Heart and Vascular Institute, Johns Hopkins University, Baltimore, MD, USA.
Cardiovascular Genetics Centre, Montreal Heart Institute, Université de Montréal, 5000 rue Bélanger, Montréal, Québec H1T 1C8, Canada.
Eur Heart J. 2024 Feb 16;45(7):538-548. doi: 10.1093/eurheartj/ehad799.
Implantable cardioverter-defibrillators (ICDs) are critical for preventing sudden cardiac death (SCD) in arrhythmogenic right ventricular cardiomyopathy (ARVC). This study aims to identify cross-continental differences in utilization of primary prevention ICDs and survival free from sustained ventricular arrhythmia (VA) in ARVC.
This was a retrospective analysis of ARVC patients without prior VA enrolled in clinical registries from 11 countries throughout Europe and North America. Patients were classified according to whether they received treatment in North America or Europe and were further stratified by baseline predicted VA risk into low- (<10%/5 years), intermediate- (10%-25%/5 years), and high-risk (>25%/5 years) groups. Differences in ICD implantation and survival free from sustained VA events (including appropriate ICD therapy) were assessed.
One thousand ninety-eight patients were followed for a median of 5.1 years; 554 (50.5%) received a primary prevention ICD, and 286 (26.0%) experienced a first VA event. After adjusting for baseline risk factors, North Americans were more than three times as likely to receive ICDs {hazard ratio (HR) 3.1 [95% confidence interval (CI) 2.5, 3.8]} but had only mildly increased risk for incident sustained VA [HR 1.4 (95% CI 1.1, 1.8)]. North Americans without ICDs were at higher risk for incident sustained VA [HR 2.1 (95% CI 1.3, 3.4)] than Europeans.
North American ARVC patients were substantially more likely than Europeans to receive primary prevention ICDs across all arrhythmic risk strata. A lower rate of ICD implantation in Europe was not associated with a higher rate of VA events in those without ICDs.
植入式心脏复律除颤器（ICD）对于预防心律失常性右心室心肌病（ARVC）导致的心脏性猝死（SCD）至关重要。本研究旨在比较 ARVC 患者在不同大陆地区接受一级预防 ICD 治疗和免于持续性室性心律失常（VA）的生存情况。
这是一项回顾性分析，纳入了来自欧洲和北美 11 个国家的临床注册登记的 ARVC 患者，这些患者均无既往 VA 病史。根据患者治疗地区（北美或欧洲）进行分类，并根据基线预测的 VA 风险进一步分为低危组（<10%/5 年）、中危组（10%-25%/5 年）和高危组（>25%/5 年）。评估 ICD 植入和免于持续性 VA 事件（包括适当的 ICD 治疗）的生存情况。
共纳入 1098 例患者，中位随访时间为 5.1 年；554 例（50.5%）患者接受了一级预防 ICD 治疗，286 例（26.0%）患者发生了首次 VA 事件。在校正基线危险因素后，北美人接受 ICD 治疗的可能性是欧洲人的三倍以上[风险比（HR）3.1（95%置信区间[CI]2.5, 3.8)]，但发生持续性 VA 的风险仅略有增加[HR 1.4（95% CI 1.1, 1.8)]。未接受 ICD 治疗的北美人发生持续性 VA 的风险更高[HR 2.1（95% CI 1.3, 3.4)]，高于欧洲人。
与欧洲人相比，ARVC 患者在所有心律失常风险分层中，北美人接受一级预防 ICD 治疗的可能性明显更高。欧洲 ICD 植入率较低与未植入 ICD 患者的 VA 事件发生率较高无关。
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