预防高性心肌病的突然死亡的范式
Martin S Maron1, Ethan J Rowin1, Barry J Maron1
1Lahey Hospital and Medical Center, HCM Center, Burlington, Massachusetts.
The American journal of cardiology
|February 17, 2024
概括
超性心肌病 (HCM) 现在是可以治疗的,植入式心脏转换器-除器显著降低了突然心脏死亡 (SCD) 的风险. 风险分层有助于向符合条件的患者推预防性除器植入体.
科学领域:
- 心脏病学 心脏病学
- 遗传学 遗传学 是一个
- 预防医学 预防医学
背景情况:
- 增高性心肌病变 (HCM) 是一种常见的遗传性心脏病.
- 从历史上看,HCM的预后很差,是年轻人突然心脏死亡 (SCD) 的主要原因.
- 最近的进展已经将HCM转变为可以通过改进的管理策略来治疗的疾病.
研究的目的:
- 突出了解HCM从无法治愈的疾病转变为可治疗的疾病的转变.
- 强调可植入心脏转换器-除器 (ICD) 对管理HCM中SCD风险的影响.
- 讨论目前用于预防性ICD植入的风险分层方法.
主要方法:
- 审查临床实践和HCM管理中的治疗进展.
- 对可植入心脏转换器-除器在预防SCD中的作用的分析.
- 评估基于指导方针的风险分层算法,包括包含心脏磁共振成像 (CMR) 的算法.
- 传统风险标记策略与用于ICD决策的较新的数学风险得分的比较.
主要成果:
- 通过终止腹腔动脉节律失常症,ICD已使HCM中SCD的初级预防成为可能,使年死亡率降低了10倍以上,至0.5%.
- 基于指南的风险分层,包括临床因素和CMR发现,有助于推预防性ICD.
- 该ACC/AHA风险标志物策略显示95%的灵敏度用于识别需要适当的ICD治疗的患者,尽管植入后往往需要数年时间.
- ESC数学SCD风险评分的灵敏度较低 (33%),但可能会减少设备过度处理.
结论:
- 现在,HCM是一种可治疗的疾病,由于ICD等进步,其结果得到了显著改善.
- 有效的风险分层是指导预防性ICD植入高风险SCD的HCM患者的关键.
- 共享决策,包括医生判断和患者因素,对于最佳管理HCM和SCD预防至关重要.
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