Department of Medicine, Brigham and Women's Hospital, Cardiovascular Medicine Division, 75 Francis Street, Boston, MA 02115, USA.
Department of Cardiology, Herlev-Gentofte Hospital, University Hospital of Copenhagen, Gentofte Hospitalsvej 1, Hellerup 2900, Denmark.
Eur Heart J. 2021 Oct 7;42(38):3932-3944. doi: 10.1093/eurheartj/ehab598.
Risk stratification algorithms for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) and regional differences in clinical practice have evolved over time. We sought to compare primary prevention implantable cardioverter defibrillator (ICD) implantation rates and associated clinical outcomes in US vs. non-US tertiary HCM centres within the international Sarcomeric Human Cardiomyopathy Registry.
We included patients with HCM enrolled from eight US sites (n = 2650) and five non-US (n = 2660) sites and used multivariable Cox-proportional hazards models to compare outcomes between sites. Primary prevention ICD implantation rates in US sites were two-fold higher than non-US sites (hazard ratio (HR) 2.27 [1.89-2.74]), including in individuals deemed at high 5-year SCD risk (≥6%) based on the HCM risk-SCD score (HR 3.27 [1.76-6.05]). US ICD recipients also had fewer traditional SCD risk factors. Among ICD recipients, rates of appropriate ICD therapy were significantly lower in US vs. non-US sites (HR 0.52 [0.28-0.97]). No significant difference was identified in the incidence of SCD/resuscitated cardiac arrest among non-recipients of ICDs in US vs. non-US sites (HR 1.21 [0.74-1.97]).
Primary prevention ICDs are implanted more frequently in patients with HCM in US vs. non-US sites across the spectrum of SCD risk. There was a lower rate of appropriate ICD therapy in US sites, consistent with a lower-risk population, and no significant difference in SCD in US vs. non-US patients who did not receive an ICD. Further studies are needed to understand what drives malignant arrhythmias, optimize ICD allocation, and examine the impact of different ICD utilization strategies on long-term outcomes in HCM.
肥厚型心肌病（HCM）患者发生心源性猝死（SCD）的风险分层算法和临床实践中的地域差异随时间而演变。我们旨在比较国际肌小节性人类心肌病注册研究中，美国与非美国三级 HCM 中心的一级预防植入式心脏转复除颤器（ICD）植入率和相关临床结局。
我们纳入了来自 8 个美国（n=2650 例）和 5 个非美国（n=2660 例）站点的 HCM 患者，并使用多变量 Cox 比例风险模型比较了站点之间的结局。美国站点的一级预防 ICD 植入率是非美国站点的两倍（风险比（HR）2.27[1.89-2.74]），包括根据 HCM 风险-SCD 评分（HR 3.27[1.76-6.05]）被认为存在高 5 年 SCD 风险（≥6%）的患者。美国 ICD 受者的传统 SCD 危险因素也较少。在 ICD 受者中，美国站点 ICD 治疗的适当率明显低于非美国站点（HR 0.52[0.28-0.97]）。在美国和非美国站点，未接受 ICD 的患者中 SCD/复苏性心脏骤停的发生率无显著差异（HR 1.21[0.74-1.97]）。
与非美国站点相比，美国各 SCD 风险谱的 HCM 患者中，一级预防 ICD 的植入更为频繁。美国站点的 ICD 治疗适当率较低，这与低危人群相符，且未接受 ICD 的美国和非美国患者的 SCD 发生率无显著差异。需要进一步研究以了解是什么驱动恶性心律失常，优化 ICD 分配，并检查不同 ICD 使用策略对 HCM 患者长期结局的影响。
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