Gan C T, Vonk Noordegraaf A, Marques K M J, Bronzwaer J G F, Postmus P E, Boonstra A
Neth Heart J. 2004 Jun;12(6):287-294.
Pulmonary arterial hypertension (PAH) is a disease characterised by an increased pulmonary artery pressure. The precapillary pulmonary arteries show distinct pathobiological changes, i.e. medial hypertrophy, intimal fibrosis, microthrombi and plexiform lesions. Although the pathogenesis is not completely understood, pulmonary vascular proliferation and remodelling, due to a variety of mediators, is believed to play the pathogenetic key role. Genetic research reveals molecular deformities and gene mutations associated with phenotypic PAH. This article covers novel insights into pathobiology, pathogenesis and genes of PAH, which led to a novel classification system and a diagnostic work-up, emanated from the World Health Organisation Symposium on Pulmonary Hypertension in Venice in June 2003.
肺动脉高压（PAH）是一种以肺动脉压力升高为特征的疾病。肺前毛细血管显示出明显的病理生物学变化，即中层肥厚、内膜纤维化、微血栓形成和丛状病变。尽管发病机制尚未完全明确，但多种介质导致的肺血管增殖和重塑被认为在发病过程中起关键作用。基因研究揭示了与表型PAH相关的分子畸形和基因突变。本文涵盖了对PAH病理生物学、发病机制和基因的新见解，这些见解促成了一个新的分类系统和诊断检查方法，源自2003年6月在威尼斯举行的世界卫生组织肺动脉高压研讨会。
