Pulmonary arterial hypertension (PAH) is a chronic, serious disease caused by remodeling of small pulmonary vessels, which leads to increase of pulmonary resistance, right heart failure and death. The 1990ths of XX century are the beginning of dynamic research into the pathophysiology and treatment of this disease. Actually, the goal oriented therapy based on three main metabolic pathways includes: prostacyclin's analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Applying this therapy, according to ESC guidelines has prolonged significantly the survival in the group of patients with PAH. Due to the high cost of the therapy, there has been created the national therapeutic program in Poland. It assures the real possibility of therapy for patients with PAH from the third FC WHO.