Hongo M, Fujii T, Hirayama J, Kinoshita O, Tanaka M, Okubo S
First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
J Am Coll Cardiol. 1989 Jan;13(1):48-53. doi: 10.1016/0735-1097(89)90547-0.
To assess left ventricular diastolic filling in amyloid heart disease, 17 patients with familial amyloid polyneuropathy and 20 normal subjects were examined by radionuclide angiography. None of the patients showed clinical evidence of restrictive cardiomyopathy. All but two patients had normal left ventricular ejection fraction. Peak filling rate was significantly lower and time to peak filling rate was significantly greater in patients than in normal subjects (2.60 +/- 0.52 versus 3.10 +/- 0.44 EDV/s, p less than 0.001, and 215 +/- 53 versus 147 +/- 18 ms, p less than 0.001, respectively). The mean left ventricular filling volume during rapid diastolic filling and atrial systole in patients was 54.5 +/- 19.5% and 44.2 +/- 21.6% of the stroke volume, respectively, compared with 83.8 +/- 6.6% (p less than 0.001) and 20.0 +/- 6.0% (p less than 0.001), respectively, in normal subjects. Although 10 of the 14 patients without clinical evidence of overt heart disease had normal ventricular wall thickness as well as normal ejection fraction, 8 of the 10 showed abnormal diastolic filling. In patients with familial amyloid polyneuropathy, indexes of diastolic filling were significantly related to ventricular wall thickness alone. The incidence and magnitude of abnormalities in time to peak filling rate and contribution of rapid filling as well as atrial systole to ventricular filling increased with age and duration of illness. Thus, abnormal diastolic filling can be seen even in the early stage of familial amyloid polyneuropathy and may be related to myocardial amyloid deposition as well as to fibrosis. Careful consideration should be given to age and duration of illness when diastolic filling is assessed in this disorder.
为评估淀粉样心脏病患者的左心室舒张期充盈情况，对17例家族性淀粉样多神经病患者和20名正常受试者进行了放射性核素血管造影检查。所有患者均无限制型心肌病的临床证据。除2例患者外，其余患者的左心室射血分数均正常。患者的峰值充盈率显著低于正常受试者，达到峰值充盈率的时间显著长于正常受试者（分别为2.60±0.52与3.10±0.44 EDV/s，p<0.001；以及215±53与147±18毫秒，p<0.001）。患者快速舒张期充盈和心房收缩期的平均左心室充盈量分别占每搏输出量的54.5±19.5%和44.2±21.6%，而正常受试者分别为83.8±6.6%（p<0.001）和20.0±6.0%（p<0.001）。虽然14例无明显心脏病临床证据的患者中有10例心室壁厚度正常且射血分数正常，但这10例中有8例舒张期充盈异常。在家族性淀粉样多神经病患者中，舒张期充盈指标仅与心室壁厚度显著相关。达到峰值充盈率的时间异常以及快速充盈和心房收缩期对心室充盈的贡献异常的发生率和程度随年龄和病程增加。因此，即使在家族性淀粉样多神经病的早期也可出现舒张期充盈异常，且可能与心肌淀粉样沉积以及纤维化有关。在评估该疾病的舒张期充盈时，应仔细考虑年龄和病程。
