New opportunities pathogenetic therapy of pulmonary arterial hypertension

The review summarizes new data on medical treatment of pulmonary arterial hypertension (PAH). Pulmonary arterial remodeling is known to be the main pathophysiological characteristic, involving cell proliferation, hypertrophy and migration, as well as apoptotic abnormalities and changes in extracellular matrix production and degradation. This makes promising the evaluation of drugs with not only vasodilatory, but also antiproliferative and anti-remodelling properties. There are two approaches to improvement of PAH therapy: 1) novel agents with higher efficacy or more convenience for use, acting on the above mentioned targets, and 2) agents aimed at new pathophysiological targets, discovered by the most recent research in PAH area. They have shown potential efficacy in experimental models as well as in early phase clinical trials and seem promising for further improvement of pathophysiologically oriented treatment of PAH.