Despite robust evidence in adult patients, up to now there was limited data on the use of sotatercept in pediatric pulmonary hypertension patients. The findings of a multi-center, retrospective study of children and young adults ≤ 21 years of age with paediatric pulmonary hypertension, treated by programs in the Pediatric Pulmonary hypertension Network (PPHNet) and institutional registries, was published on October 5 in the CHEST Jounral.
Sotatercept add-on therapy was well tolerated and associated with improvements in Functional Class (FC), biomarkers, echocardiographic parameters, 6 minute walking distance 6MWD, and invasive hemodynamics including mean pulmonary artery pressure and pulmonary vascular resistance.
The findings provide the largest supportive evidence base to date for sotatercept use in pediatric pulmonary hypertension. Adverse events occurred in 33 (50%) subjects with bleeding in 15 (23%). No patients had escalation of pulmonary hypertension therapy, were hospitalized for clinical worsening, underwent lung transplant, or died. Parenteral prostacyclin was weaned or discontinued in 13 patients.
PDF with full text available at this link on the CHEST Journal
Citation
Critser P, Krishnan U, Avitabile C …Sotatercept in Pediatric Pulmonary Hypertension: Experience from the Pediatric Pulmonary Hypertension Network.CHEST, 2026; 0