Case report of isolated aortic valve AL-amyloidosis following aortic valve replacement.

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

European Heart Journal: Case Reports Pub Date : 2025-08-08 eCollection Date: 2025-09-01 DOI:10.1093/ehjcr/ytaf393

Rajin Choudhury, Victor H Jimenez-Zepeda, Etienne Mahe, Nowell M Fine

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Abstract

Background: Light chain (AL)-amyloidosis is a haematologic malignancy where cardiac involvement confers a worse prognosis. There is a recognized association between aortic stenosis (AS) and transthyretin amyloidosis (ATTR) cardiomyopathy. However, there is no such reported association with AL amyloidosis.

Case summary: We present a case of degenerative AS where the pathology analysis post-surgical replacement demonstrated amyloid deposits subsequently identified as AL subtype by mass spectrometry. Subsequent investigations demonstrated no myocardial or systemic involvement. Following multidisciplinary discussion, cardiac biopsy and chemotherapy were deferred given the isolated nature of aortic valve involvement and clinical stability.

Discussion: Incidental detection of aortic valve AL-amyloidosis in this case demonstrates that such deposits cannot be assumed to be ATTR, and further work-up and amyloid typing is necessary.

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主动脉瓣置换术后孤立性主动脉瓣al -淀粉样变1例报告。

背景：轻链(AL)-淀粉样变是一种血液恶性肿瘤，累及心脏会导致较差的预后。主动脉瓣狭窄（AS）和甲状腺转蛋白淀粉样变（ATTR）心肌病之间有公认的联系。然而，没有这种与AL淀粉样变性相关的报道。病例总结：我们报告了一例退行性AS，手术后的病理分析显示淀粉样蛋白沉积，随后通过质谱鉴定为AL亚型。随后的调查显示没有心肌或全身受累。经过多学科讨论，考虑到主动脉瓣受累的孤立性和临床稳定性，心脏活检和化疗被推迟。讨论：本病例中偶然发现的主动脉瓣al -淀粉样变表明这种沉积物不能被认为是ATTR，需要进一步的检查和淀粉样样分型。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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