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

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.

SCN5A channelopathy: arrhythmia, cardiomyopathy, epilepsy and beyond

“…In addition to primary electrical disorders, SCN5A mutations have also been associated with cardiac structural alterations such as myocardial fibrosis and (dilated) cardiomyopathy [60][61][62][63][64]. In BrS patients, ventricular hypertrophy has been reported in addition to increased collagen content and fibrosis, particularly in the subepicardium of the RVOT [64].…”

“…In addition to primary electrical disorders, SCN5A mutations have also been associated with cardiac structural alterations such as myocardial fibrosis and (dilated) cardiomyopathy [60][61][62][63][64]. In BrS patients, ventricular hypertrophy has been reported in addition to increased collagen content and fibrosis, particularly in the subepicardium of the RVOT [64]. Furthermore, familial forms of dilated cardiomyopathy (DCM) have been reported in patients with SCN5A mutations, often presenting in combination with conduction disease, atrial arrhythmias and/or fibrillation [62,65].…”

Brugada syndrome unmasked by dengue fever

“…The gene involved is SCN5A which encodes the alpha portion of the sodium channels in cardiac muscles 6 . There's not enough evidence to connect this disease with structural changes in the heart 5 …”

Brugada Syndrome Unmasked by Dengue Fever

“…Serum electrolytes, urea, and creatinine were all within the normal range. Brugada syndrome is an inherited sodium channel defect that gives rise to fatal ventricular arrhythmia and sudden death (5). Brugada disease is an autosomal dominant inherited disease that runs in families, however, more than 60% of cases are sporadic.…”

“…The gene involved is SCN5A which encodes the alpha portion of the sodium channels in cardiac muscles. (6) There's not enough evidence to connect this disease with structural changes in the heart (5).…”