背景:儿茶酚胺能多形性室性心动过速(CPVT)可能会导致心脏性猝死(SCD)。因此,植入式心律转复除颤器(ICD)通常被推荐.然而,关于儿童使用ICD的结果的数据有限.
目的:比较有和没有ICD的儿童CPVT患者发生心律失常事件的风险。
方法:我们比较了有或没有ICD的RYR2变异和表型阳性症状CPVT患者的SCD风险,年龄<19岁,表型诊断时无心脏骤停(SCA)病史。主要结果是SCD;次要结果是SCD的复合发病率,SCA,适当的ICD冲击,有/没有心律失常性晕厥。
结果:该研究包括235名患者,73(31.1%)有ICD,162(68.9%)没有ICD。中位随访时间为8.0年(IQR4.3-13.4),7例(3.0%)患者发生SCD,其中4例(57.1%)不符合药物治疗,且无ICD.ICD患者有两个次要复合结局的风险较高(无晕厥:HR5.85(CI3.40-10.09);p<0.0001;有晕厥:HR2.55(CI1.50-4.34);p=0.0005)。31名(42.5%)ICD患者经历了适当的电击,18(24.7%)不适当的冲击,21例(28.8%)器械相关并发症。
结论:SCD事件仅发生在无ICD组,那些没有接受最佳药物治疗的人。ICD患者发生适当和不适当电击的风险很高,这可以通过适当的设备编程来减少。严重的ICD并发症很常见,需要考虑ICD的风险与益处。
BACKGROUND: 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.
OBJECTIVE: The purpose of this
study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.