[Pulmonary hypertension--classification and treatment: new guidelines]

Pulmonary arterial hypertension is a severe pulmonary vascular disease characterized by elevated pulmonary vascular resistance and low cardiac output. Prior to prostacyclin therapy, it was a fatal disease with a median survival of 3 years. In recent years, the understanding and treatment of the disease have significantly improved patient outcome. The new therapeutic guidelines are based on the finding that vasoconstriction and vascular remodeling correlated with endothelial dysfunction, endothelin overexpression and diminished prostacyclin and nitric oxide synthesis. This article outlines the new classification and the current therapeutic guidelines accepted in the world presenting the role of prostacyclins, endothelin antagonist, phosphodiesterase V inhibitor, and the potential value of combination therapies.