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具有肥厚型心肌病超声心动图特征的轻链型淀粉样变性

Light-chain amyloidosis with echocardiographic features of hypertrophic cardiomyopathy.

作者信息

Dinwoodey Danya L, Skinner Martha, Maron Martin S, Davidoff Ravin, Ruberg Frederick L

机构信息

Section of Cardiology, Department of Medicine, Boston Medical Center, Boston, MA, USA.

出版信息

Am J Cardiol. 2008 Mar 1;101(5):674-6. doi: 10.1016/j.amjcard.2007.10.031. Epub 2007 Dec 21.

Abstract

Hypertrophic cardiomyopathy and cardiac amyloidosis result in thickening of the left ventricle, as visualized by 2-dimensional echocardiography. Hemodynamically, hypertrophic cardiomyopathy can be typified by a left ventricular outflow tract gradient and systolic anterior motion of the mitral apparatus, findings rarely seen in cardiac amyloidosis. This case series reports 4 patients with cardiac light-chain amyloidosis and left ventricular outflow tract obstruction at rest, suggesting that there may be echocardiographic overlap between these 2 disparate disease processes. In a series of consecutive patients with cardiac light-chain amyloidosis over a 2-year period, the prevalence of these echocardiographic findings was approximately 4%. In conclusion, awareness of this overlap in echocardiographic presentation may permit more accurate diagnosis, particularly at early stages of amyloid disease, when more treatment options exist.

摘要

肥厚型心肌病和心脏淀粉样变性会导致左心室增厚,二维超声心动图可显示这一情况。在血流动力学方面,肥厚型心肌病的典型表现为左心室流出道梯度和二尖瓣装置的收缩期前向运动,而这些表现很少见于心脏淀粉样变性。本病例系列报告了4例静息时存在左心室流出道梗阻的心脏轻链淀粉样变性患者,提示这两种不同的疾病过程在超声心动图表现上可能存在重叠。在一项为期2年的连续心脏轻链淀粉样变性患者系列研究中,这些超声心动图表现的发生率约为4%。总之,认识到超声心动图表现上的这种重叠可能有助于更准确的诊断,尤其是在淀粉样疾病的早期阶段,此时存在更多的治疗选择。

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