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舒张功能障碍揭示心脏轻链淀粉样变:一例报告

Diastolic Dysfunction Unveiling Cardiac Light-Chain Amyloidosis: A Case Report.

作者信息

Saha Somesh, Mondal Ritwick, Deb Shramana, Sarkar Biswarup, Benito-León Julián

机构信息

Department of Critical Care Medicine, Belle Vue Clinic, Kolkata, India.

Department of Clinical Pharmacology and Therapeutic Medicine, IPGMER and SSKM Hospital, Kolkata, India.

出版信息

Med Res Arch. 2024 Dec;12(12). doi: 10.18103/mra.v12i12.6168.

Abstract

BACKGROUND

Cardiac light-chain amyloidosis represents a critical component of this multi-systemic disease, significantly impacting prognosis. The extent of cardiac free light-chain deposition is the primary determinant of survival.

CASE PRESENTATION

We report the case of a 67-year-old male with a 10-year history of diabetes mellitus and arterial hypertension who presented with a two-day history of chest discomfort and difficulty lying down or sleeping, along with a two-month history of progressively worsening exertional dyspnea. On examination, the patient exhibited low blood pressure. A 12-lead electrocardiogram revealed poor R-wave progression and left ventricular hypertrophy. Further evaluation using 2D echocardiography demonstrated significant concentric left ventricular hypertrophy, a restrictive filling pattern, and mild pericardial effusion. Cardiac magnetic resonance imaging, nuclear imaging, and biopsy confirmed the diagnosis of cardiac light-chain amyloidosis.

CONCLUSION

Timely recognition and a high index of suspicion are essential for the early diagnosis of cardiac amyloidosis. Prompt diagnosis enables the initiation of definitive therapy, which may halt disease progression and significantly improve prognosis.

摘要

背景

心脏轻链淀粉样变性是这种多系统疾病的关键组成部分,对预后有重大影响。心脏游离轻链沉积的程度是生存的主要决定因素。

病例介绍

我们报告一例67岁男性,有10年糖尿病和动脉高血压病史,出现胸痛不适、平卧或睡眠困难2天,以及进行性加重的劳力性呼吸困难2个月。检查时,患者血压低。12导联心电图显示R波进展不良和左心室肥厚。进一步使用二维超声心动图评估显示明显的同心性左心室肥厚、限制性充盈模式和轻度心包积液。心脏磁共振成像、核成像和活检确诊为心脏轻链淀粉样变性。

结论

及时识别和高度怀疑对于心脏淀粉样变性的早期诊断至关重要。及时诊断能够启动确定性治疗,这可能会阻止疾病进展并显著改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a5f/11900892/2f61d34c0fce/nihms-2046110-f0001.jpg

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