Long-term follow-up of patients with long-QT syndrome treated with β-blockers and continuous pacing
Long-term follow-up of patients with long-QT syndrome treated with β-blockers and continuous pacing
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DOI：
10.1161/01.cir.100.24.2431
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发表时间：
1999-12-14
期刊：
影响因子：
37.8
通讯作者：
Scheinman, MM
中科院分区：
文献类型：
--
作者：
Dorostkar, PC;Eldar, M;Scheinman, MM
长QT综合征与心源性猝死有关。已提出将P-受体阻滞剂和起搏治疗联合用于治疗耐药患者。本研究的目的是总结我们的长期经验，与长QT syndrome.Methods和Results-A的患者共37例特发性长QT syndrome的综合治疗组成的连续心脏起搏和最大耐受性β受体阻滞剂治疗，并随访6.3+/-4.6年（平均+/-SD）。该组包括32名女性和5名男性患者，平均年龄为31.6岁。平均起搏频率为82+/-7 bpm（范围：60 - 100 bpm）。随访时，3例患者记录了起搏器故障引起的复发症状。4例患者在随访期间死亡：2例青少年停止β受体阻滞剂治疗，1例患者在接受联合治疗时突然死亡，1例患者死于无关原因。此外，3例患者复苏心脏骤停，而在综合治疗，1例患者反复，适当的植入式心律转复除颤器放电follow-up. Conclusions，因为28的37例患者仍然没有症状与β受体阻滞剂治疗和连续起搏，综合治疗似乎提供合理的，长期的控制，这一高风险群体。然而，猝死和流产猝死的发生率（所有患者中为24%，依从性患者中为17%）强烈建议使用“备用”除颤器，特别是在不依从的青少年患者中。然而，植入式心律转复除颤器治疗可能与易感患者的复发性休克有关。
Background-The long-QT syndrome is associated with sudden cardiac death. Combination of P-blocker and pacing therapy has been proposed for treatment of drug-resistant patients. The purpose of this study was to summarize our long-term experience with combined therapy in patients with long-QT syndrome.Methods and Results-A total of 37 patients with idiopathic long-QT syndrome were treated with combined therapy consisting of continuous cardiac pacing and maximally tolerated beta-blocker therapy and followed up for 6.3+/-4.6 years (mean+/-SD). The group consisted of 32 female and 5 male patients with a mean age of 31.6 years. The mean paced rate was 82+/-7 bpm (range, 60 to 100 bpm). On follow-up, recurrent symptoms caused by pacemaker malfunction were documented in 3 patients. Four patients died during the follow-up period: 2 adolescents stopped beta-blocker therapy, 1 patient died suddenly while treated with combined therapy, and 1 patient died of unrelated causes. In addition, 3 patients had resuscitated cardiac arrest while on combined therapy, and 1 patient had repeated, appropriate implantable cardioverter-defibrillator discharges on follow-up.Conclusions-Because 28 of 37 patients remain without symptoms with beta-blocker therapy and continuous pacing, combined therapy appears to provide reasonable, long-term control for this high-risk group. However, the incidence of sudden death and aborted sudden death (24% in all patients and 17% in compliant patients) strongly suggests the use of a "back-up" defibrillator, particularly in noncompliant adolescent patients. Implantable cardioverter-defibrillator therapy, however, may be associated with recurrent shocks in susceptible patients.