At the beginning a new definition of pulmonal hypertension according to WHO 2003 is reported in the survey. The main attention is given to progress of pharmacological treatment of pulmonary arterial hypertension. Pulmonary arterial hypertension treatment is focused on patients in NYHA III or IV stage. The treatment with calcium channel blockers is useful only in patients with positive vasodilatory test. This test, however, is positive only in minority of patients. In majority of patients the drug of choice in the NYHA III class is the therapy with bosentan, a nonselective blocker of endothelin receptors or with epoprostenol – continuous infusion therapy. Good results were reported with prostanoid analogues – iloprost administered by inhalation or treprostinil administred continuously subcutaneously. Intensive studies of treatment with peroral prostanoid analogue beroprost are reported. The effectivness of sildenafil (phosphodiesterase inhibitor) is also being interesting. The results of this new therapy, which is markedly costly, are superior to the results of lung transplantation or lung and heart transplantation in patients with severe pulmonary arterial hypertension. Transplantation is indicated in patients with worsening of their clinical condition in spite of this therapy. This survey reports new substances and algorithm of pulmonary arterial hypertension therapy according to Galie et al. 2004. Some therapeutical methods (infusion treatment with epoprostenol) contributed to lowering of mortality and extending of life expectancy. Because of side effects of this therapy, possibilities of combined therapy have been studied that could lead to dosage lowering and consequently to side effects lowering. New therapeutical methods should be centred in a few selected cardiocentres for this is a rare disease and costly treatment.