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意义未明的单克隆丙种球蛋白病快速进展为伴有心内血栓的心脏淀粉样变性:通过心脏磁共振揭示

Rapid progression of monoclonal gammopathy of undetermined significance to cardiac amyloidosis with intracardiac thrombus: Unveiling via cardiac magnetic resonance.

作者信息

Kayed Hadeel A Al, Hamam Younes, Kayed Noor Al, Alajarmeh Ali O

机构信息

Faculty of Medicine, The University of Jordan, Amman, Jordan.

Deparment of Cardiac Radiology, Royal Jordanian Medical Services, Amman, Jordan.

出版信息

Radiol Case Rep. 2025 Mar 8;20(5):2566-2571. doi: 10.1016/j.radcr.2025.02.022. eCollection 2025 May.

DOI:10.1016/j.radcr.2025.02.022
PMID:40129832
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11930664/
Abstract

We present the case of a 67-year-old male diagnosed with Monoclonal Gammopathy of Undetermined Significance (MGUS) who developed progressive peripheral edema and abdominal distension, raising concerns for right-sided heart failure. Initial investigations, including electrocardiography and transthoracic echocardiography (TTE), revealed findings suggestive of restrictive diastolic dysfunction, including low-voltage QRS complexes, concentric left ventricular hypertrophy, left atrial enlargement, and valvular thickening, despite a preserved ejection fraction (EF) of 62%. These findings raised suspicion for AL-type cardiac amyloidosis in the context of the patient's recent MGUS diagnosis. Although no thrombus was detected on TTE, cardiac magnetic resonance imaging (CMR) conducted 2 weeks later demonstrated findings supportive of cardiac amyloidosis, such as myocardial infiltration and increased extracellular volume, and identified a left atrial appendage thrombus which was not visualized on the initial TTE. This case highlights the diagnostic superiority of CMR over TTE in detecting intracardiac thrombi. While transesophageal echocardiography (TEE) remains the gold standard for thrombus detection, its semi-invasive nature limits its routine use in initial evaluations. The findings underscore the importance of early utilization of CMR in suspected cardiac amyloidosis cases, emphasizing its comprehensive noninvasive assessment capabilities for guiding timely diagnosis and management.

摘要

我们报告了一例67岁男性病例,该患者被诊断为意义未明的单克隆丙种球蛋白病(MGUS),出现进行性外周水肿和腹胀,引发了对右心衰竭的担忧。包括心电图和经胸超声心动图(TTE)在内的初步检查显示,尽管射血分数(EF)为62%,但存在提示限制性舒张功能障碍的表现,包括低电压QRS波群、向心性左心室肥厚、左心房扩大和瓣膜增厚。鉴于患者近期诊断为MGUS,这些发现引发了对AL型心脏淀粉样变的怀疑。尽管TTE未检测到血栓,但2周后进行的心脏磁共振成像(CMR)显示了支持心脏淀粉样变的表现,如心肌浸润和细胞外容积增加,并发现了最初TTE未显示的左心耳血栓。该病例突出了CMR在检测心内血栓方面优于TTE的诊断优势。虽然经食管超声心动图(TEE)仍然是血栓检测的金标准,但其半侵入性限制了其在初始评估中的常规使用。这些发现强调了在疑似心脏淀粉样变病例中早期使用CMR的重要性,突出了其在指导及时诊断和管理方面的全面无创评估能力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/379176914192/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/95b6310e872d/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/aa0d19685dd6/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/46511b84f54c/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/9e3f1c8a95e4/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/379176914192/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/95b6310e872d/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/aa0d19685dd6/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/46511b84f54c/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/9e3f1c8a95e4/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f53/11930664/379176914192/gr5.jpg

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Thromboembolic and bleeding risk in cardiac amyloidosis.心脏淀粉样变性的血栓栓塞和出血风险。
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The Auxiliary Role of Cardiac Magnetic Resonance Feature-Tracking Parameters in the Differentiation between Cardiac Amyloidosis and Constrictive Pericarditis.心脏磁共振特征追踪参数在心脏淀粉样变性与缩窄性心包炎鉴别诊断中的辅助作用
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