心脏轻链淀粉样变性的舒张功能障碍1例报告。

Somesh Saha, Ritwick Mondal, Shramana Deb, Biswarup Sarkar, Julián Benito-León
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引用次数: 0

摘要

背景:心脏轻链淀粉样变是这种多系统疾病的重要组成部分,显著影响预后。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,有2天的胸部不适、躺下或睡眠困难,以及2个月的渐进式呼吸困难病史。经检查,患者表现为低血压。12导联心电图显示r波恶化和左心室肥厚。进一步的二维超声心动图显示明显的左心室同心性肥厚,限制性充盈模式和轻度心包积液。心脏磁共振成像、核成像和活检证实了心脏轻链淀粉样变的诊断。结论:对心脏淀粉样变的早期诊断应及时识别,提高怀疑指数。及时诊断可以开始明确的治疗,这可能会阻止疾病进展并显着改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Diastolic Dysfunction Unveiling Cardiac Light-Chain Amyloidosis: A Case Report.

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.

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