BC Children's Hospital, Division of Cardiology, Department of Pediatrics, The University of British Columbia, Vancouver, British Columbia, Canada.
BC Children's Hospital, Division of Cardiology, Department of Pediatrics, The University of British Columbia, Vancouver, British Columbia, Canada; Center for Cardiovascular Innovation, Division of Cardiology, The University of British Columbia, Vancouver, Canada.
Heart Rhythm. 2024 Oct;21(10):1767-1776. doi: 10.1016/j.hrthm.2024.04.006. Epub 2024 Apr 7.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there is limited data on the outcomes of ICD use in children.
The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.
We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.
The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.
SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.
儿茶酚胺多形性室性心动过速（CPVT）尽管接受了药物治疗，仍可能导致心源性猝死（SCD）。因此，通常建议植入式心脏复律除颤器（ICD）。然而，关于儿童使用 ICD 的结果的数据有限。
本研究旨在比较 CPVT 患儿使用和不使用 ICD 的心律失常事件风险。
我们比较了 RYR2（兰尼碱受体 2）变异和表型阳性症状性 CPVT 患者的 SCD 风险，这些患者年龄小于 19 岁，在表型诊断时无心脏骤停史。主要结局是 SCD；次要结局是 SCD、心脏骤停或适当 ICD 电击的复合终点，无论是否伴有心律失常性晕厥。
这项研究纳入了 235 名患者，其中 73 名（31.1%）使用了 ICD，162 名（68.9%）未使用 ICD。在中位数为 8.0 年（四分位距 4.3-13.4 年）的随访期间，7 名患者（3.0%）发生了 SCD，其中 4 名（57.1%）未遵医嘱服药，且均未使用 ICD。使用 ICD 的患者发生次要复合结局的风险更高（无晕厥：风险比 5.85；95%置信区间 3.40-10.09；P<0.0001；有晕厥：风险比 2.55；95%置信区间 1.50-4.34；P=0.0005）。31 名使用 ICD 的患者（42.5%）经历了适当的电击，18 名（24.7%）经历了不适当的电击，21 名（28.8%）经历了器械相关并发症。
SCD 事件仅发生在未使用 ICD 的患者中，且主要发生在未接受最佳药物治疗的患者中。使用 ICD 的患者发生适当和不适当电击的风险较高，通过适当的设备编程可以降低这种风险。严重的 ICD 并发症很常见，需要权衡利弊。
