Yamaki S, Horiuchi T, Miura M, Suzuki Y, Abe Y, Ishizawa E, Kagawa Y
Tohoku J Exp Med. 1985 Apr;145(4):455-63. doi: 10.1620/tjem.145.455.
Interesting findings were obtained in the lung biopsy of a 46 year old female patient with secundum atrial septal defect and pulmonary hypertension. In the intima of small pulmonary arteries, there were a marked increase in elastic fibers and marked proliferation of longitudinal smooth muscle cells. The vascular lumen was markedly stenotic, but reconstruction of small pulmonary arteries indicated that all vascular lumina were patent. The case was accordingly diagnosed as operable and closure of atrial septal defect was undertaken. Three years postoperatively, the patient is well with much improved condition and lowered pulmonary arterial pressure was demonstrated by cardiac catheterization. This case had apparently serious vascular lesions due to severe intimal thickening which we labeled "musculoelastosis", but it was found that such thickening alone did not cause peripheral pulmonary arterial occlusion. It is therefore thought to be a benign pulmonary vascular condition.
在一名患有继发孔型房间隔缺损和肺动脉高压的46岁女性患者的肺活检中获得了有趣的发现。在小肺动脉内膜中，弹性纤维明显增加，纵向平滑肌细胞明显增殖。血管腔明显狭窄，但小肺动脉重建显示所有血管腔均通畅。因此，该病例被诊断为可手术，并进行了房间隔缺损闭合术。术后三年，患者情况良好，病情明显改善，心导管检查显示肺动脉压降低。该病例因严重的内膜增厚而有明显严重的血管病变，我们将其标记为“肌弹性组织增生”，但发现仅这种增厚并不会导致外周肺动脉闭塞。因此，它被认为是一种良性肺血管疾病。
