythmogenic episodes.

Keywords: Brugada syndrome; endomyocardial biopsy; forensic pathology; histopathology; sudden cardiac death.

Publication types

  • Review