Christensen Emma Basse, Vissing Christoffer Rasmus, Silajdzija Elvira, Mills Helen Lamiokor, Thune Jens Jakob, Larroudé Charlotte, Bosselmann Helle Skovmand, Philbert Berit Thornvig, Raja Anna Axelsson, Christensen Alex Hørby, Bundgaard Henning
The Capital Region's Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital-Rigshospitalet, Copenhagen, Denmark
The Capital Region's Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital-Rigshospitalet, Copenhagen, Denmark.
Heart. 2025 May 23;111(12):575-582. doi: 10.1136/heartjnl-2024-325020.
Treatment with implantable cardioverter-defibrillators (ICDs) effectively prevents sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Identifying patients most likely to benefit from a primary prevention ICD remains challenging. We aimed to investigate the long-term incidence of ICD therapy in patients with HCM according to SCD-risk at baseline.
The study retrospectively included all patients with HCM treated with an ICD for primary or secondary prevention between 1995 and 2022 in Eastern Denmark. Medical records for each patient were evaluated. Patients were stratified into risk groups according to the European Society of Cardiology HCM Risk-SCD score.
We included 208 patients (66% male) with HCM and an ICD for primary (78%) or secondary prevention (22%). During a median 10-year follow-up, 66 patients (32%) received appropriate ICD therapy (antitachycardia pacing and/or shock), while 20 (10%) received inappropriate therapy. Patients with an ICD implanted for secondary prevention were almost twice as likely to receive appropriate therapy compared with patients with an ICD implanted for primary prevention (47% vs 28%, p=0.02). The 5-year cumulative incidences of appropriate shock therapy were 17% in patients with a high HCM Risk-SCD score, 16% in patients with an intermediate-risk score and 6% in patients with a low-risk score. A high-risk score was associated with higher cumulative incidence of appropriate shock therapy (p=0.012).
One-third of patients with HCM treated with an ICD experienced appropriate ICD therapy. The HCM-Risk SCD score adequately distinguished between low-risk and high-risk patients among those who underwent ICD implantation. Further improvements of risk-tools are needed to identify a larger proportion of the two-thirds of patients who did not benefit from ICD implantation after 10 years of observation.
植入式心脏复律除颤器（ICD）治疗可有效预防肥厚型心肌病（HCM）患者的心源性猝死（SCD）。确定最有可能从一级预防ICD中获益的患者仍然具有挑战性。我们旨在根据基线时的SCD风险调查HCM患者接受ICD治疗的长期发生率。
本研究回顾性纳入了1995年至2022年在丹麦东部接受ICD一级或二级预防治疗的所有HCM患者。对每位患者的病历进行了评估。根据欧洲心脏病学会HCM风险-SCD评分将患者分层为风险组。
我们纳入了208例HCM患者（66%为男性），他们接受了ICD一级预防（78%）或二级预防（22%）。在中位10年的随访期间，66例患者（32%）接受了适当的ICD治疗（抗心动过速起搏和/或电击），而20例患者（10%）接受了不适当的治疗。接受二级预防植入ICD的患者接受适当治疗的可能性几乎是接受一级预防植入ICD患者的两倍（47%对28%，p=0.02）。HCM风险-SCD评分高的患者适当电击治疗的5年累积发生率为17%，中风险评分患者为16%，低风险评分患者为6%。高风险评分与适当电击治疗的累积发生率较高相关（p=0.012）。
接受ICD治疗的HCM患者中有三分之一经历了适当的ICD治疗。HCM风险-SCD评分在接受ICD植入的患者中能够充分区分低风险和高风险患者。在经过10年观察后，需要进一步改进风险工具，以识别未从ICD植入中获益的三分之二患者中的更大比例。
