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野生型转甲状腺素蛋白心脏淀粉样变性的右心房变异型

A Right-atrial Variant of Wild-type Transthyretin Cardiac Amyloidosis.

作者信息

Lavanya Narra, Oomman Abraham

机构信息

Department of Cardiology, KIMS Hospitals, Kondapur, Hyderabad Telangana, India.

Department of Cardiology, Apollo Main Hospital Chennai, Tamil Nadu, India.

出版信息

US Cardiol. 2025 Apr 1;19:e08. doi: 10.15420/usc.2024.30. eCollection 2025.

Abstract

Amyloidosis is caused by extracellular deposition of amyloid protein in various organs and tissues. Light-chain amyloidosis is the most common systemic amyloidosis, whereas transthyretin amyloid cardiomyopathy is emerging as the underdiagnosed variant, especially in the elderly. Cardiac MRI and technetium-99m-pyrophosphate scintigraphy are specific non-invasive modalities that have simplified the diagnostic accuracy of cardiac amyloidosis. Identifying the type of amyloidosis is of paramount importance, given the differences in management protocols. Increased left-ventricular wall thickness and diastolic dysfunction are the most easily detectable manifestations of cardiac amyloidosis. Atrial involvement is early in both light-chain and transthyretin amyloidosis and is associated with high risk of arrhythmias and thromboembolic events. We report a case of wild-type transthyretin amyloid cardiomyopathy with predominant involvement of the right atrium and patchy involvement of the right and left ventricles.

摘要

淀粉样变性是由淀粉样蛋白在各种器官和组织中的细胞外沉积引起的。轻链淀粉样变性是最常见的全身性淀粉样变性,而转甲状腺素蛋白淀粉样心肌病正逐渐成为诊断不足的变体,尤其是在老年人中。心脏磁共振成像和锝-99m焦磷酸盐闪烁扫描是特定的非侵入性检查方法,它们简化了心脏淀粉样变性的诊断准确性。鉴于治疗方案的差异,确定淀粉样变性的类型至关重要。左心室壁厚度增加和舒张功能障碍是心脏淀粉样变性最容易检测到的表现。在轻链和转甲状腺素蛋白淀粉样变性中,心房受累均较早,且与心律失常和血栓栓塞事件的高风险相关。我们报告一例野生型转甲状腺素蛋白淀粉样心肌病,主要累及右心房,右心室和左心室有散在受累。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f50/11976736/28716665ec81/usc-19-e08-g001.jpg

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