Atrial Fibrillation in Long QT Syndrome by Genotype.

P. Platonov, S. McNitt, B. Polonsky, S. Rosero, W. Zareba
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引用次数: 25

Abstract

BACKGROUND Long QT syndrome (LQTS) is caused by the abnormal function of ion channels, which may also affect atrial electrophysiology and be associated with the risk of atrial fibrillation (AF). However, large-scale studies of AF risk among patients with LQTS and its relation to LQTS manifestations are lacking. We aimed to assess the risk of AF and its relationship to the LQTS genotype and the long-term prognosis in patients with LQTS. METHODS Genotype-positive patients with LQTS (784 LQT1, 746 LQT2, and 233 LQT3) were compared with 2043 genotype-negative family members. Information on the occurrence of AF was based on physician-reported ECG-verified events. Multivariate Cox proportional hazards regression analyses were performed for ages 0 to 60 and after 60 years (reflecting an early and late-onset of AF) to assess the risk of incident AF by genotype and the relationship of AF to the risk of cardiac events defined as syncope, documented torsades de pointes, and aborted cardiac arrest or sudden cardiac death. RESULTS In patients followed from birth to 60 years of age, patients with LQT3 had an increased risk of AF compared with genotype-negative family members (hazard ratio=6.62; 95% CI, 2.04-21.49; P<0.001), while neither LQT1 nor LQT2 demonstrated increased AF risk. After the age of 60 years, patients with LQT2 had significantly lower risk of AF compared with genotype-negative controls (hazard ratio=0.07; 95% CI, 0.01-0.53, P=0.011). AF was a significant predictor of cardiac events in patients with LQT3 through the age of 60 (hazard ratio=5.38; 95% CI, 1.17-24.82; P=0.031). CONCLUSIONS Our data demonstrate an increased risk of early age AF in patients with LQT3 and also indicate a protective effect of the LQT2 genotype in it's association with a decreased risk of AF after the age of 60.
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长QT综合征心房颤动的基因型分析。
背景长QT综合征(LQTS)是由离子通道功能异常引起的,它也可能影响心房电生理,并与心房颤动(AF)的风险相关。然而,缺乏LQTS患者AF风险及其与LQTS表现之间关系的大规模研究。我们的目的是评估房颤的风险及其与LQTS基因型的关系以及LQTS患者的长期预后。方法将基因型阳性LQTS患者(784例LQT1、746例LQT2、233例LQT3)与基因型阴性LQTS家族成员2043例进行比较。房颤发生的信息是基于医生报告的心电图证实的事件。对0 - 60岁和60岁后(反映早发性和晚发性房颤)进行多变量Cox比例风险回归分析,以评估基因型房颤发生的风险,以及房颤与心脏事件风险的关系,心脏事件定义为晕厥、记录的椎体扭曲、流产性心脏骤停或心源性猝死。结果在出生至60岁随访期间,LQT3患者发生房颤的风险高于基因型阴性的家庭成员(风险比=6.62;95% ci, 2.04-21.49;P<0.001),而LQT1和LQT2均未显示AF风险增加。60岁后,与基因型阴性对照相比,LQT2患者发生房颤的风险显著降低(风险比=0.07;95% ci, 0.01-0.53, p =0.011)。AF是LQT3患者60岁前心脏事件的重要预测因子(危险比=5.38;95% ci, 1.17-24.82;P = 0.031)。结论:我们的数据表明LQT3患者早期房颤风险增加,同时也表明LQT2基因型与60岁后房颤风险降低相关。
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