Brief report
Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan

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Abstract

In 7 of 8 children with idiopathic pulmonary arterial hypertension treated with intravenous epoprostenol for >1 year, concomitant use of bosentan allowed a reduction of epoprostenol and decreased its associated side effects without deterioration of clinical and hemodynamic parameters. In 3 children with normal or near-normal pulmonary artery pressure on epoprostenol, the addition of bosentan allowed discontinuation of epoprostenol and stabilization of hemodynamics for up to 1 year.

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    Citation Excerpt :

    These results have been extrapolated to children.109,127–134 Bosentan therapy added on to epoprostenol in children allowed for a decrease in epoprostenol dose and its associated side effects.109 A more recent retrospective study of 86 children on bosentan for a median exposure of 14 months with and without concomitant therapy found that bosentan as part of an overall treatment strategy provided a sustained clinical and hemodynamic improvement that was overall well tolerated, and 2 year survival estimates were 91%.

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This research was supported by grant MO1 RR00069 from the General Clinical Research Centers Program, National Center for Research Resources, National Institutes of Health, Bethesda, Maryland.

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