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Review Article
Drug Therapy
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Volume 351:1425-1436 September 30, 2004 Number 14
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Treatment of Pulmonary Arterial Hypertension
Marc Humbert, M.D., Ph.D., Olivier Sitbon, M.D., and Gérald Simonneau, M.D.

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Pulmonary arterial hypertension is a disease of the small pulmonary arteries that is characterized by vascular proliferation and remodeling.1,2 It results in a progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. A diagnosis of primary (or idiopathic) pulmonary hypertension is made when no known risk factor is identified.3,4 The diagnostic classification of pulmonary arterial hypertension is described in Table 1.3 Despite recent major improvements in symptomatic treatments, no current treatment cures this devastating condition.1,2,5,6,7,8,9 However, during the past 20 years, treatment options for patients with the disease have evolved to help prolong their survival . . . [Full Text of this Article]

Pathophysiological Features

Natural History and Survival

Therapeutic Strategies

Basic Therapy

Calcium-Channel Blockers

Prostacyclin Therapy

Intravenous Prostacyclin

Subcutaneous Treprostinil

Oral Beraprost

Inhaled Iloprost

Endothelin-Receptor Antagonists

Bosentan

Sitaxsentan and Ambrisentan

Potential Future Therapies

Nitric Oxide

Sildenafil

Vasoactive Intestinal Peptide

Selective Serotonin-Reuptake Inhibitors

Combination Therapy

Treatment Algorithms


Source Information

From Centre des Maladies Vasculaires Pulmonaires, Unité Propre de Recherche de l'Enseignement Superieur EA2705, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Université Paris-Sud, Assistance Publique–Hôpitaux de Paris, Clamart, France.

Address reprint requests to Dr. Humbert at the Service de Pneumologie, Hôpital Antoine-Béclère, 157 Rue de la Porte de Trivaux, 92140 Clamart, France, or at marc.humbert@abc.aphp.fr.


Related Letters:

Treatment of Pulmonary Arterial Hypertension
Huisman A.-M., Vreugdenhil S. A., van Paassen H. C., Humbert M., Sitbon O., Simonneau G.
Extract | Full Text | PDF  
N Engl J Med 2005; 352:308-309, Jan 20, 2005. Correspondence

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