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肥厚型心肌病心脏性猝死的高危因素及预防

潘玉惠 桑文涛 边圆 徐峰

潘玉惠, 桑文涛, 边圆, 徐峰. 肥厚型心肌病心脏性猝死的高危因素及预防[J]. 协和医学杂志, 2023, 14(3): 465-471. doi: 10.12290/xhyxzz.2022-0627
引用本文: 潘玉惠, 桑文涛, 边圆, 徐峰. 肥厚型心肌病心脏性猝死的高危因素及预防[J]. 协和医学杂志, 2023, 14(3): 465-471. doi: 10.12290/xhyxzz.2022-0627
PAN Yuhui, SANG Wentao, BIAN Yuan, XU Feng. Sudden Cardiac Death in Hypertrophic Cardiomyopathy: Hig-risk Factors and Prevention[J]. Medical Journal of Peking Union Medical College Hospital, 2023, 14(3): 465-471. doi: 10.12290/xhyxzz.2022-0627
Citation: PAN Yuhui, SANG Wentao, BIAN Yuan, XU Feng. Sudden Cardiac Death in Hypertrophic Cardiomyopathy: Hig-risk Factors and Prevention[J]. Medical Journal of Peking Union Medical College Hospital, 2023, 14(3): 465-471. doi: 10.12290/xhyxzz.2022-0627

肥厚型心肌病心脏性猝死的高危因素及预防

doi: 10.12290/xhyxzz.2022-0627
基金项目: 

国家重点研发计划 2020YFC1512700

山东省重点研发计划 2021ZLGX02

泰山学者攀登计划专家建设工程专项经费 tspd20181220

详细信息
    通讯作者:

    边圆, E-mail:bianyuan@sdu.edu.cn

    徐峰, E-mail:xufengsdu@126.com

  • 中图分类号: R459.7;R541.7+8

Sudden Cardiac Death in Hypertrophic Cardiomyopathy: Hig-risk Factors and Prevention

Funds: 

National Key Research and Development Program of China 2020YFC1512700

Key Research and Development Program of Shandong Province 2021ZLGX02

Taishan Pandeng Scholar Program of Shandong Province tspd20181220

More Information
  • 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。
    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
    下载: 导出CSV

    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
    下载: 导出CSV
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  • 收稿日期:  2022-10-31
  • 录用日期:  2023-02-07
  • 网络出版日期:  2023-02-20
  • 刊出日期:  2023-05-30

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