Risk of life-threatening cardiac events among patients with long QT syndrome and multiple mutations.

ABSTRACT:

Background

Patients with long QT syndrome (LQTS) who harbor multiple mutations (i.e. ≥ 2 mutations in ≥ 1 LQTS-susceptibility gene) may experience increased risk for life-threatening cardiac events.

Objectives

The present study was designed to compare the clinical course of LQTS patients with multiple mutations to those with a single mutation.

Methods

The risk for life-threatening cardiac events (comprising aborted cardiac arrest, implantable defibrillator shock, or sudden cardiac death) from birth through age 40 years, by the presence of multiple vs. single mutations, was assessed among 403 patients from the LQTS Registry.

Results

Patients with multiple mutations (n=57) exhibited a longer QTc at enrollment compared with those with a single mutation (mean ± SD:

SUBMITTER: Mullally J 

PROVIDER: S-EPMC3690288 | biostudies-literature | 2013 Mar

REPOSITORIES: biostudies-literature