Immediate postpartum administration of sotatercept in a 40-year-old female with severe heritable PAH was feasible, well tolerated, and associated with functional and hemodynamic improvement.
Case Report (n=1)
Immediate postpartum administration of sotatercept as part of quadruple therapy was feasible and well-tolerated in a patient with severe heritable PAH diagnosed in late pregnancy.
Abstract Introduction Pregnancy in pulmonary arterial hypertension (PAH) carries high morbidity and mortality, particularly when PAH is newly diagnosed during pregnancy. Management requires supportive care, PAH-specific therapies including prostacyclin analogues and phosphodiesterase-5 inhibitors, multidisciplinary delivery planning, and monitoring for decompensation and right ventricular (RV) failure. Sotatercept, a novel activin signaling inhibitor approved for treatment of PAH, improves exercise capacity and World Health Organization functional class (FC), and reduces the risk of clinical worsening events. While sotatercept is contraindicated in pregnancy and breastfeeding, experience with postpartum use is limited. We present a case of heritable PAH, newly diagnosed with high-risk features and RV failure in late pregnancy, successfully managed with immediate postpartum sotatercept. Case A 40-year-old female with a family history of PAH, gravida 6 para 5 at 31 weeks gestation, presented with progressive dyspnea, edema, and hypoxemia requiring intensive care for high-flow supplemental oxygen. Echocardiography revealed severe RV dilation, moderately reduced RV systolic function, and estimated systolic pulmonary artery (PA) pressure of 96 mmHg. Right heart catheterization confirmed severe precapillary pulmonary hypertension (Table 1). She was started on inhaled nitric oxide and then initiated on intravenous epoprostenol and oral sildenafil, however had minimal improvement in oxygenation despite rapid titration of epoprostenol. After extensive multidisciplinary discussions, she underwent induction of labor with epidural anesthesia and forceps-assisted delivery at 32 weeks, with venoarterial extracorporeal membrane oxygenation on standby. Pulmonary hemodynamics and oxygenation did not immediately improve after delivery. Sotatercept was first administered on postpartum day five. She transitioned to subcutaneous treprostinil, initiated ambrisentan, weaned off oxygen, and was discharged with continued sotatercept every three weeks as part of upfront quadruple therapy. Genetic testing revealed a pathogenic bone morphogenetic protein receptor type II mutation. Over several months, she had improved FC, hemodynamics, and risk scores (Table 1), without any significant bleeding. Discussion We report the first case of near-immediate postpartum administration of sotatercept. In this case, the severity of disease at diagnosis and strong suspicion for heritable PAH prompted rapid initiation of quadruple therapy. The etiology of symptomatic, functional, and hemodynamic improvement over the subsequent months was multifactorial, reflecting the combined effects of targeted PAH therapy and postpartum physiologic changes. While the effect of sotatercept cannot be isolated, this case illustrates that early initiation postpartum is feasible and well tolerated. As sotatercept enters broader clinical use, further research is needed in this unique population regarding timing of initiation, bleeding risk, lactation safety, and long-term outcomes. This abstract is funded by: None
Lynn et al. (Fri,) conducted a case report in Heritable Pulmonary Arterial Hypertension (n=1). Sotatercept was evaluated on Functional, hemodynamic, and risk score improvement. Immediate postpartum administration of sotatercept in a 40-year-old female with severe heritable PAH was feasible, well tolerated, and associated with functional and hemodynamic improvement.