Background: Primary pulmonary hypertension carries a significant mortality risk during pregnancy and delivery.
Case: A 36-year-old pregnant woman with primary pulmonary hypertension was transferred to us with severe dyspnea. Intravenous epoprostenol was started, titrated, and maintained until labor augmentation. Because systemic epoprostenol treatment can interfere with platelet aggregation, we switched to inhaled epoprostenol, administered under a U.S. Food and Drug Administration-approved investigational new drug license, before epidural catheter placement. The inhaled drug was continued because it achieved better control of pulmonary hypertension. An uneventful forceps-assisted vaginal delivery was performed, and intravenous epoprostenol was restarted after the delivery. Mother and baby were well 6 months postpartum.
Conclusion: Intravenous epoprostenol treatment is effective in management of pregnant and postpartum women with primary pulmonary hypertension. Inhaled epoprostenol was effective during the intrapartum and immediate postpartum period.