Rich S, Brundage B H
Department of Medicine, University of Illinois, College of Medicine, Chicago 60680.
J Am Coll Cardiol. 1989 Sep;14(3):545-50. doi: 10.1016/0735-1097(89)90090-9.
Much of the understanding of hypertensive pulmonary vascular disease comes from studies of primary pulmonary hypertension. The three subtypes of primary pulmonary hypertension, plexogenic pulmonary arteriopathy, thromboembolic pulmonary hypertension and venoocclusive disease, have served as a basis to understand the mechanisms and to develop treatments of all forms of pulmonary hypertension. However, many inconsistencies regarding presumed pulmonary vasoconstriction and recurrent embolization remain. With newer data on the influence of the endothelium on vascular responsiveness and thrombosis, it appears that older concepts regarding the pathophysiology of pulmonary hypertension need to be revised. Recent studies have shown that plexogenic pulmonary arteriopathy is associated with abnormalities of endothelial structure and function that could result in impaired release of endothelial derived relaxing factors. Thromboembolic pulmonary arteriopathy, or more properly thrombotic pulmonary hypertension, appears to be the result of endothelial cell injury that creates a procoagulant environment in the pulmonary vascular bed with the development of widespread eccentric intimal proliferation and thrombosis in situ. It is possible that the effectiveness of vasodilator or anticoagulant therapy depends on the nature of the endothelial injury. Secondary pulmonary hypertension without endothelial injury, such as that which occurs with hypoxic lung disease or mitral stenosis, appears more satisfactorily treated when the primary cause is reversed.
对高血压性肺血管疾病的许多认识都来自于对原发性肺动脉高压的研究。原发性肺动脉高压的三种亚型，即丛状肺血管病、血栓栓塞性肺动脉高压和静脉闭塞性疾病，已成为理解所有形式肺动脉高压的发病机制和开发治疗方法的基础。然而，关于假定的肺血管收缩和反复栓塞仍存在许多不一致之处。随着有关内皮对血管反应性和血栓形成影响的新数据出现，似乎需要修正关于肺动脉高压病理生理学的旧观念。最近的研究表明，丛状肺血管病与内皮结构和功能异常有关，这可能导致内皮衍生舒张因子释放受损。血栓栓塞性肺血管病，或更确切地说是血栓性肺动脉高压，似乎是内皮细胞损伤的结果，这种损伤在肺血管床中形成促凝环境，并伴有广泛的偏心性内膜增生和原位血栓形成。血管扩张剂或抗凝治疗的有效性可能取决于内皮损伤的性质。无内皮损伤的继发性肺动脉高压，如在低氧性肺病或二尖瓣狭窄时发生的情况，当原发病因得到纠正时，治疗效果似乎更令人满意。
