Brugada syndrome and arrhythmogenic cardiomyopathy: overlapping disorders of the connexome?

ABSTRACT

Arrhythmogenic cardiomyopathy (ACM) and Brugada syndrome (BrS) are inherited diseases characterized by an increased risk for arrhythmias and sudden cardiac death. Possible overlap between the two was suggested soon after the description of BrS. Since then, various studies focusing on different aspects have been published pointing to similar findings in the two diseases. More recent findings on the structure of the cardiac cell-cell junctions may unite the pathophysiology of both diseases and give further evidence to the theory that they may in part be variants of the same disease spectrum. In this review, we aim to summarize the studies indicating the pathophysiological, genetic, structural, and electrophysiological overlap between ACM and BrS.