title: [Pulmonary arterial hypertension].
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a rare condition characterised by progressively elevated pulmonary arterial resistance leading to right heart failure.
  
  
  BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: D
authors.initials: D
authors.lastname: Montani
authors.forename: X
authors.initials: X
authors.lastname: Jaïs
authors.forename: O
authors.initials: O
authors.lastname: Sitbon
authors.forename: F
authors.initials: F
authors.lastname: Capron
authors.forename: G
authors.initials: G
authors.lastname: Simonneau
authors.forename: M
authors.initials: M
authors.lastname: Humbert
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D004359:Drug Therapy, Combination; D004562:Electrocardiography; D011464:Epoprostenol / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D010726:Phosphodiesterase Inhibitors; D013449:Sulfonamides; D011464:Epoprostenol; D000077300:Bosentan