High Efficacy of β-Blockers in Long-QT Syndrome Type 1 Contribution of Noncompliance and QT-Prolonging Drugs to the Occurrence of β-Blocker Treatment "Failures"
High Efficacy of β-Blockers in Long-QT Syndrome Type 1 Contribution of Noncompliance and QT-Prolonging Drugs to the Occurrence of β-Blocker Treatment "Failures"
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DOI：
10.1161/circulationaha.108.772533
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发表时间：
2009-01-20
期刊：
影响因子：
37.8
通讯作者：
Zhang, Li
中科院分区：
文献类型：
--
作者：
Vincent, G. Michael;Schwartz, Peter J.;Zhang, Li
背景 β 受体阻滞剂对 1 型长 QT 综合征的疗效良好，但报道不一，尽管使用 β 受体阻滞剂治疗，但心脏事件的原因尚未确定。 方法和结果 - 这是一项回顾性研究，涉及 216 名基因型长 QT 综合征 1 型患者，接受 β 受体阻滞剂治疗并随访中位时间 10 年，了解有关心脏事件的细节。在使用 β 受体阻滞剂之前，157 名患者 (73%) 发生心脏事件，中位年龄为 9 岁，其中 26 名患者 (12%) 出现心脏骤停 (CA)。 17名患者使用了QT间期延长药物； 17 人中有 9 人 (53%) 患有 CA，而 199 名非使用者中有 17 人患有 CA (8.5%；比值比，12.0；95% 置信区间，4.1 至 35.3；P < 0.001)。使用 β 受体阻滞剂后，75% 的人无症状，心脏事件显着减少 (P < 0.001)，每人中位事件计数（四分位数 1 至 3）为 0（0 至 1）。 12 名患者 (5.5%) 发生 CA/猝死，但 12 名患者中有 11 名 (92%) 不依从 (n = 8)，在事件发生时正在服用 QT 延长药物 (n = 2)，或两者兼而有之 (n = 1)。与不服用 QT 延长药物的不依从患者相比，不服用 QT 延长药物的依从性患者发生 CA/猝死的风险显着降低（比值比，0.03；95% 置信区间，0.003 至 0.22；P = 0.001）。 26 名在使用 β 受体阻滞剂之前患有 CA 的患者中，没有一人在服用 β 受体阻滞剂后发生 CA/猝死。 结论：β 受体阻滞剂对于 1 型长 QT 综合征非常有效，应在诊断时、最好在青少年前给药。 β-受体阻滞剂不依从和使用延长 QT 的药物是几乎所有危及生命的“β-受体阻滞剂失败”的原因。 β 受体阻滞剂适合无症状患者和从未接受过 CA 或 β 受体阻滞剂治疗的患者。在此类患者中常规植入心脏除颤器似乎并不合理。 （流通。2009 年；119：215-221。）
Background-beta-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite beta-blocker therapy have not been ascertained.Methods and Results-This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with beta-blocker and followed up for a median time of 10 years. Before beta-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; P < 0.001). After beta-blocker, 75% were asymptomatic, and cardiac events were significantly reduced (P < 0.001), with a median event count (quartile 1 to 3) per person of 0 ( 0 to 1). Twelve patients (5.5%) suffered CA/sudden death, but 11 of 12 (92%) were noncompliant (n = 8), were on a QT-prolonging drug (n = 2), or both (n = 1) at the time of the event. The risk for CA/sudden death in compliant patients not taking QT-prolonging drugs was dramatically less compared with noncompliant patients on QT-prolonging drugs (odds ratio, 0.03; 95% confidence interval, 0.003 to 0.22; P = 0.001). None of the 26 patients with CA before beta-blocker had CA/sudden death on beta-blockers.Conclusions-beta-Blockers are extremely effective in long-QT syndrome type 1 and should be administered at diagnosis and ideally before the preteen years. beta-Blocker noncompliance and use of QT-prolonging drug are responsible for almost all life-threatening "beta-blocker failures." beta-Blockers are appropriate therapy for asymptomatic patients and those who have never had a CA or beta-blocker therapy. Routine implantation of cardiac defibrillators in such patients does not appear justified. (Circulation. 2009;119:215-221.)