Author/Authors :
Goldenberg، نويسنده , , Ilan and Horr، نويسنده , , Samuel and Moss، نويسنده , , Arthur J. and Lopes، نويسنده , , Coeli M. and Barsheshet، نويسنده , , Alon and McNitt، نويسنده , , Scott and Zareba، نويسنده , , Wojciech and Andrews، نويسنده , , Mark L. and Robinson، نويسنده , , Jennifer L. and Locati، نويسنده , , Emanuela H. and Ackerman، نويسنده , , Michael J. and Benhorin، نويسنده , , Jesaia and Kaufman، نويسنده , , Elizabeth S. and Napolitano، نويسنده , , Carlo and Platonov، نويسنده , , Pyotr G. and Priori، نويسنده , , Silvia G. and Qi، نويسنده , , Ming and Schwartz، نويسنده , , Peter J. and Shimizu، نويسنده , , Wataru and Towbin، نويسنده , , Jeffrey A. and Vincent، نويسنده , , G. Michael and Wilde، نويسنده , , Arthur A.M. and Zhang، نويسنده , , Li، نويسنده ,
Abstract :
Objectives
tudy was designed to assess the clinical course and to identify risk factors for life-threatening events in patients with long-QT syndrome (LQTS) with normal corrected QT (QTc) intervals.
ound
t data regarding the outcome of patients with concealed LQTS are limited.
s
al and genetic risk factors for aborted cardiac arrest (ACA) or sudden cardiac death (SCD) from birth through age 40 years were examined in 3,386 genotyped subjects from 7 multinational LQTS registries, categorized as LQTS with normal-range QTc (≤440 ms [n = 469]), LQTS with prolonged QTc interval (>440 ms [n = 1,392]), and unaffected family members (genotyped negative with ≤440 ms [n = 1,525]).
s
mulative probability of ACA or SCD in patients with LQTS with normal-range QTc intervals (4%) was significantly lower than in those with prolonged QTc intervals (15%) (p < 0.001) but higher than in unaffected family members (0.4%) (p < 0.001). Risk factors ACA or SCD in patients with normal-range QTc intervals included mutation characteristics (transmembrane-missense vs. nontransmembrane or nonmissense mutations: hazard ratio: 6.32; p = 0.006) and the LQTS genotypes (LQTS type 1:LQTS type 2, hazard ratio: 9.88; p = 0.03; LQTS type 3:LQTS type 2, hazard ratio: 8.04; p = 0.07), whereas clinical factors, including sex and QTc duration, were associated with a significant increase in the risk for ACA or SCD only in patients with prolonged QTc intervals (female age >13 years, hazard ratio: 1.90; p = 0.002; QTc duration, 8% risk increase per 10-ms increment; p = 0.002).
sions
pe-confirmed patients with concealed LQTS make up about 25% of the at-risk LQTS population. Genetic data, including information regarding mutation characteristics and the LQTS genotype, identify increased risk for ACA or SCD in this overall lower risk LQTS subgroup.
