Risk of death in the long QT syndrome when a sibling has died
Risk of death in the long QT syndrome when a sibling has died
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DOI：
10.1016/j.hrthm.2008.02.029
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发表时间：
2008-06-01
期刊：
影响因子：
5.5
通讯作者：
Zhang, Li
中科院分区：
文献类型：
--
作者：
Kaufman, Elizabeth S.;McNitt, Scott;Zhang, Li
背景：兄弟姐妹猝死被认为与长QT综合征(LOTS)死亡风险增加有关。本研究旨在验证兄弟姐妹猝死是LOTS患者死亡或流产心脏骤停的危险因素的假设。方法我们调查了国际长QT登记中所有先证者以及一级和二级亲属从出生到40岁的QTC>；=0.45QT；S。协变量包括兄弟姐妹死亡、非处方药、年龄性别、晕厥、植入性心律转复除颤器和β-受体阻滞剂治疗。终点是(1)严重事件(ACA、LQTS相关死亡)和(2)任何心脏事件(晕厥、ACA或LQTS相关死亡)。结果在1915名受试者中，270人有兄弟姐妹死亡。严重事件213例，心脏事件829例。更多有兄弟姐妹死亡史的受试者接受了β-受体阻滞剂治疗。兄弟姐妹死亡与ACA或LQTS相关死亡的风险不显著相关，但与晕厥风险增加相关。QTC>；=0.53 S(危险比2.5，P
BACKGROUND Sudden death of a sibling is thought to be associated with greater risk of death in long QT syndrome (LOTS). However, there is no evidence of such an association.OBJECTIVE This study sought to test the hypothesis that sudden death of a sibling is a risk factor for death or aborted cardiac arrest (ACA) in patients with LOTS.METHODS We examined all probands and first-degree and second-degree relatives in the International Long QT Registry from birth to age 40 years with QTc >= 0.45 s. Covariates included sibling death, OTc, gender by age, syncope, and implantable cardioverter-defibrillator (ICD) and beta-blocker treatment. End points were (1) severe events (ACA, LQTS-related death) and (2) any cardiac event (syncope, ACA, or LQTS-related death).RESULTS Of 1915 subjects, 270 had a sibling who died. There were 213 severe events and 829 total cardiac events. More subjects with history of sibling death received beta-blocker therapy. Sibling death was not significantly associated with risk of ACA or LQTS-related death, but was associated with increased risk of syncope. QTc >= 0.53 s (hazard ratio 2.5, P