Pulmonary hypertension and right ventricular failure. Part X. Prostanoids in the treatment of primary pulmonary arterial hypertension

In a series of articles the authors discuss literature data concerning epidemiology of pulmonary arterial hypertension (PAH), its modern classification; peculiarities of its pathogenesis and treatment in various. diseases and conditions. The tenth communication contains consideration of results of controlled trials of prostacyclin and its synthetic analogues in patients with primary (idiopathic) PAH,. and PAH associated with diffuse diseases of connective tissue, congenital heart disease, HIV-infection. Continuous intravenous infusion of prostacyclin (epoprostenol) has made a revolution in the treatment of patient with severe PAH because its long-term use is associated with improvement of survival of patients refractory to high doses of calcium antagonists. Subcutaneous administration of treprostinil and especially inhaled iloprost substantially widen possibilities of prolonged use of prostanoids in patients with various forms of PAH. Literature data on application of different prostanoids for long term treatment of patients with PAH, their side effects, indications and counterindications for their administration are analyzed in this communication.