The use of selexipag, a prostacyclin receptor analog, for treatment of severe pulmonary artery hypertension during pregnancy, a case report

Pregnancy in patients with pulmonary artery hypertension (PAH) is associated with high mortality and morbidity. Despite the risks, more patients with PAH are becoming pregnant. Case reports and case series have described the use of IV epoprostenol in these patients with some success. However, there are no published reports regarding the use of oral prostacyclins and prostacyclin receptor agonists in pregnancy. We describe the use of selexipag, an oral prostacyclin receptor agonist, for treating severe PAH during pregnancy in a patient who refused IV prostacyclin therapy. She remained stable throughout pregnancy and delivered a healthy baby girl; however, she died 13 days after her delivery by cesarean section due to developing worsening heart failure. While there is data and support for IV prostacyclins in pregnancy, patients may opt for oral formulations, like in our case. Registry data on the use of oral prostacyclins and prostacyclin receptor agonists in pregnancy may help improve patient outcomes.