Pulmonary arterial hypertension is a disease of the small pulmonaryarteries that is characterized by vascular proliferation andremodeling.1,2 It results in a progressive increase in pulmonaryvascular resistance and, ultimately, right ventricular failureand death. A diagnosis of primary (or idiopathic) pulmonaryhypertension is made when no known risk factor is identified.3,4The diagnostic classification of pulmonary arterial hypertensionis described in Table 1.3 Despite recent major improvementsin symptomatic treatments, no current treatment cures this devastatingcondition.1,2,5,6,7,8,9 However, during the past 20 years, treatmentoptions for patients with the disease have evolved to help prolongtheir 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 PubliqueHôpitaux 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.
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