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转氨基氨基酸心肌病症在患者中,左心室壁厚度不明原因增加
Katarzyna Holcman1,2, Magdalena Kostkiewicz3,4, Wojciech Szot4,5
1Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, John Paul II Hospital, Pradnicka 80, 31-202, Krakow, Poland. katarzyna.holcman@gmail.com.
The international journal of cardiovascular imaging
|June 10, 2024
概括
晶氨基粉症 (ATTR) 心肌病比以前认为的更常见,患有无法解释的左心室壁加厚的患者. 早期诊断对于管理这种情况至关重要.
科学领域:
- 心脏病学 心脏病学
- 心血管成像 - 心血管成像
- 氨基粉症研究研究
背景情况:
- 粉样性心肌病 (CA) 在历史上被认为是罕见的.
- 影像学的进步增加了CA诊断频率.
- 晶氨基粉症 (ATTR) 是一种关键的CA类型.
研究的目的:
- 为了确定CA的患病率,特别是ATTR,在没有解释的增加左心室 (LV) 壁厚度的患者中.
- 在这个人群中确定与ATTR相关的临床和成像特征.
主要方法:
- 预计将招募100名成年人,这些成年人患有无法解释的LV壁厚增加 (2020-2022年).
- 综合分析包括临床数据,心电图,心声学,SPECT/CT和遗传检测.
- 使用LOGIT和多变量模型进行统计分析,以确定ATTR的预测因子.
主要成果:
- 18%的患者被诊断为CA (5%的轻链,12%的ATTR).
- 许多与ATTR相关的因素,包括年龄,多神经病变和特定的心声回声学发现 (LV质量,GLS,RV厚度).
- 使用LV质量,GLS和RV厚度的预测模型实现了ATTR诊断的AUC为0.99.
结论:
- 很大一部分患有不明原因的 LV 壁加厚的患者有潜在的 CA,特别是 ATTR.
- 在患有 LV 质量增加,全球纵向应变减少和右心室厚度增加的患者中,应该怀疑 ATTR .
- 这凸显了在未解释的 LV 缩中考虑 CA 的重要性.
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