Case Report: Challenges in the diagnosis and treatment of cardiac amyloidosis: a case misdiagnosed as dual cardiac amyloidosis

病例报告:心脏淀粉样变性诊断和治疗的挑战:一例误诊为双重心脏淀粉样变性的病例

阅读:2

Abstract

Cardiac amyloidosis (CA) is a pathologic condition characterized by the cardiac deposition of insoluble misfolded proteins, often resulting in nonspecific symptoms that complicate diagnosis. Although advances in diagnostic techniques have improved the diagnostic rates, accurate identification of amyloid fibril deposits remains challenging. Here, we present a 52-year-old Chinese woman who was initially diagnosed with both wild-type cardiac transthyretin amyloidosis (ATTR-CA) and lambda cardiac light-chain amyloidosis (AL-CA) after the standardized diagnostic workup. Subsequent liquid chromatography-tandem mass spectrometry, however, identified lambda light chains as the primary amyloidogenic protein, confirming a final diagnosis of lambda AL-CA. The diagnostic process was further complicated when a review of (99m)Tc-pyrophosphate scintigraphy turned strongly positive after an eleven-month interval. Following the patient's refusal of chemotherapy, her heart failure progressively worsened, requiring the support of vasoactive and inotropic drugs, and she ultimately succumbed to cardiogenic shock 17 months after initial presentation. This case underscores the diagnostic challenges and necessity of sophisticated techniques for accurately typing CA.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。