Sokol Ivan, Vincelj Josip, Sarić Muhamed
Zavod Za Kardiovaskularne Bolesti, Klinika Za Unutarnje Bolesti Klinicka Bolnica Dubrava, Zagreb, Hrvatska.
Med Arh. 2005;59(6):388-90.
Amyloid cardiomyopathy is myocardial infiltrative disorder which mostly has been seen as the consequence of systemic amiloidosis. The diffuse global myocardial infiltration of nonfunctional amyloid displaces the contractile myocites giving rice to relaxation abnormality and diastolic dysfunction of restrictive or congestive type of both ventricles, but more frequently with right-sided congestion, while systolic left ventricular function deteriorates late in disease process. We report a patient with amyloid cardiomyopathy and nephrotic syndrome underlying primary amiloidosis. Our aim is to point out at echocardiographic assessment of diagnosis and prognosis of amyloid cardiomyopathy, which is proven by postmortal endomyocardial biopsy. The hallmark of echocardiographic diagnostics are the findings of the thickened ventricular and septal walls, small ventricular cavities, dilated atria with thickened interatrial septum and atrioventricular valves, and granular-sparkling and hyperrefractile myocardium. Doppler assessment diagnostically gives us the insight in restrictive physiology of both ventricles, and the inverse relation of the left ventricular thickness and voltage on the ECG is high specific. Echocardiographic evaluation of mean left ventricular thickness in amyloid cardiomyopathy is very important prognostic parameter. so that if it is > or =15 mm, median survival is 0.4 years, whereas in our patient with median thickness of 2.76 cm the survival was only three months. The advanced diastolic dysfunction of the left ventricle with an increased transmitral E/A ratio and deceleration time of < or =150 ms is strong predictor of cardiac death. In this case of restrictive transmitral flow E/A was 1.7 and DT 100 ms and they were ominous prognostic signs of survival.
淀粉样心肌病是一种心肌浸润性疾病，大多被视为系统性淀粉样变性的后果。无功能淀粉样蛋白的弥漫性全心肌浸润取代了收缩性心肌细胞，导致舒张异常以及双心室限制性或充血性类型的舒张功能障碍，但更常见的是右侧充血，而左心室收缩功能在疾病后期恶化。我们报告了一例患有淀粉样心肌病和潜在原发性淀粉样变性肾病综合征的患者。我们的目的是指出超声心动图对淀粉样心肌病诊断和预后的评估，这在死后心内膜活检中得到了证实。超声心动图诊断的标志是心室壁和室间隔增厚、心室腔小、心房扩张伴房间隔和房室瓣增厚，以及心肌呈颗粒状闪烁和高回声。多普勒评估从诊断上让我们了解双心室的限制性生理情况，并且心电图上左心室厚度与电压的反比关系具有很高的特异性。淀粉样心肌病中平均左心室厚度的超声心动图评估是非常重要的预后参数，因此如果厚度≥15mm，中位生存期为0.4年，而在我们的患者中，中位厚度为2.76cm，生存期仅为三个月。左心室舒张功能障碍进展，二尖瓣E/A比值增加且减速时间≤150ms是心脏死亡的强烈预测指标。在这种限制性二尖瓣血流的情况下，E/A为1.7，DT为100ms，它们是生存的不祥预后迹象。
