Short-Term Outcomes of Infants and Children Receiving Selexipag for Pulmonary Hypertension

Abstract

Selexipag is a long acting, immediate release, prostacyclin IP receptor agonist used to treat pulmonary hypertension (PH). Little is known about its use in infants and children. Our study reports short-term outcomes in pediatric patients receiving selexipag. We reviewed pediatric PH patients who received selexipag at our hospital. Previously published dosing/titration recommendations from our group were followed. Nine children (3 infants, 1 toddler, & 5 adolescents) received selexipag since Feb. 2018. Median age and weight at initiation were 10 years (97 days to 18.6 years) and 34 kg (3.4-162 kg). The 4 youngest subjects were transitioned from intravenous treprostinil to selexipag and 5 subjects initiated de novo. Transitions from treprostinil were uneventful, with one infant requiring slower titration due to asymptomatic hypotension. Three adolescents required selexipag dose reduction, with two unable to achieve target dose. Median duration of therapy was 322 days (54-600 days) with five subjects remaining on selexipag. Two subjects remain on PH therapy after discontinuation of selexipag. One subject's follow up catheterization showed elevated pulmonary vascular resistance after discontinuing selexipag, thus it was re-initiated. A subject with worsening PH required transition to subcutaneous treprostinil while undergoing lung transplant evaluation. Children with a variety of other disease processes have received selexipag, including one with single ventricle anatomy status post BT shunt, one awaiting heart transplant with ventricular assist device support, and one status post heart transplant receiving treprostinil. There were no deaths and side effects mirror those seen in adults [Figure]. Administration of selexipag in infants and children appears safe, and is a viable alternative to other forms of prostacyclin administration in stable patients. Selexipag may also have a role as tertiary adjunct therapy for those who do not improve on dual oral therapy.