Hsieh S P, Wang J S, Tsui C Y, Liu H C
Department of Pathology, Veterans General Hospital-Taipei, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1990 Feb;45(2):134-8.
Pulmonary hypertension is caused largely by an increase in pulmonary vascular resistance and is most frequently secondary to chronic pulmonary obstructive or interstitial diseases, recurrent pulmonary emboli, or antecedent heart diseases. Primary pulmonary hypertension (plexogenic pulmonary arteriopathy) is a rare disease and diagnosed only when the underlying causing factors are undetermined. Two autopsy cases of primary pulmonary hypertension, first documented at Taipei Veterans General Hospital, are reported. They showed apparent pulmonary vascular changes including medial hypertrophy, intimal proliferation and fibrosis and plexiform lesions of the muscular pulmonary arteries. The plexiform lesion has been considered to be characteristic in the histopathological diagnosis of primary pulmonary hypertension when there are no other associated diseases, such as cirrhosis of liver, pre- or post- tricuspid congenital cardiac shunts and portal vein thrombosis. The pathogenesis of the plexiform lesion is obscure. In the present report, we shall emphasize pathological changes of the "plexiform lesions" and discuss their pathogenesis.
肺动脉高压主要由肺血管阻力增加引起，最常见于慢性肺阻塞性或间质性疾病、复发性肺栓塞或既往心脏病之后。原发性肺动脉高压（丛状肺血管病）是一种罕见疾病，仅在潜在病因不明时才能诊断。本文报告了台北荣民总医院首次记录的两例原发性肺动脉高压尸检病例。它们显示出明显的肺血管变化，包括中膜肥厚、内膜增生和纤维化以及肌性肺动脉的丛状病变。当不存在其他相关疾病，如肝硬化、三尖瓣先天性心脏分流术前或术后以及门静脉血栓形成时，丛状病变被认为是原发性肺动脉高压组织病理学诊断的特征。丛状病变的发病机制尚不清楚。在本报告中，我们将重点强调“丛状病变”的病理变化并讨论其发病机制。
