Concealed Substrates in Brugada Syndrome: Isolated Channelopathy or Associated Cardiomyopathy?

ABSTRACT: Brugada syndrome (BrS) is an inherited autosomal dominant genetic disorder responsible for sudden cardiac death from malignant ventricular arrhythmia. The term "channelopathy" is nowadays used to classify BrS as a purely electrical disease, mainly occurring secondarily to loss-of-function mutations in the α subunit of the cardiac sodium channel protein Nav1.5. In this setting, arrhythmic manifestations of the disease have been reported in the absence of any apparent structural heart disease or cardiomyopathy. Over the last few years, however, a consistent amount of evidence has grown in support of myocardial structural and functional abnormalities in patients with BrS. In detail, abnormal ventricular dimensions, either systolic or diastolic dysfunctions, regional wall motion abnormalities,

SUBMITTER: Di Resta C 

PROVIDER: S-EPMC9602309 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature