Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest
Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest
复制标题
DOI：
10.1093/eurheartj/ehz309
复制
发表时间：
2019-09-14
影响因子：
39.3
通讯作者：
Wilde, Arthur A.
中科院分区：
文献类型：
--
作者：
van der Werf, Christian;Lieve, Krystien V.;Wilde, Arthur A.
目的在儿茶酚胺能多态性室性心动过速（CPVT）患者中，植入式心律转复除颤器（ICD）电击有时无效，甚至可能引发致命的电风暴。我们评估了在CPVT患者中放置icd的疗效和并发症，这些患者在未确诊和未经治疗的情况下出现心脏骤停（SCA）前哨事件。方法和结果我们分析了136例SCA患者，这些患者随后被诊断为CPVT，导致开始指导治疗，包括β受体阻滞剂，flecainide和/或左心交感神经去支配。植入ICD 79例（58.1%）。该研究的主要结局是心源性猝死（SCD）。次要结局是SCD、SCA、适当的ICD电击和晕厥的综合结局。中位随访4.8年后，有3例（3.8%）有ICD的患者发生了SCD，无ICD的患者没有发生SCD （P = 0.1）。37例有ICD的患者（46.8%）发生SCD、SCA或适当的ICD休克，9例无ICD的患者（15.8%）发生SCD休克（P < 0.0001）。19例（24.7%）患者发生不适当的ICD电击，22例（28.9%）患者发生其他与设备相关的并发症。结论：在先前未确诊的CPVT患者中，出现SCA， ICD与生存率的提高无关。相反，ICD与适当的ICD电击率和不适当的ICD电击率以及其他与设备相关的并发症有关。严格遵守无ICD的指导治疗可以为这些患者提供足够的保护，而不会出现ICD的所有潜在缺点。
Aims In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), implantable cardioverter-defibrillator (ICD) shocks are sometimes ineffective and may even trigger fatal electrical storms. We assessed the efficacy and complications of ICDs placed in patients with CPVT who presented with a sentinel event of sudden cardiac arrest (SCA) while undiagnosed and therefore untreated.Methods and results We analysed 136 patients who presented with SCA and in whom CPVT was diagnosed subsequently, leading to the initiation of guideline-directed therapy, including beta-blockers, flecainide, and/or left cardiac sympathetic denervation. An ICD was implanted in 79 patients (58.1%). The primary outcome of the study was sudden cardiac death (SCD). The secondary outcomes were composite outcomes of SCD, SCA, appropriate ICD shocks, and syncope. After a median follow-up of 4.8 years, SCD had occurred in three patients (3.8%) with an ICD and none of the patients without an ICD (P = 0.1). SCD, SCA, or appropriate ICD shocks occurred in 37 patients (46.8%) with an ICD and 9 patients (15.8%) without an ICD (P < 0.0001). Inappropriate ICD shocks occurred in 19 patients (24.7%) and other device-related complications in 22 patients (28.9%).Conclusion In previously undiagnosed patients with CPVT who presented with SCA, an ICD was not associated with improved survival. Instead, the ICD was associated with both a high rate of appropriate ICD shocks and inappropriate ICD shocks along with other device-related complications. Strict adherence to guideline-directed therapy without an ICD may provide adequate protection in these patients without all the potential disadvantages of an ICD.