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轻链型心脏淀粉样变性沉积所致二尖瓣和三尖瓣狭窄

Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition.

作者信息

Randhawa Varinder K, Vakamudi Sneha, Phelan Dermot M, Samaras Christy J, McKenney Jesse K, Hanna Mazen, Perez Antonio L

机构信息

Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH, 44195, USA.

Kaufman Center for Heart Failure, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.

出版信息

ESC Heart Fail. 2020 Jun;7(3):1130-1135. doi: 10.1002/ehf2.12668. Epub 2020 Mar 16.

Abstract

Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence-based chemotherapy and heart failure pharmacotherapy led to end-stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease condition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.

摘要

心脏淀粉样变性导致浸润性限制性心肌病,具有一些特征性表现:双心室肥厚、心肌整体纵向应变异常且心尖相对保留、双房扩大、少量心包积液以及传导异常。导致具有血流动力学意义的瓣膜性心脏病的淀粉样沉积非常罕见。我们描述了一例罕见病例,一名患有进行性多发性骨髓瘤且经脂肪垫活检证实为轻链淀粉样变性的患者,由于持续的淀粉样沉积,同时出现了中度严重的三尖瓣和二尖瓣狭窄。尽管进行了循证化疗和心力衰竭药物治疗,但浸润性心肌病和瓣膜病仍不断恶化,最终发展为终末期疾病并导致死亡。心脏淀粉样变性中的瓣膜受累需要早期识别潜在疾病状况,以指导针对性的药物治疗并防止其进展。在这种情况下,瓣膜成形术或瓣膜置换不是可行的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/477f/7261535/9a43f3c96496/EHF2-7-1130-g001.jpg

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本文引用的文献

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Amyloidosis-induced tricuspid stenosis mimicking rheumatic heart disease.淀粉样变性所致三尖瓣狭窄酷似风湿性心脏病。
Eur Heart J Cardiovasc Imaging. 2014 Oct;15(10):1167. doi: 10.1093/ehjci/jeu075. Epub 2014 May 5.
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Localized dystrophic amyloidosis of heart valves.心脏瓣膜局限性营养不良性淀粉样变
Hum Pathol. 1983 Jul;14(7):649-53. doi: 10.1016/s0046-8177(83)80208-1.

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