Medical management of pulmonary arterial hypertension in pregnancy: A single center experience

Background: Pulmonary arterial hypertension (PAH) is a disease characterized by increased pulmonary vascular resistance (PVR). Pregnancy in the setting of PAH is associated with significant maternal morbidity and mortality, typically present during labor and the early postpartum. Historically, observed maternal mortality has been as high as 56%. However, with improved recognition, understanding, and treatment of PAH, reassessment of maternal outcomes is of increasing interest. Study design: This is a retrospective observational case series of all women with a diagnosis of moderate to severe World Health Organization Group 1 PAH who had pregnancy managed in our tertiary care center between 2004 and 2018. Results: Ten women (Age 28 [SD 6] years, mean pulmonary artery pressure 48 [SD 16.5] mmHg, PVR 7.5 [SD 4.3] Wood units) were identified and included. Seven women (70%) had an antepartum diagnosis of PAH, 2 women (20%) were diagnosed during pregnancy and 1 (10%) postpartum. Four women (40%) delivered preterm, and 3 women (30%) had a successful vaginal delivery. Cardiovascular complications occurred in 4 women (40%) and non-cardiovascular complications occurred in 5 women (50%). There were no significant changes in right ventricular function or non-invasive assessment of PA pressures at long-term follow-up. All patients are alive to date at a mean follow-up of 8 [SD 4] years. Conclusion: In our single center, 10 women with moderate to severe PAH receiving directed medical therapy survived pregnancy and delivery. Further evaluation and review are required to optimize medical management for women with PAH who pursue pregnancy.