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心肌 Amyloidosis 的心肌结构和功能变化:从潜在的观察性患者注册表的见解

Franz Duca¹, René Rettl¹, Christina Kronberger¹

¹Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.

European heart journal. Cardiovascular Imaging

|August 7, 2023

概括

随着时间的推移,心脏粉症 (CA) 患者的连续细胞外体积 (ECV) 量化显示出显著的进展. 这种ECV变化预测了患者的结果,并有助于评估粉样蛋白特定治疗方法.

科学领域:

心脏病学心脏病学
放射学放射学是一门学科.
生物医学成像技术生物医学成像技术

背景情况:

心脏氨基粉症 (CA) 的特征在于心肌中的氨基粉沉积,导致细胞外体积 (ECV) 增加.
在CA患者中ECV进展的长度研究是有限的.
了解ECV动态对于评估疾病严重程度和治疗疗效至关重要.

研究的目的:

在心脏粉症 (CA) 患者中研究细胞外体积 (ECV) 的进展.
为了确定ECV随时间变化的预后影响,在CA.
评估串行ECV量化在监测CA和治疗响应中的实用性.

主要方法:

在2012年至2021年期间,在103名CA患者中进行了带有ECV量化的连续心脏磁共振 (CMR) 检查.
在基线和随访 (中位数为12个月) 测量了ECV,用于总体,跨甲状腺素 (ATTR) 和轻链 (AL) CA队列.
进行了生存分析,以评估ECV变化的预后值.

主要成果:

在随访期间,所有CA队列中,ECV中位数显著增加 (P < 0.001).
与接受治疗的患者相比,未接受治疗的ATTR患者的ECV中位数增加较高 (+5.7%与+2.3%,P = 0.004).
在所有CA类型中,ECV中位变化是结果的显著预测因素 (HR>1.07,P <0.013).

结论:

序列ECV量化对于管理CA患者是有价值的.
ECV变化是CA患者预后结果的预后标志物.
监测ECV进展可以为评估粉样蛋白特异性疗法提供信息.

关键词:

T1映射 T1映射 T1映射 T1映射心脏氨基粉症的发生.心脏磁共振成像 - 心脏磁共振成像细胞外体积的细胞外体积.轻链氨基粉症的发生转胺氨基粉症的发生.

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