Hypertension artérielle pulmonaire

Pulmonary arterial hypertension (PAH) is a rare condition characterized by an elevated pulmonary arterial resistance that, if untreated, leads to right heart failure. PAH can be sporadic (idiopathic PAH, or primary pulmonary hypertension), familial or related to other conditions including connective tissue diseases, congenital heart disease, human immunodeficiency virus infection, portal hypertension, appetite suppressant exposure …. The incidence of idiopathic PAH has been estimated to be 2 cases per million per year, but this figure may be underestimated due to the lack of specificity of PAH clinical signs (mostly the dyspnea). Echocardiography is the investigation of choice for non invasive screening. Measurement of haemodynamic parameters during right-heart catheterization is mandatory to establish definite diagnosis (mean pulmonary artery pressure > 25 mmHg with a pulmonary artery wedge pressure < 15 mmHg). Recent advances in the management of PAH including prostacyclin derivatives and endothelin receptor antagonists have improved markedly the patients' prognosis. Novel treatments such as type 5 phosphodiesterase inhibitors are promising in this setting. Lung transplantation is the last option for patients deteriorating despite optimal medical treatment.