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Therapeutic Advances in Respiratory Disease
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Review: Therapeutic advances in pulmonary arterial hypertension

K. Boutet

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

David Montani

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

Xavier Jaïs

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

Azzedine Yaïci

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

Oliver Sitbon

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

Gérald Simonneau

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France

Marc Humbert

Université Paris-Sud 11, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France, marc.humbert @abc.aphp.fr

Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, in situ thrombosis, and vascular remodeling of small pulmonary arteries inducing increased pulmonary arterial resistance. Conventional treatment is based on life style modification and nonspecific treatment (warfarine, diuretics, oxygen). Calcium channel blockers are vasodilatators that have been shown to be of great efficacy in a very specific subpopulation of patients with PAH. For the majority of patients, specific PAH therapies are still lacking. Numerous studies evaluating prostacyclin agonists, endothelin-receptor antagonists, and phosphodiesterase type 5 inhibitors are now available to guide therapeutic choices. Despite those important advances there is still no cure for PAH. Fortunately, research is ongoing and many drugs show promises.

Key Words: pulmonary arterial hypertension • endothelin receptor antagonists • prostacyclin • type 5 phosphodiesterase • combination therapy • lung transplantation

Therapeutic Advances in Respiratory Disease, Vol. 2, No. 4, 249-265 (2008)
DOI: 10.1177/1753465808094762
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