左心室静脉功能障碍的过敏心肌病:来自SHaRe注册的见解
Peter Marstrand1,2, Larry Han3, Sharlene M Day4
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (P.M., N.K.L., C.Y.H.).
Circulation
|April 2, 2020
概括
左心室静脉功能障碍 (HCM- LVSD) 影响8% 的患者. 75%的患者经历不良事件,而35%的患者在8.4年内面临复合后果.
科学领域:
- 心脏病学
- 遗传学
- 心脏衰竭的研究
背景情况:
- 左心室静脉功能障碍 (HCM-LVSD) 的过高心肌病被定义为排气分数<50%.
- 由于HCM-LVSD的罕见性,预后和自然史仍然不明.
研究的目的:
- 描述HCM-LVSD的自然史.
- 确定HCM-LVSD的预后和事件发展的预测因素.
主要方法:
- 利用11个国际专科中心 (SHaRe注册) 的6793名HCM患者的数据.
- 使用Cox的比例风险模型来分析结果和风险因素.
主要成果:
- 在该队列中,HCM- LVSD发生在8% (553名患者) 的患者中.
- 75% 的 HCM- LVSD 患者出现了不良事件; 35% 的患者达到了复合结局 (死亡,移植,LVAD),平均时间为 8. 4 年.
- 综合结局的预测因素包括多种瘤变异,心房动和LVEF<5%. 发生HCM-LVSD的预测因素包括LV大小,壁厚,LVEF50-60%,晚期加多增强和瘤变异.
结论:
- HCM-LVSD影响了很少的HCM患者,往往具有严重的后果.
- 遗传因素,特别是体变体 (多或细丝),在预后和疾病进展中起着至关重要的作用.
- 临床特征和心脏成像发现也是HCM-LVSD不良事件和疾病发展的关键预测因素.
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