Kinoshita O, Hongo M, Yamada H, Misawa T, Kono J, Okubo S, Ikeda S
First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Br Heart J. 1989 Feb;61(2):198-203. doi: 10.1136/hrt.61.2.198.
To assess left ventricular diastolic filling in patients with amyloid heart disease 12 patients with familial amyloid polyneuropathy and 15 normal subjects were studied by pulsed Doppler echocardiography. None of the patients had clinical evidence of overt heart disease or restrictive cardiomyopathy and only two of them showed ventricular wall thickening. The peak flow velocity of rapid diastolic filling and the acceleration rate of early diastolic inflow were significantly lower in patients with familial amyloid polyneuropathy than in controls. The pressure half time was significantly longer in patients than in controls. In addition, the peak flow velocity during atrial contraction and the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity were significantly greater in patients than in controls. Although there were no significant correlations between measurements of diastolic filling and clinical findings in patients with familial amyloid polyneuropathy, the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity was significantly related to left ventricular posterior wall thickness. These findings suggest that in patients with cardiac amyloidosis without restrictive cardiomyopathy, abnormal left ventricular diastolic filling, manifested by a reduction in the rate and volume of rapid diastolic filling with enhanced atrial contraction, can be seen even in the early stage of the disease.
为评估淀粉样心脏病患者的左心室舒张期充盈情况，采用脉冲多普勒超声心动图对12例家族性淀粉样多神经病患者和15名正常受试者进行了研究。所有患者均无明显心脏病或限制性心肌病的临床证据，仅2例出现心室壁增厚。家族性淀粉样多神经病患者舒张期快速充盈的峰值流速和舒张早期流入的加速度显著低于对照组。患者的压力减半时间显著长于对照组。此外，患者心房收缩期的峰值流速以及心房峰值流速与舒张期快速峰值流速之比均显著高于对照组。虽然家族性淀粉样多神经病患者舒张期充盈测量值与临床发现之间无显著相关性，但心房峰值流速与舒张期快速峰值流速之比与左心室后壁厚度显著相关。这些发现表明，在无限制性心肌病的心脏淀粉样变性患者中，即使在疾病早期也可出现左心室舒张期充盈异常，表现为舒张期快速充盈速率和容量降低，同时心房收缩增强。
