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在法布里病中发展左心室缩的发生率和危险因素

Emanuele Monda^1,2, Athanasios Bakalakos^2,3, Robin Lachmann⁴

¹Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Napoli, Italy emanuelemonda@me.com.

Heart (British Cardiac Society)

|April 30, 2024

概括

大约四分之一的法布里病患者随着时间的推移发展出左心室缩. 年龄,男性性别和异常心电图预测了法布里病中这种心脏并发症.

科学领域:

心脏病学心脏病学
遗传学是一个遗传学.
内部医学内部医学

背景情况:

左心室缩 (LVH) 是法布里病 (FD) 中的主要心脏并发症.
了解FD中的LVH发病率和预测因素对于患者管理至关重要.
这项研究侧重于没有基线LVH的当代队列.

研究的目的:

为了确定患有法布里病 (FD) 的成年患者左心室缩 (LVH) 的发生率.
在没有基线LVH的FD患者队列中确定LVH发展的预测因子.
分析左心室质量指数 (LVMi) 随着时间的推移而变化的速度.

主要方法:

对患有FD的成年患者 (≥16岁) 进行了一项观察性队列研究.
患者在专业心肌病中心进行了前性跟踪.
主要终点是LVH的第一次检测 (男性LVMi≥115g/m2,女性≥95g/m2).

主要成果:

在没有基线LVH的214名患者中,55人 (24.6%) 在中位数9.4年的随访期间发展出LVH.
5,10年和15年的发生率分别为11.3%,29.1%和45.0%.
LVH的独立预测因素包括年龄较大,男性性别和异常心电图. 年度LVMi增加在男性中高于女性.

结论:

在研究期间,近四分之一的法布里病患者出现左心室缩.
年龄,男性性别和心电图异常是FD中LVH发展的重要风险因素.
这些发现强调了定期对FD患者进行心脏监测的重要性.

关键词:

心肌病,高性心肌病,高性心肌病.心声回声图 (Echocardiography) 是一种心声回声图.遗传学是一个遗传学.

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