title: [Treatment of pulmonary arterial hypertension].
abstract: Patients with pulmonary arterial hypertension (PAH) must be referred to expert centers (reference center and competences centers in the French PAH network). Despite progresses in the knowledge of PAH pathophysiology, it is still a devastating disease needing an aggressive approach of therapy to improve long-term outcomes. The target of current therapies is endothelial dysfunction of pulmonary arteries. Continuous intravenous infusion of epoprostenol remains the only recommended therapy for the most severe patients with PAH. If treatment goals are not met, a sequential combination therapy is recommended (i.e. combination of drugs targeting different dysfunctional pathways). Lung transplantation remains the only curative treatment of PAH.
authors.affiliation: Faculté de médecine, université Paris-Sud, 94270 Le Kremlin-Bicêtre, France; CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France. Electronic address: olivier.sitbon@bct.aphp.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.affiliation: CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Savale
authors.affiliation: CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.affiliation: Faculté de médecine, université Paris-Sud, 94270 Le Kremlin-Bicêtre, France; CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: David
authors.initials: D
authors.lastname: Montani
authors.affiliation: Faculté de médecine, université Paris-Sud, 94270 Le Kremlin-Bicêtre, France; CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Faculté de médecine, université Paris-Sud, 94270 Le Kremlin-Bicêtre, France; CHU de Bicêtre, AP-HP, centre de référence de l'hypertension pulmonaire sévère, service de pneumologie et soins intensifs, DHU thorax innovation, 94270 Le Kremlin-Bicêtre, France; Inserm U999, LabEx LERMIT, centre chirurgical Marie-Lannelongue, 92350 Le Plessis-Robinson, France.
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D000465:Algorithms; D000531:Altitude; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D054087:Atrial Septum / Q000601:surgery; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002637:Chest Pain / Q000503:physiopathology / Q000534:rehabilitation; D003278:Contraceptives, Oral, Hormonal; D000075202:Contraindications; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000503:physiopathology / Q000534:rehabilitation; D065128:Endothelin Receptor Antagonists; D015444:Exercise / Q000502:physiology; D005221:Fatigue / Q000503:physiopathology / Q000534:rehabilitation; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000628:therapy*; D016040:Lung Transplantation; D010102:Oxygen Inhalation Therapy; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011247:Pregnancy; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D013575:Syncope / Q000503:physiopathology / Q000534:rehabilitation
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D003278:Contraceptives, Oral, Hormonal; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D047428:Protein Kinase Inhibitors