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Diagnosis and treatment of idiopathic pulmonary arterial hypertension in 2008 Volume 4, issue 3, Mai-Juin 2008

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Université Paris-Descartes, Faculté de Médecine, Service de pneumologie et soins intensifs, Hôpital européen Georges-Pompidou, 20 rue Leblanc, 75015 Paris

Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by an important pulmonary microvascular remodelling. PAH occurs sporadically, clustered in families or as a complication of several associated conditions (connective tissue diseases, HIV infection…). Echocardiography has a key role in the early detection of the disease but right heart catheterisation must be performed in order to confirm the diagnosis. General measures (oral anticoagulation, diuretics, oxygen) are associated with specific treatment, targeted at correcting endothelial dysfunction, (prostanoids, endothelin receptor antagonists, phosphodiesterase 5 inhibitors). These treatments have led to a substantial increase in life expectancy. However, none of these medications can bring a complete cure of PAH. Early detection and treatment of the disease, new molecules targeting additional pathophysiologic mechanisms (tyrosine kinase inhibitors, serotonin reuptake inhibitors…) and the combination of currently available medications represent a major challenge for the future.