Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN 55407, USA.
J Am Coll Cardiol. 2013 Apr 9;61(14):1527-35. doi: 10.1016/j.jacc.2013.01.037.
The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM).
HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients.
A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia.
Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age.
In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.
本研究旨在确定植入式心脏复律除颤器（ICD）在肥厚型心肌病（HCM）儿童和青少年中的疗效。
HCM 是年轻人猝死的最常见原因。过去十年中，ICD 可用于 HCM，已证明其具有预防猝死的潜力，主要在成年患者中。
组建了一个多中心国际 ICD 植入注册中心，该中心于 1987 年至 2011 年间在 224 名在美国、加拿大、欧洲和澳大利亚的 22 家转诊和非转诊机构接受评估的、具有高度猝死风险的 HCM 非相关儿童和青少年中植入（n = 224）。这些患者因一级（n = 188）或二级（n = 36）预防而植入 ICD，一级预防是在心脏骤停后进行，二级预防是在心脏骤停后进行。
在平均 4.3 ± 3.3 年的时间内，224 名患者中有 43 名（19%）因室性心动过速或心室颤动而接受 ICD 治疗。ICD 干预率为每年 4.5%，二级预防后每年 14.0%，基于危险因素的一级预防每年 3.1%（5 年累积概率为 17%）。从植入到首次适当放电的平均时间为 2.9 ± 2.7 年（范围为 8.6 年）。一级预防放电终止室性心动过速或心室颤动的速率在植入 1、2 或≥3 个危险因素的患者中相同（12/88 [14%]、10/71 [14%]和 4/29 [14%]，p = 1.00）。在经历一级预防干预的患者中，最常见的危险因素是（单独或与其他标志物联合）极度左心室肥厚（17/26 [65%]）。ICD 相关并发症，特别是不适当的电击和导联故障，在 91 名（41%）患者中于 17 ± 5 岁时发生。
在高危儿科 HCM 队列中，ICD 干预终止危及生命的室性心动过速非常频繁。极度左心室肥厚与适当的干预最相关。设备并发症的发生率为该年龄组的 ICD 决策增加了一个复杂性指标。
