Sotatercept initiation in a real-world PAH cohort significantly improved 6-minute walk distance (from 238 to 301m) and right ventricular dilation (p<0.05), with 87.5% remaining clinically stable.
Cohort (n=16)
Does sotatercept improve functional class, hemodynamics, and echocardiographic outcomes in patients with Group 1 Pulmonary Arterial Hypertension?
In a real-world cohort of PAH patients, sotatercept improved 6-minute walk distance, RV remodeling, and hemodynamics, supporting its role as a disease-modifying therapy.
p-value: p=<0.05
Abstract Sotatercept, a first-in-class activin signaling inhibitor, has shown efficacy in PAH in randomized trials. However, real-world evidence remains limited. We evaluated functional classes, biomarker, echocardiographic, hemodynamic, and safety outcomes in patients treated with sotatercept. We folowed 16 PAH patients initiated on sotatercept. Baseline demographics, right heart catheterization (RHC), 6-minute walk distance (6MWD), BNP, hemoglobin, echocardiography, and clinical outcomes were collected. Paired comparisons (pre vs. post-sotatercept) were assessed using t-tests and Wilcoxon signed-rank tests. Patients were followed for therapy escalation, and changes in their baseline parameters over time. Sotatercept initiation was associated with improvement in clinical and hemodynamic metrics, figure 1, with statistical significance(p 0.05) seen in 6MWD and RV Dilation. Median sotatercept exposure was 406 days (mean 326). At last follow up; one patient (6.25%) required PAH therapy up titration; 14/16 (87.5%) remained stable without escalation, 4(25%) were hospitalized for respiratory failure and 1 patient passed away due to progression of PAH. RAP decreased from 7.83 to 3.67mmHg (n = 6, Δ-53.1%), PASP from 65.83 to 53mmHg (n = 6, Δ-19.5%), and mPAP from 40.50 to 33.17mmHg (n = 6, Δ-18.1%). PVR increased from 6.93 to 7.39WU (n = 6, Δ + 6.6%, p = 0.84). BNP improved from 130 to 72pg/mL (n = 15, Δ-44.6%). Oxygen requirement fell 3.27 to 2.93 l/min (n = 15, Δ-10.4%), and 6MWD improved 238 to 301m (n = 12, Δ + 26.4%). FC distribution improved (n = 15): the proportion of patients in NYHA class IV decreased from 1 to 0, class III from 6 to 5, class II 8 to 7, and class I increased from 0 to 3 patients. TTE demonstrated improvement in RV remodeling: the number of patients with no RV dysfunction increased from 3 to 9, while those with mild-to-moderate dysfunction decreased from 8 to 2. Similarly, no RV dilation increased from 1 to 6 patients, mild-to-moderate fell from 6 to 4, and moderate-to-severe dilation fell from 6 to 3, in figure 1. Additionally, there was an average increase in hemoglobin of + 1.7g/dL. In this real-world PAH cohort, most patients, 14/16(87.5%) did not require up titration of background therapy and 15/16(93.75%) remained alive and transplant-free at follow up with initiation of sotatercept. In addition, sotatercept was associated with favorable trends in BNP reduction, improvement in FC distribution, RAP, mPAP, PASP, echocardiographic RV remodeling, and clinical stability without therapy escalation. PVR was noted to have slightly increased, but this was non-significant, p = 0.84, due to the small sample size. These findings support sotatercept as a disease-modifying therapy in PAH. This abstract is funded by: None
Patel et al. (2026) conducted a cohort in Group 1 Pulmonary Arterial Hypertension (n=16). Sotatercept vs. Baseline (pre-sotatercept) was evaluated on Clinical and hemodynamic metrics including 6-minute walk distance and RV dilation (p=<0.05). Sotatercept initiation in a real-world PAH cohort significantly improved 6-minute walk distance (from 238 to 301m) and right ventricular dilation (p<0.05), with 87.5% remaining clinically stable.