title: [Pulmonary arterial hypertension].
abstract: 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.
authors.affiliation: Centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et réanimation respiratoire, hôpital Antoine-Béclère, 92141 Clamart Cedex, France. xavier.jais@abc.aphp.fr
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Laurent
authors.initials: L
authors.lastname: Savale
authors.forename: David
authors.initials: D
authors.lastname: Montani
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000209:etiology; D065128:Endothelin Receptor Antagonists; D016232:Endothelins / Q000037:antagonists & inhibitors; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D016040:Lung Transplantation; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D044062:Prostaglandins I / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D002121:Calcium Channel Blockers; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D016232:Endothelins; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I