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[以开始的浸润性心肌病形式存在且无限制性生理学表现的孤立性心脏淀粉样变性]

[The isolated form of cardiac amyloidosis in the form of beginning infiltrative cardiomyopathy without restrictive physiology].

作者信息

Kuchynka P, Palecek T, Simek S, Lubanda J C, Elleder M, Spicka I, Jansa P, Linhart A

机构信息

II. interní klinika, klinika kardiologie a angiologie 1. lékarské fakulty UK a VFN, Praha.

出版信息

Vnitr Lek. 2008 Oct;54(10):1010-3.

Abstract

The authors describe an interesting case of isolated cardiac manifestation of AL-amyloidosis manifesting as an incipient infiltrative cardiomyopathy with heart failure symptoms due to moderate left ventricular diastolic dysfunction. Restrictive cardiomyopathy with severe diastolic dysfunction is considered as the characteristic manifestation of fully developed cardiac amyloidosis. However, the organ deposition of amyloid is progressive and left ventricular filling worsens continuously, starting with less advanced forms of diastolic dysfunction; the restrictive physiology is characteristic only for advanced phases of the disease. Therefore, the possibility of the incipient infiltrative cardiomyopathy due to the amyloidosis should be considered in patients with heart failure symptoms and echocardiographic findings of unexplained left ventricular hypertrophy with only mild or moderate diastolic dysfunction.

摘要

作者描述了一例有趣的AL型淀粉样变性孤立性心脏表现病例,其表现为初期浸润性心肌病,因中度左心室舒张功能障碍而出现心力衰竭症状。严重舒张功能障碍的限制性心肌病被认为是完全发展的心脏淀粉样变性的特征性表现。然而,淀粉样蛋白的器官沉积是渐进性的,左心室充盈不断恶化,始于舒张功能障碍不太严重的形式;限制性生理仅在疾病晚期具有特征性。因此,对于有心力衰竭症状且超声心动图显示有无法解释的左心室肥厚且仅有轻度或中度舒张功能障碍的患者,应考虑淀粉样变性导致初期浸润性心肌病的可能性。

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