Multidisciplinary management of pregnant women with Group 1 pulmonary arterial hypertension resulted in a 5-year survival rate of 73%.
Observational (n=11)
No
In a modern cohort of pregnant women with PAH, intensive multidisciplinary care including parenteral prostacyclins resulted in a 73% 5-year survival, though mortality and care burden remain high.
Abstract Rationale Despite advancements in medical care for pulmonary arterial hypertension (PAH), pregnancy remains a significant risk to health and provides unique management challenges. In the modern PAH treatment era, multiple reports note improved maternal and fetal outcomes. Multidisciplinary management of these complex patients is imperative. This series describes the management and outcome of women with Group 1 PAH and pregnancy who received care at our tertiary care center. Methods Patients who received care for pregnancy at our institution between 2009-2025 and who had Group 1 PAH diagnosed before or during pregnancy were identified. Data were collected from our electronic health system including demographics, PAH history, echocardiogram and cardiac catheterization reports from before, during and after pregnancy, peripartum course and post-partum follow-up and management. Results Eleven patients were identified with PAH complicating pregnancy, five of whom had PAH diagnosed during pregnancy. The median age at PAH diagnosis and delivery were 28 and 26 years, respectively. The five incident cases all presented with right heart failure; median PVR at diagnosis was 9.9 WU. For prevalent cases, median PVR was 5.7 WU (unavailable in one). At delivery, 9 patients were on an intravenous prostacyclin analog (PCA) and 8 on a phosphodiesterase type 5 inhibitor (PDE5i). Delivery was largely by cesarean section at approximately 34 weeks. For the hospitalization including delivery, median hospital length of stay was 16 days. One patient died within 90 days of delivery. Two additional patients died due to complications of PAH at 2 and 4 years post-delivery. Overall, 5-year survival was 73%. Conclusions Mortality from pregnancy and PAH was relatively low in patients managed in our tertiary care center between 2009-2025 and aligns with other recently published reports. One important finding is that patients presenting with incident PAH during pregnancy tended to have more severe disease and accounted for the sole case of peripartum mortality. Notably, all patients’ care involved intensive multidisciplinary engagement and collaboration and, in 9 of 11, parenteral prostacyclin therapy. Patients themselves experienced a high burden of care, with a median post-delivery hospital stay of 2 weeks. Current guidelines recommend women with PAH considering pregnancy receive counseling from a physician with expertise in PAH who can facilitate shared decision making. We re-emphasize this recommendation and note that mortality remains unacceptably high, though high-risk perinatal care and PAH management continue to advance. This abstract is funded by: None
Kolousek et al. (Fri,) conducted a observational in Group 1 Pulmonary Arterial Hypertension in pregnancy (n=11). Multidisciplinary management was evaluated on 5-year survival. Multidisciplinary management of pregnant women with Group 1 pulmonary arterial hypertension resulted in a 5-year survival rate of 73%.