关于植入式心脏除颤器治疗有症状的儿茶酚胺能多形性室性心动过速患儿的国际多中心队列研究。

IF 8.3 2区材料科学 Q1 MATERIALS SCIENCE, MULTIDISCIPLINARY

ACS Applied Materials & Interfaces Pub Date : 2024-10-01 DOI:10.1016/j.hrthm.2024.04.006

{"title":"关于植入式心脏除颤器治疗有症状的儿茶酚胺能多形性室性心动过速患儿的国际多中心队列研究。","authors":"","doi":"10.1016/j.hrthm.2024.04.006","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div><span>Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause </span>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.</div></div><div><h3>Objective</h3><div>The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.</div></div><div><h3>Methods</h3><div>We compared the risk of SCD in patients with <em>RYR2</em><span> (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.</span></div></div><div><h3>Results</h3><div>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; <em>P</em> < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50–4.34; <em>P</em> = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":5,"journal":{"name":"ACS Applied Materials & Interfaces","volume":null,"pages":null},"PeriodicalIF":8.3000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia\",\"authors\":\"\",\"doi\":\"10.1016/j.hrthm.2024.04.006\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><div><span>Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause </span>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.</div></div><div><h3>Objective</h3><div>The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.</div></div><div><h3>Methods</h3><div>We compared the risk of SCD in patients with <em>RYR2</em><span> (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.</span></div></div><div><h3>Results</h3><div>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; <em>P</em> < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50–4.34; <em>P</em> = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.</div></div><div><h3>Conclusion</h3><div>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.</div></div>\",\"PeriodicalId\":5,\"journal\":{\"name\":\"ACS Applied Materials & Interfaces\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":8.3000,\"publicationDate\":\"2024-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"ACS Applied Materials & Interfaces\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1547527124023233\",\"RegionNum\":2,\"RegionCategory\":\"材料科学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MATERIALS SCIENCE, MULTIDISCIPLINARY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"ACS Applied Materials & Interfaces","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1547527124023233","RegionNum":2,"RegionCategory":"材料科学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MATERIALS SCIENCE, MULTIDISCIPLINARY","Score":null,"Total":0}

引用次数: 0

摘要

背景儿茶酚胺能多形性室性心动过速（CPVT）尽管可以通过药物治疗，但仍可能导致心脏性猝死（SCD）。因此，通常建议使用植入式心律转复除颤器（ICD）。方法我们比较了 RYR2（雷诺丁受体 2）变异型患者和表型阳性无症状 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病史。主要结果是 SCD；次要结果是 SCD、心脏骤停或适当的 ICD 电击与或不与心律失常性晕厥的复合终点。结果该研究共纳入 235 例患者，其中 73 例有 ICD（31.1%），162 例无 ICD（68.9%）。中位随访时间为 8.0 年（四分位数间距为 4.3-13.4 年），7 名患者（3.0%）发生了 SCD，其中 4 名患者（57.1%）不遵医嘱用药，没有人使用 ICD。使用 ICD 的患者发生两种次要综合结果的风险都更高（无晕厥：危险比 5.85；95% 置信区间 3.40-10.09；P <；.0001；有晕厥：危险比 2.55；95% 置信区间 1.50-4.34；P = .0005）。31名ICD患者（42.5%）经历了适当电击，18名（24.7%）经历了不适当电击，21名（28.8%）经历了设备相关并发症。使用 ICD 的患者发生适当和不适当电击的风险很高，但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，因此需要考虑 ICD 的风险与益处。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

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.

Conclusion

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.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

ACS Applied Materials & Interfaces 工程技术-材料科学：综合

CiteScore

16.00

自引率

6.30%

发文量

4978

审稿时长

1.8 months

期刊介绍： ACS Applied Materials & Interfaces is a leading interdisciplinary journal that brings together chemists, engineers, physicists, and biologists to explore the development and utilization of newly-discovered materials and interfacial processes for specific applications. Our journal has experienced remarkable growth since its establishment in 2009, both in terms of the number of articles published and the impact of the research showcased. We are proud to foster a truly global community, with the majority of published articles originating from outside the United States, reflecting the rapid growth of applied research worldwide.