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快速进展性心脏淀粉样变性非典型表现的超声心动图特征

Echocardiographic features of an atypical presentation of rapidly progressive cardiac amyloidosis.

作者信息

Brugts Jasper J, Houtgraaf Jaco, Hazenberg Bouke Pc, Kofflard Marcel Jm

机构信息

Jasper J Brugts, Jaco Houtgraaf, Marcel JM Kofflard, Department of Cardiology, Albert Schweitzer hospital, 3300 AK Dordrecht, The Netherlands.

出版信息

World J Cardiol. 2013 May 26;5(5):154-6. doi: 10.4330/wjc.v5.i5.154.

Abstract

We present the case of a 66 year old male who presented with dyspnea and reduced exercise tolerance. Echocardiography demonstrated impaired left ventricular (LV) function and restrictive diastolic function with pronounced concentric left ventricular hypertrophy (LVH) without a history of hypertension and no aortic valve stenosis. Differential diagnostics of concentric LVH are discussed in detail. In the current case, cardiac amyloidosis (AL) amyloidosis was diagnosed and confirmed by serum amyloid P (SAP) scintigraphy and abdominal fat aspiration biopsy. This case shows the rapid decline in clinical condition with progression of cardiac involvement of AL. As discussed in detail, cardiac involvement in AL-amyloidosis generally denotes a poor prognosis, regardless of the method of treatment.

摘要

我们报告一例66岁男性患者,其表现为呼吸困难和运动耐量下降。超声心动图显示左心室(LV)功能受损和舒张功能受限,伴有明显的同心性左心室肥厚(LVH),无高血压病史且无主动脉瓣狭窄。详细讨论了同心性LVH的鉴别诊断。在本病例中,通过血清淀粉样蛋白P(SAP)闪烁扫描和腹部脂肪抽吸活检诊断并确诊为心脏淀粉样变性(AL)淀粉样变性。该病例显示随着AL心脏受累的进展,临床状况迅速恶化。如详细讨论的那样,无论治疗方法如何,AL淀粉样变性的心脏受累通常预示预后不良。

相似文献

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Amyloidosis and cardiac involvement.淀粉样变性与心脏受累。
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本文引用的文献

3
Evaluation and management of the cardiac amyloidosis.心脏淀粉样变性的评估与管理
J Am Coll Cardiol. 2007 Nov 27;50(22):2101-10. doi: 10.1016/j.jacc.2007.08.028. Epub 2007 Nov 13.
4
Light-chain (AL) amyloidosis: diagnosis and treatment.轻链(AL)淀粉样变性:诊断与治疗
Clin J Am Soc Nephrol. 2006 Nov;1(6):1331-41. doi: 10.2215/CJN.02740806. Epub 2006 Oct 11.
6
Five or more years of survival in patients with primary systemic amyloidosis and biopsy-proven cardiac involvement.
Am J Cardiol. 2000 Mar 1;85(5):664-5, A11. doi: 10.1016/s0002-9149(99)00832-2.

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