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The article refers to actual data on pathophysiology of pulmonary arterial hypertension (PAH). PAH is a group of diseases characterized by a progressive increase of resistance and pressure in pulmonary vascular bed, leading to systolic overload and failure of the right heart ventricle, and in consequence - to premature death. The latest classification of PAH, basing on pathophysiology of the disease, was proposed in 2003 in Venice. The genetic background, including genetic mutations disturbing intracellular TGF-beta-like ligands signaling pathway, is essential for the development of PAH. In some cases secondary risk factors (e.g.: portal hypertension, congenital heart defects, HIV infection, connective tissue disorders, intoxications, myeloproliferative and storage diseases) are also important. In all types of PAH the same four pathological processes are reported: vasoconstriction, inflammation, thrombosis and remodelling. The growing knowledge of pathophysiology of PAH has enabled its better diagnosing and treatment, which has improved both symptoms and survival in the patients suffering from the disease.