Abstract
Pulmonary arterial hypertension (PAH) is no longer an orphan disease. Three different classes of drugs for the treatment of PAH are being used and an increasing number of patients are being treated with a single drug or a combination therapy. During the last twenty-five years, new insights into the pathobiology of PAH have been gained. The classical mechanical concepts of pressure, flow, shear stress, RV wall stress and impedance, have been complemented with the new concepts of cell injury and repair and interactions of complex multicellular systems. Integrating these concepts will become critical, as we design new medical therapies in order to change the prognosis of the patients with this fatal group of diseases. This review intends to summarize recent pathobiological concepts of PAH and RVF derived mainly from human studies, which reflect the progress made in the understanding of these complex group of pulmonary vascular diseases.
- ERS
