Mutation and gender-specific risk in type 2 long QT syndrome: implications for risk stratification for life-threatening cardiac events in patients with long QT syndrome.

ABSTRACT:

Background

Men and women with type 2 long QT syndrome (LQT2) exhibit time-dependent differences in the risk for cardiac events. We hypothesized that data regarding the location of the disease-causing mutation in the KCNH2 channel may affect gender-specific risk in LQT2.

Objective

This study sought to risk-stratify LQT2 patients for life-threatening cardiac events based on clinical and genetic information.

Methods

The risk for life-threatening cardiac events from birth through age 40 years (comprising aborted cardiac arrest [ACA] or sudden cardiac death [SCD]) was assessed among 1,166 LQT2 male (n = 490) and female (n = 676) patients by the location of the LQTS-causing mutation in the KCNH2 channel (prespecified in the primary analysis as pore-loop vs. non-pore-loop).<

SUBMITTER: Migdalovich D 

PROVIDER: S-EPMC4028036 | biostudies-literature | 2011 Oct

REPOSITORIES: biostudies-literature