Arbustini E, Dander B, Buonanno C, Pennelli N, Zardini P, Thiene G
G Ital Cardiol. 1984 Jul;14(7):512-6.
A case of cardiac amyloidosis is described in which clinical and scintigraphic findings were those of ischemic heart disease. Electrocardiogram disclosed low voltages in the peripheral leads and a pattern of anterior pseudo-myocardial infarction. 2D-echocardiography showed thickened cardiac walls and a "granular sparkling" appearance of the septum. The curve of the left ventricular diastolic pressure suggested impaired ventricular relaxation without a true dip-plateau morphology. Stains for amyloid of rectal biopsy specimens were negative. Histological study of left ventricular endomyocardial biopsy indicated the presence of basophilic material among the myocytes; at ultrastructural examination this infiltrating substance resulted to be amyloid. This case stresses the effectiveness of ultrastructural investigation of left ventricular endomyocardial biopsy in the diagnosis of infiltrative and/or metabolic cardiac disorders.
本文描述了一例心脏淀粉样变性病例,其临床和闪烁扫描结果提示为缺血性心脏病。心电图显示外周导联电压降低及前壁假性心肌梗死图形。二维超声心动图显示心脏壁增厚及室间隔“颗粒状闪烁”外观。左心室舒张压曲线提示心室舒张功能受损,但无真正的下陷-平台形态。直肠活检标本的淀粉样蛋白染色为阴性。左心室心内膜活检的组织学研究表明心肌细胞间存在嗜碱性物质;超微结构检查显示这种浸润性物质为淀粉样蛋白。该病例强调了左心室心内膜活检超微结构检查在诊断浸润性和/或代谢性心脏疾病中的有效性。