Risks of Ventricular Arrhythmia and Heart Failure in Carriers of RBM20 Variants.

ABSTRACT:

Background

Variants in RBM20 are reported in 2% to 6% of familial cases of dilated cardiomyopathy and may be associated with fatal ventricular arrhythmia and rapid heart failure progression. We sought to determine the risk of adverse events in RBM20 variant carriers and the impact of sex on outcomes.

Methods

Consecutive probands and relatives carrying RBM20 variants were retrospectively recruited from 12 cardiomyopathy units. The primary end point was a composite of malignant ventricular arrhythmia (MVA) and end-stage heart failure (ESHF). MVA and ESHF end points were also analyzed separately and men and women compared. Left ventricular ejection fraction (LVEF) contemporary to MVA was examined. RBM20 variant carriers with left ventricular systolic

SUBMITTER: Cannie DE 

PROVIDER: S-EPMC10581410 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature