Clinical aspects of the three major genetic forms of long QT syndrome (LQT1, LQT2, LQT3)

被引:29
作者
Kutyifa, Valentina [1 ]
Daimee, Usama A. [1 ]
McNitt, Scott [1 ]
Polonsky, Bronislava [1 ]
Lowenstein, Charles [2 ]
Cutter, Kris [1 ]
Lopes, Coeli [3 ]
Zareba, Wojciech [1 ]
Moss, Arthur J. [1 ]
机构
[1] Univ Rochester, Med Ctr, Cardiol Unit, Heart Res Follow Up Program, Rochester, NY 14642 USA
[2] Univ Rochester, Med Ctr, Dept Med, Rochester, NY 14642 USA
[3] Univ Rochester, Med Ctr, Cardiovasc Res Inst, Rochester, NY 14642 USA
基金
美国国家卫生研究院;
关键词
Long QT syndrome; outcomes; risk factors; IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR; CONGENITAL DEAF-MUTISM; BETA-BLOCKERS; SUDDEN DEATH; GENOTYPE; INTERVAL; REGISTRY; CHILDREN; THERAPY; AGE;
D O I
10.1111/anec.12537
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BackgroundA comprehensive report on the clinical course of the three major genotypes of the long QT syndrome (LQTS) in a large U.S. patient cohort is lacking. MethodsOur study consisted of 1,923 U.S. subjects from the Rochester-based LQTS Registry with genotype-positive LQT1 (n=879), LQT2 (n=807), and LQT3 (n=237). We evaluated the risk of a first cardiac event (syncope, aborted cardiac arrest, or sudden cardiac death, whichever occurred first) from birth through age 50years. Cox proportional hazards regression models incorporating clinical covariates were used to assess genotype-specific risk of cardiac events. ResultsFor all three genotypes, the cumulative probability of a first cardiac event increased most markedly during adolescence. Multivariate analysis identified proband status and QTc>500ms as predictors of cardiac events in all three genotypes, and males <14years and females >14years as predictors of cardiac events in LQT1 and LQT2 only. Beta-blockers significantly reduced the risk of cardiac events in LQT1 (HR: 0.49, p=.002) and LQT2 patients (HR: 0.48, p=.001). A trend toward beta-blocker benefit in reducing cardiac events was found in LQT3 females (HR: 0.32, p=.078), but not in LQT3 males (HR: 1.37, p=.611). ConclusionRisk factors and outcomes in LQTS patients varied by genotype. In all three genotypes, proband status and prolonged QTc were risk factors for cardiac events. Younger males and older females experienced increased risk in LQT1 and LQT2 only. Beta-blockers were most effective in reducing cardiac events in LQT1 and LQT2, with a potential benefit in LQT3 females.
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页数:7
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