Clinical characteristics and electrophysiologic properties of SCN5A variants in fever-induced Brugada syndrome.

ABSTRACT:

Background

Brugada syndrome (BrS) is a severe inherited arrhythmia syndrome that can be unmasked by fever.

Methods

A multicentre clinical analysis was performed in 261 patients diagnosed with fever-induced BrS, including 198 (75.9%) and 27 (10.3%) patients who received next-generation genetic sequencing and epicardial arrhythmogenic substrate (AS) mapping, respectively.

Findings

In fever-induced BrS patients, pathogenic or likely pathogenic (P/LP) SCN5A variant carriers developed fever-induced BrS at a younger age, and more often in females and those of Caucasian descent. They exhibited significant electrophysical abnormalities, including a larger epicardial AS area, and more prolonged abnormal epicardial electrograms. During a median follow-up of 50.5 months (quartil

SUBMITTER: Chen GX 

PROVIDER: S-EPMC9768239 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature