Noma S, Akaishi M, Murayama A, Akiyama H, Ogawa S, Handa S, Nakamura Y, Sohma Y, Hosoda Y, Gotoh M
J Cardiogr. 1982 Mar;12(1):267-78.
This report described 56-year-old male with cardiac amyloidosis, whose echocardiographic and hemodynamic findings suggested hypertrophic obstructive cardiomyopathy. M-mode echocardiography demonstrated asymmetric septal hypertrophy (2.1 cm/1.6 cm), systolic anterior motion of the mitral valve, and pericardial effusion. Two-dimensional echocardiography revealed marked hypertrophy of the septum and papillary muscles with characteristic fine grannular sparkling appearance of the myocardium. Bilateral heart catheterization showed normal hemodynamics except a pressure gradient of 35 mmHg within the left ventricular outflow tract. Moreover, no hemodynamic evidence of restrictive cardiomyopathy was observed. Myocardial biopsy was performed at the time of pericardiectomy, and diffuse amyloid deposits were identified between myocardial fibers. It is of particular interest that cardiac amyloidosis presented the form of ventricular hypertrophy associated with left ventricular outflow obstruction, probably due to uneven deposition of amyloid.
本报告描述了一名56岁患有心脏淀粉样变性的男性,其超声心动图和血流动力学检查结果提示肥厚型梗阻性心肌病。M型超声心动图显示不对称性室间隔肥厚(2.1厘米/1.6厘米)、二尖瓣收缩期前向运动和心包积液。二维超声心动图显示室间隔和乳头肌明显肥厚,心肌具有特征性的细颗粒状闪烁外观。双侧心导管检查显示,除左心室流出道内有35毫米汞柱的压力阶差外,血流动力学正常。此外,未观察到限制性心肌病的血流动力学证据。在心包切除时进行了心肌活检,发现心肌纤维间有弥漫性淀粉样沉积物。特别值得注意的是,心脏淀粉样变性表现为伴有左心室流出道梗阻的心室肥厚形式,这可能是由于淀粉样物质沉积不均匀所致。