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The review is devoted to modern pharmaceutical treatment of pulmonary arterial hypertension (PAH). In 1970-80s, it was based on high-dose calcium antagonists, CA (e.g., nifedipine 240 mg/d), which were effective only in some patients with primary (idiopathic) PAH. CA were combined with digoxin, indirect anticoagulants and oxygen. In 1990s, PAH therapy included prostanoids, endothelin receptor (ER) blockers and phosphodiesterase (PDE) inhibitors, which are more selective pulmonary vasodilatators than CA, and therefore are safer for long-term treatment. Prospective studies have demonstrated that continuous intravenous infusion of epoprostenol and ET receptor blocker bosentan treatment improve survival of PAH patients receiving traditional therapy. Clinical effectiveness of epoprostenol is similar to that of subcutaneously administered trepostinil, and bosentan is similar to PDE inhibitor Type 5 sildenafil. Bosentan increases the effectiveness of intravenous epoprostenol and inhaled trepostinil

In a series of articles the authors discuss literature data concerning epidemiology of pulmonary arterial hypertension (PAH), its current classification; peculiarities of its pathogenesis and treatment in various diseases and conditions. In the eleventh communication the authors discuss literature data related to the role of endothelin system in pathogenesis of primary (idiopathic) PAH, as well as PAH associated with diffuse diseases of connective tissue and congenital heart disease. This communication also contains presentation of clinical pharmacology of three available endothelin receptor blockers - bosentan, sitaxsentan, ambrisentan, and analysis of results of randomized controlled trials of efficacy and safety of these agents in patients with idiopathic PAH and PAH associated with diffuse diseases of connective tissue and congenital heart disease.

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 last 14-th communication contains consideration of issues of differentiated administration of modern pulmonary vasodilators to patients with PAH taking into account etiology and severity of the disease, availability of evidence based efficacy and safety data, method of application, and contraindications. In patients with PAH of II and III functional class (FC) endothelin receptor blocker bosentan is believed to be the drug of first choice. Bosentan can be administered orally. In patients with significant liver involvement phosphodiesterase inhibitor type 5 sildenafil should be used instead of bosentan for long term treatment of PAH. Sildenafil also can be taken orally. If bosentan is not sufficiently effective it can be combined with sildenafil; inhalations of prostanoid iloprost can be added to this combination when necessary. Is this tiple combination is not effective iloprost inhalations can be replaced by subcutaneous treprostinil or continuous intravenous infusion of epoprostenol. In patients with IV FC PAH therapy is started with subcutaneous administration of treprostinil or infusion of epoprostenol, while bosentan or/and sildenafil is added when necessary.