Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome
Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome
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DOI：
10.1001/jama.296.10.1249
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发表时间：
2006-09-13
影响因子：
120.7
通讯作者：
Zhang, Li
中科院分区：
文献类型：
--
作者：
Hobbs, Jenny B.;Peterson, Derick R.;Zhang, Li
遗传性长 QT 综合征 (LQTS) 心脏事件预测因素的背景分析主要将晕厥视为主要终点。尚未调查心脏骤停和心源性猝死的具体危险因素。 目的 确定临床怀疑患有 LQTS 的患者青春期期间与心脏骤停和心源性猝死相关的危险因素。 设计、设置和参与者 该研究纳入了来自国际长 QT 综合征登记处的 2772 名参与者，他们在 10 岁时还活着，并在青春期期间随访至 20 岁。登记注册于 1979 年开始在美国和欧洲的 5 个心脏病中心进行登记。 主要结果指标 中止心脏骤停或与 LQTS 相关的心源性猝死；随访至2005年2月15日结束。结果 81例患者出现心搏骤停，45例出现心源性猝死。 81 名曾发生过心脏骤停事件的患者中，有 9 名随后出现心脏性猝死。青春期心脏骤停或心源性猝死的重要独立预测因素包括近期晕厥、QTc 间期和性别。与过去10年没有发生晕厥事件的患者相比，2至10年前发生过1次或2次或以上晕厥（但最近2年内没有发生过）的患者的调整后风险比（HR）为2.7；对于危及生命的事件，（95% 置信区间 [CI]，1.3-5.7；P < .01）和调整后的 HR 分别为 5.8（95% CI，3.6-9.4；P < .001）；过去 2 年内发生 1 次晕厥发作的患者的调整后 HR 为 11.7 (95% CI, 7.0-19.5; P < .001)，过去 2 年内发生 2 次或以上晕厥发作的患者调整后 HR 为 18.1 (95% CI, 10.4-31.2; P < .001)。无论发生在 2 年前的事件如何，与 QTc 较短的患者相比，530 ms 或更长的 QTc 与风险增加相关（调整后 HR，2.3；95% CI，1.6-3.3；P < .001）。 10 至 12 岁之间的男性风险高于女性（HR，4.0；95% CI，1.8-9.2；P = .001），但 13 至 20 岁之间男性和女性之间没有显着风险差异。在过去 2 年内发生过晕厥的个体中，β 受体阻滞剂治疗可将风险降低 64%（HR，0.36；95% CI，0.18-0.72；P < .01）。 结论 在 LQTS 中，晕厥的时间和频率、QTc 延长和性别可预测青春期心脏骤停和心源性猝死的风险。在最近发生晕厥的患者中，β-受体阻滞剂治疗与风险降低相关。
Context Analysis of predictors of cardiac events in hereditary long-QT syndrome (LQTS) has primarily considered syncope as the predominant end point. Risk factors specific for aborted cardiac arrest and sudden cardiac death have not been investigated.Objective To identify risk factors associated with aborted cardiac arrest and sudden cardiac death during adolescence in patients with clinically suspected LQTS.Design, Setting, and Participants The study involved 2772 participants from the International Long QT Syndrome Registry who were alive at age 10 years and were followed up during adolescence until age 20 years. The registry enrollment began in 1979 at 5 cardiology centers in the United States and Europe.Main Outcome Measures Aborted cardiac arrest or LQTS-related sudden cardiac death; follow-up ended on February 15, 2005. Results There were 81 patients who experienced aborted cardiac arrest and 45 who had sudden cardiac death; 9 of the 81 patients who had an aborted cardiac arrest event experienced subsequent sudden cardiac death. Significant independent predictors of aborted cardiac arrest or sudden cardiac death during adolescence included recent syncope, QTc interval, and sex. Compared with those with no syncopal events in the last 10 years, patients with 1 or 2 or more episodes of syncope 2 to 10 years ago (but none in the last 2 years) had an adjusted hazard ratio (HR) of 2.7; (95% confidence interval [CI], 1.3-5.7; P < .01) and an adjusted HR of 5.8 (95% CI, 3.6-9.4; P < .001), respectively, for life-threatening events; those with 1 syncopal episodes in the last 2 years had an adjusted HR of 11.7 (95% CI, 7.0-19.5; P < .001) and those with 2 or more syncopal episodes in the last 2 years had an adjusted HR of 18.1 (95% CI, 10.4-31.2; P < .001). Irrespective of events occurring more than 2 years ago, QTc of 530 ms or longer was associated with increased risk (adjusted HR, 2.3; 95% CI, 1.6-3.3; P < .001) compared with those having a shorter QTc. Males between the ages of 10 and 12 years had higher risk than females (HR, 4.0; 95% CI, 1.8-9.2; P = .001), but there was no significant risk difference between males and females between the ages of 13 and 20 years. Among individuals with syncope in the past 2 years, beta-blocker therapy was associated with a 64% reduced risk (HR, 0.36; 95% CI, 0.18-0.72; P < .01).Conclusions In LQTS, the timing and frequency of syncope, QTc prolongation, and sex were predictive of risk for aborted cardiac arrest and sudden cardiac death during adolescence. Among patients with recent syncope, beta-blocker treatment was associated with reduced risk.