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摘要: 原发性肥厚型心肌病(HCM)是常染色体显性遗传性心肌病，是青年人发生心源性猝死(SCD)最常见的病因。SCD也是HCM最严重的并发症，在中国成年人群中的发生率为0.5%~2.0%。因HCM心肌细胞肥大，形成局限性心肌纤维化及电重构，成为发生恶性心律失常的主要发病基础，从而发生SCD。由于HCM本身的结构特点，需要早期关注及识别SCD的危险因素。除指南推荐的危险因素外，很多其他因素也与SCD显著相关，需进行综合评估。同时应根据危险因素和评估结果进行严密预防，评估植入式心律复律除颤器(ICD)植入的合理性。本文就HCM患者发生SCD的机制、危险因素及预防措施的最新进展进行综述。Abstract: Hypertrophic cardiomyopathy(HCM) is an autosomal dominant inherited cardiomyopathy and the most common cause of sudden cardiac death(SCD) in adults. SCD is also the most severe complication of HCM, with an incidence rate of 0.5% to 2.0% in the adult population in China. Due to hypertrophy of the HCM myocardial cells, localized myocardial fibrosis and electrical remodeling occur, which are the main pathological bases for the occurrence of malignant arrhythmias and subsequent SCD. Due to the structural characteristics of HCM itself, early attention and identification of risk factors for SCD are necessary. In addition to the risk factors recommended by guidelines, many other factors are significantly associated with SCD and require comprehensive evaluation. Based on the risk factors and assessment results, preventive measures for SCD and the rationality of implantable cardioverter-defibrillator(ICD) implantation should be evaluated. This article reviews the latest advances in the mechanism, risk factors, and preventive measures of SCD in patients with HCM.
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