Efficacy of Endothelin Blockade in Adults with Fontan Physiology

Abstract

In this cohort of adult patients with Fontan physiology, endothelin blockade with bosentan resulted in improved 6MWD and MRI-derived resting cardiac output, suggesting a positive effect on pulmonary vascular resistance and pulmonary blood flow. Bosentan was well tolerated and hepatic function was not adversely affected.