• Olivier Sitbon, Laurent Savale, Xavier Jaïs, David Montani, Marc Humbert, and Gérald Simonneau.
• 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.
• Presse Med. 2014 Sep 1; 43 (9): 981-93.

AbstractPatients 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.Copyright © 2014 Elsevier Masson SAS. All rights reserved.

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