Jaïs Xavier, Sitbon Olivier, Savale Laurent, Montani David, Humbert Marc, Simonneau Gérald
Centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et réanimation respiratoire, hôpital Antoine-Béclère, 92141 Clamart Cedex, France.
Rev Prat. 2008 Nov 30;58(18):1997-2010.
Pulmonary arterial hypertension (PAH) is a rare condition characterised by increased pulmonary vascular resistance leading to right heart failure and death. The clinical classification distinguishes idiopathic PAH, familial PAH, and PAH associated with 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 is mandatory to confirm the diagnosis. Conventional therapy includes non-specific drugs (warfarin, diuretics). Intravenous epoprostenol is the first line treatment for the most severe patients. In the other situations, the first-line therapy may include bosentan, sildenafil, or a prostacyclin analogue. Recent advances in the management of PAH have markedly improved prognosis. 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，其特征为肺血管阻力增加，最终导致右心衰竭和死亡。临床分类包括特发性PAH、家族性PAH以及与其他疾病相关的PAH（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曾接触食欲抑制剂）。超声心动图是无创检测PAH的首选初始检查方法，但右心导管检查时测量肺压力和心输出量对于确诊至关重要。传统治疗包括非特异性药物（华法林、利尿剂）。静脉注射依前列醇是最严重患者的一线治疗方法。在其他情况下，一线治疗可能包括波生坦、西地那非或前列环素类似物。PAH管理方面的最新进展显著改善了预后。治疗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。
