Division of Cardiology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
Korean J Intern Med. 2023 Jan;38(1):7-15. doi: 10.3904/kjim.2022.144. Epub 2022 Nov 10.
Hypertrophic cardiomyopathy (HCM) is a genetic disease associated with a risk of malignant ventricular tachyarrhythmias and sudden cardiac death (SCD). Assessment of the SCD risk is crucial for its clinical management, and there has been considerable interest in developing risk stratification strategies. An implantable cardioverter-defibrillator (ICD) is a life-saving treatment for patients with HCM who are at a high-risk of ventricular tachyarrhythmias and SCD. However, a substantial number of ICD recipients experience adverse effects arising from inappropriate device therapy and implant-related complications. This has led to numerous investigations of the risk of SCD and the indications for ICD implantation. American guidelines were recently updated to include new risk markers, including left ventricular systolic dysfunction, apical aneurysm, and extensive late gadolinium enhancement, while European guidelines recommend individualized estimated 5-year SCD risk assessment models. Studies evaluating other risk factors for SCD in patients with HCM have also been published. Drawing on recent guidelines and publications on clinical risk factors, we focus this review on updated risk assessments for SCD with ICD therapy in patients with HCM.
肥厚型心肌病（HCM）是一种与恶性室性心律失常和心源性猝死（SCD）风险相关的遗传性疾病。评估 SCD 风险对于其临床管理至关重要，因此人们对开发风险分层策略产生了浓厚的兴趣。植入式心脏复律除颤器（ICD）是一种挽救生命的治疗方法，适用于存在室性心动过速和 SCD 高风险的 HCM 患者。然而，相当数量的 ICD 接受者会出现因设备治疗不当和植入相关并发症引起的不良影响。这导致了对 SCD 风险和 ICD 植入适应证的大量研究。美国指南最近进行了更新，纳入了新的风险标志物，包括左心室收缩功能障碍、心尖部瘤和广泛的晚期钆增强，而欧洲指南建议个体化估计 5 年 SCD 风险评估模型。评估 HCM 患者 SCD 其他危险因素的研究也已发表。本综述基于最近关于临床危险因素的指南和出版物，重点关注 HCM 患者 ICD 治疗的 SCD 更新风险评估。
