Transitioning intravenous epoprostenol to oral selexipag in idiopathic pulmonary arterial hypertension: a case report

Abstract

AbstractIntravenous (i.v.) prostacyclin is the cornerstone treatment in high‐risk pulmonary arterial hypertension (PAH) patients. Selexipag is an orally available prostacyclin receptor agonist. Limited data are available regarding the feasibility of transitioning from i.v. epoprostenol to selexipag. A 50‐year‐old woman with idiopathic PAH was diagnosed in a World Health Organization (WHO) Functional Class (FC) IV. She improved with upfront triple combination therapy, including i.v. epoprostenol. Over 2 years of follow‐up, the patient remained at low risk and expressed strong preference towards oral therapies. After careful risk–benefit clinical consideration, she was transitioned from i.v. epoprostenol to selexipag. Selexipag was started at dosage of 200 μg twice daily (b.i.d.) and titrated up to 1600 μg b.i.d. over 8 weeks (up‐titration of 200 μg b.i.d. every week). Simultaneously, i.v. epoprostenol was down‐titrated 3.0 ng/kg/min every week from a dosage of 27.5 ng/kg/min. The transition occurred under strict medical surveillance and was well tolerated. One year after discontinuation of epoprostenol, the patient remains in WHO FC I and has no signs of clinical deterioration. Although not generalizable to most PAH patients, this case highlights that a carefully planned transition from epoprostenol to selexipag is feasible in selected low‐risk patients within a shared medical decision‐making framework.