Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study.

ABSTRACT:

Background

Risk stratification in patients with type 3 long-QT syndrome (LQT3) by clinical and genetic characteristics and effectiveness of β-blocker therapy has not been studied previously in a large LQT3 population.

Methods

The study population included 406 LQT3 patients with 51 sodium channel mutations; 391 patients were known to be event free during the first year of life and were the focus of our study. Clinical, electrocardiographic, and genetic parameters were acquired for patients from 7 participating LQT3 registries. Cox regression analysis was used to evaluate the independent contribution of clinical, genetic, and therapeutic factors to the first occurrence of time-dependent cardiac events (CEs) from age 1 to 41 years.

Results

Of the 391 patients, 118 (41 ma

SUBMITTER: Wilde AA 

PROVIDER: S-EPMC5030177 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature