Pregnancy and pulmonary arterial hypertension: A case series and literature review.

Abstract Background Despite the development of advanced therapies for pulmonary arterial hypertension (PAH), pregnancy remains contraindicated in these patients due to high maternal and fetal morbidity and mortality. Limited data exists regarding pregnancy management and outcome in this unique patient population. We describe a series of pregnant patients diagnosed with PAH prior to or during pregnancy who were delivered at a tertiary center with a comprehensive and established pulmonary vascular disease program. Objective Describe a single institution's experience and review the existing literature for pregnancy management and outcomes in patients with PAH. Study Design A review of all patients with PAH who were admitted for delivery between 2005-2019 at our institution was performed. All data was extracted from the electronic health record and included patient demographics, PAH subtype, PAH-targeted therapies, and mode of delivery and anesthesia. Results Seven patients were identified. Five patients had a pre-partum diagnosis of PAH, while two patients were diagnosed with PAH during the third trimester. All patients were started on prostacyclins and the majority were on combination PAH-targeted therapy. The maternal mortality rate was 29%. Elective caesarean delivery was performed in over 70% of cases, while one patient required an urgent caesarean section and one patient had a successful vaginal delivery. The majority of patients had epidural anesthesia. Two patients required extracorporeal membrane oxygenation postpartum and both died. There were no cases of neonatal mortality. Conclusion Our cases series and the published literature to date show that pregnancy in PAH remains poorly tolerated despite significant advancements in PAH-targeted therapies and postpartum care. A multidisciplinary team approach remains essential for the management of these patients.