Abstract
This review presents the modern evidence of specific pulmonary vasodilators in the treatment of circulatory failure in patients after
Fontan operation. Discussed in detail the pathological physiology of the circulation on the principle of Fontan. Efficiency of iloprost
after Fontan operation to date are shown in one, the effectiveness of sildenafil in several randomized and non-randomized studies, the
efficacy of bosentan in several randomized and non-randomized studies and in meta-analysis. Bosentan is the only specific pulmonary
vasodilator is approved in Russia for use in children and with appropriate children's dosage form.
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