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Sudden Cardiac Death in Hypertrophic Cardiomyopathy: Hig-risk Factors and Prevention
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摘要: 肥厚型心肌病(hypertrophic cardiomyopathy, HCM)是最常见的遗传性心肌病之一, 心脏性猝死(sudden cardiac death, SCD)是其最严重的临床结局。及早识别、早期预防可推迟或避免心脏骤停的发生, 因此, 明确HCM的猝死高危因素对于患者预后至关重要。目前公认的HCM患者SCD高危因素主要包括SCD家族史、非持续性室性心动过速、严重左心室肥厚、不明原因的晕厥、左心室射血分数＜50%的终末期HCM、任何大小的左心室心尖室壁瘤以及广泛晚期钆增强等, 预防措施主要包括药物治疗、外科室间隔心肌切除术、室间隔心肌酒精消融术和植入式心脏复律除颤器等。本文围绕识别HCM引发SCD的危险因素以及预防策略, 就HCM诊断、治疗、预防方面的最新研究进展作一综述, 以期为改善HCM患者的临床结局提供指导。Abstract: Hypertrophic cardiomyopathy(HCM) is one of the most common hereditary cardiomyopathy, the most serious outcome of which is sudden cardiac death(SCD). Early recognition and prevention may delay or avoid the onset of cardiac arrest. Therefore, it is important to identify the high-risk factors of SCD in patients with HCM and prevent cardiac arrest. At present, the recognized high risk factors mainly include family history of sudden death, non-sustained ventricular tachycardia, severe left ventricular hypertrophy, unexplained syncope, end-stage HCM with left ventricular ejection fraction < 50%, left ventricular systolic dysfunction of any size, and extensive late gadolinium enhancement. The main preventive measures involvemedical treatment, surgical septal myectomy, alcohol septal ablation, and implantable cardiac defibrillator. This article mainly focuses on the identification of high-risk factors and prevention strategies of SCD caused by HCM, and reviews the latest progress of diagnosis, treatment and prevention of HCM, to provide guidance for prevention of SCD caused by HCM.
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Key words:
- hypertrophic cardiomyopathy /
- sudden cardiac death /
- high-risk factors /
- prevention
作者贡献：潘玉惠负责查阅文献、撰写论文；桑文涛负责修订论文；边圆、徐峰负责终审及校对。利益冲突：所有作者均声明不存在利益冲突 -
表 1 HCM患者SCD的高危因素
高危因素 具体描述 SCD家族史 明确判断或可能归因于HCM，一般认为发生于≥1级或可能的其他近亲中，年龄≤50岁 不明原因的晕厥 在过去6个月内出现一次不明原因的意识丧失，除外神经源性晕厥、血管源性晕厥等，无明确病因引起的晕厥史 NVST 连续≥3次室性期前收缩波形，频率至少为100次/min，持续时间＜30 s 严重左心室肥厚 在左心室的任何位置测量的最大厚度≥30 mm 左心室收缩功能不全 超声心动图显示LVEF＜50%的收缩功能障碍，通常发生在无流出梗阻的有症状患者中 左心室心尖室壁瘤 通常通过超声心动图识别，与大小无关，与邻近区域“边缘区”心肌瘢痕有关，通常伴有心尖肥大和恶性室性快速性心律失常 LGE 弥漫性和广泛的LGE分布，可量化或通过目视检查估计，约占左心室质量的15%，单独或与其他危险标志物相关，是室性快速心律失常的可能来源 HCM：肥厚型心肌病；SCD: 心脏性猝死；NVST：非持续性室性心动过速；LVEF：左心室射血分数；LGE：广泛晚期钆增强 
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表 2 HCM患者SCD的预防
治疗方式 具体描述 药物治疗 β受体阻滞剂 目前仍是HCM的一线用药，主要用于静息时或刺激后出现左心室流出道梗阻，是否能够降低SCD风险仍存在争议 维拉帕米 静息时或刺激后出现左心室流出道梗阻但无法耐受β受体阻滞剂或有禁忌证，是否能够降低SCD风险仍存在争议 Mavacamten 新型靶向药，可改善HCM患者NYHA心功能分级、运动后左心室流出道压力阶差梯度，且具有良好的安全性和耐受性，目前尚未发现可改善SCD结局 非药物治疗 外科室间隔心肌切除术 临床治疗HOCM的金标准，可改善90%以上HOCM患者的临床症，显著降低SCD风险，远期生存率接近正常人 ASA 可有效控制患者症状，降低猝死率，但远期预后尚不清楚 ICD 高危人群应植入ICD，是目前预防SCD最有效的措施 HCM、SCD：同表 1；NYHA：纽约心脏病协会；HOCM：肥厚型梗阻性心肌病；ASA：室间隔心肌酒精消融术；ICD：植入式心脏复律除颤器
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