Risk of syncope in family members who are genotype-negative for a family-associated long-QT syndrome mutation.

ABSTRACT:

Background

Current clinical diagnosis of long-QT syndrome (LQTS) includes genetic testing of family members of mutation-positive patients. The present study was designed to assess the clinical course of individuals who are found negative for the LQTS-causing mutation in their families.

Methods and results

Multivariate Cox proportional hazards model was used to assess the risk for cardiac events (comprising syncope, aborted cardiac arrest [ACA], or sudden cardiac death [SCD]) from birth through age 40 years among 1828 subjects from the LQTS Registry who were found negative for their family LQTS-causing mutation. The median QTc of study subjects was 423 ms (interquartile range, 402-442 ms). The cumulative probability of a first syncope through age 40 years was 15%. However, on

SUBMITTER: Barsheshet A 

PROVIDER: S-EPMC3690290 | biostudies-literature | 2011 Oct

REPOSITORIES: biostudies-literature