Lelguen C, Fernandez F, Saudemont J P, Barrillon A, Gerbaux A, Geschwind H, Duret J C, Pernod J
Arch Mal Coeur Vaiss. 1980 Feb;73(2):131-8.
19 cases of endomyocardial fibrosis were studied. Angiocardiography localises the site of fibrosis and seems to be the best diagnostic method. All cases in this series had left ventricular involvement which resulted in changes of the silhouette (square, polylobulated or deformed like the shape of a heart on a playing card) and of the ventricular contour (smooth, lacunar or "doubled"). Ten patients had mitral incompetence. The ejection fraction was normal in 8 patients but significantly reduced in the series as a whole (EF = 0,56, p less than 0,05). 15 patients had right ventricular involvement. Apart from the smooth contour of the anterior wall, the only abnormality in the mild cases, the most suggestive feature was an amputation of the ventricular apex, giving rise to a ventricular appearance of a narrow, akinetic (apart from the infundibular region) tube. The catheter data demonstrated the haemodynamic changes due to the fibrosis. A constrictive syndrome was observed in all the severe poorly tolerated cases. This was not apparent under basal conditions in milder cases. The value of pharmacodynamic testing and endomyocardial biopsy in cases where the diagnosis is uncertain should be stressed. The results of resection of the fibrosis and valvular replacement in severe cases depend to a large extent on the degree of myocardial involvement.
对19例心内膜心肌纤维化患者进行了研究。心血管造影可确定纤维化部位，似乎是最佳诊断方法。本系列所有病例均有左心室受累，导致心脏轮廓（方形、多叶状或类似扑克牌上心脏的变形）及心室轮廓（光滑、有腔隙或“双轮廓”）改变。10例患者存在二尖瓣关闭不全。8例患者射血分数正常，但整个系列射血分数显著降低（EF = 0.56，p < 0.05）。15例患者有右心室受累。除轻度病例中前壁轮廓光滑这一唯一异常外，最具提示性的特征是心室尖部截断，形成一个狭窄、无运动（除漏斗部区域外）的管状心室外观。导管检查数据显示了纤维化导致的血流动力学变化。在所有严重且耐受性差的病例中均观察到缩窄综合征。在较轻病例的基础状态下并不明显。应强调在诊断不确定的病例中进行药效学检测和心内膜心肌活检的价值。严重病例中纤维化切除术和瓣膜置换术的结果在很大程度上取决于心肌受累程度。
