Montani D, Jaïs X, Sitbon O, Capron F, Simonneau G, Humbert M
Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Université Paris-Sud, Assistance Publique, Hôpitaux de Paris, Clamart, France.
Rev Mal Respir. 2005 Sep;22(4):651-66. doi: 10.1016/s0761-8425(05)85616-5.
Pulmonary arterial hypertension (PAH) is a rare condition characterised by progressively elevated pulmonary arterial resistance leading to right heart failure.
A recent classification distinguishes idiopathic PAH, familial PAH and PAH secondary to other conditions (connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus infection or appetite suppressant exposure). Echocardiography is the initial investigation of choice for non-invasive detection of PAH but measurement of pulmonary pressures and cardiac output during right-heart catheterization are necessary to confirm the diagnosis of PAH. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). Intravenous epoprostenol is the first-line treatment for the most severely affected patients. In less severe cases, the first-line treatment may include bosentan or a prostacyclin analogue.
Recent advances in the management of PAH have markedly improved prognosis. The avai-lability of novel specific drugs including type 5 phosphodiesterase inhibitors offers novel therapeutic perspectives but their exact role in the treatment of PAH is still uncertain. The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in our understanding of the mechanisms mediating pulmonary arterial hypertension.
肺动脉高压（PAH）是一种罕见疾病，其特征为肺动脉阻力逐渐升高，最终导致右心衰竭。
最近的分类将特发性PAH、家族性PAH以及继发于其他疾病（结缔组织病、先天性心脏病、门静脉高压、人类免疫缺陷病毒感染或接触食欲抑制剂）的PAH区分开来。超声心动图是无创检测PAH的首选初始检查方法，但右心导管检查期间测量肺动脉压力和心输出量对于确诊PAH是必要的。传统治疗包括非特异性药物（华法林、利尿剂、氧气）。静脉注射依前列醇是病情最严重患者的一线治疗方法。在病情较轻的情况下，一线治疗可能包括波生坦或前列环素类似物。
PAH管理方面的最新进展显著改善了预后。包括5型磷酸二酯酶抑制剂在内的新型特异性药物的出现提供了新的治疗前景，但其在PAH治疗中的确切作用仍不确定。治疗方法从血管扩张剂向抗增殖药物的演变反映了我们对介导肺动脉高压机制的理解取得了进展。
