Eriksson P, Backman C, Eriksson A, Eriksson S, Karp K, Olofsson B O
Acta Med Scand. 1987;221(1):39-46.
The clinical and echocardiographic features of cardiac amyloidosis may closely resemble those of hypertrophic cardiomyopathy, and the disorders may thus be mixed up. The present study was undertaken in an attempt to identify features separating the two conditions by analysis of electro- and echocardiographic findings in patients with familial amyloid polyneuropathy and hypertrophic cardiomyopathy. Twenty-nine patients with familial amyloidosis and 22 with hypertrophic cardiomyopathy were studied. Particular attention was given to the sum of the S wave in V1 and R wave in V5 or V6, the echocardiographic left ventricular mass and cross-sectional area, the presence or absence of asymmetrical septal thickening, granular and sparkling myocardial appearance, thickened heart valves, systolic anterior motion of the mitral valve, and pericardial effusion. A granular and sparkling appearance of the myocardium and thickened heart valves were found to be the best predictors of cardiac amyloidosis, while low QRS amplitudes in relation to echocardiographic left ventricular mass and a pericardial effusion seemed less important. The presence of systolic anterior movement of the mitral valve, a large left ventricular mass and a sum of S in V1 and R in V5 or V6 greater than 35 mm indicated hypertrophic cardiomyopathy. When the four strongest predictors (left ventricular mass, thickened heart valves, a granular sparkling myocardial appearance, and systolic anterior movement of the mitral valve) were used to reclassify the present patients, 28 of 29 amyloidosis patients and 21 of 22 patients with hypertrophic cardiomyopathy were correctly categorized. Noninvasive methods may thus be useful for detecting the myocardial infiltrative process, and cardiac amyloidosis may be confidently diagnosed by typical noninvasive findings together with histopathological documentation of amyloid in an organ other than the heart.
心脏淀粉样变性的临床和超声心动图特征可能与肥厚型心肌病极为相似，因此这两种病症可能会被混淆。本研究旨在通过分析家族性淀粉样多神经病和肥厚型心肌病患者的心电图和超声心动图检查结果，找出区分这两种病症的特征。对29例家族性淀粉样变性患者和22例肥厚型心肌病患者进行了研究。特别关注了V1导联S波与V5或V6导联R波的总和、超声心动图测得的左心室质量和横截面积、是否存在不对称性室间隔增厚、心肌颗粒状和闪烁样外观、心脏瓣膜增厚、二尖瓣收缩期前向运动以及心包积液情况。发现心肌颗粒状和闪烁样外观以及心脏瓣膜增厚是心脏淀粉样变性的最佳预测指标，而相对于超声心动图测得的左心室质量而言QRS波幅较低以及心包积液似乎不太重要。二尖瓣收缩期前向运动、左心室质量较大以及V1导联S波与V5或V6导联R波总和大于35mm提示肥厚型心肌病。当使用四个最强的预测指标（左心室质量、心脏瓣膜增厚、心肌颗粒状闪烁样外观和二尖瓣收缩期前向运动）对本研究中的患者重新分类时，29例淀粉样变性患者中有28例、22例肥厚型心肌病患者中有21例被正确分类。因此，非侵入性方法可能有助于检测心肌浸润过程，结合心脏以外器官淀粉样蛋白的组织病理学证据，典型的非侵入性检查结果可确诊心脏淀粉样变性。
