Sivaram C A, Jugdutt B I, Amy R W, Basualdo C A, Haraphongse M, Shnitka T K
Clin Cardiol. 1985 Oct;8(10):511-8. doi: 10.1002/clc.4960081004.
Cardiac amyloidosis (CA) presenting with intractable congestive heart failure, electrocardiographic (ECG) normal or low voltage, and conduction or rhythm disturbances, is rapidly fatal. During life, CA often mimics other cardiomyopathies so that definitive diagnosis depends on demonstration of amyloid on myocardial biopsy. On two-dimensional echocardiography (2-D echo), nonspecific features, such as increased ventricular wall thicknesses, predominant diastolic dysfunction, and diffuse myocardial "sparkling," are consistently found in CA. The combined presence of these 2-D echo features and normal or low voltage on ECG is highly suggestive of CA, allows differentiation from other cardiomyopathies, and might be useful in noninvasive screening before myocardial biopsy.
表现为顽固性充血性心力衰竭、心电图(ECG)正常或低电压以及传导或节律紊乱的心脏淀粉样变性(CA)进展迅速,可导致死亡。在病程中,CA常与其他心肌病相似,因此明确诊断依赖于心肌活检显示淀粉样物质。二维超声心动图(2-D echo)检查时,CA常表现为非特异性特征,如心室壁厚度增加、以舒张功能障碍为主以及心肌弥漫性“闪烁”。2-D echo的这些特征与ECG正常或低电压同时存在,高度提示CA,有助于与其他心肌病相鉴别,并且可能在心肌活检前的无创筛查中发挥作用。