Winrevair (sotatercept-csrk) / BMS, Merck (MSD) - LARVOL DELTA

Efficacy and Safety of Sotatercept in Patients with Pulmonary Arterial Hypertension (PAH) Associated with CTD: A Pooled Analysis of Three Phase 3 Trials (ACR Convergence 2026) - No abstract available

P3 data • Retrospective data • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Mitomycin-Associated Pulmonary Veno-Occlusive Disease Following Chemoradiation: A Cardio-Oncology Case Treated with Sotatercept (AHA 2026) - "Abstract is embargoed at this time."

Clinical • Cardiovascular • Hepatology

Sotatercept Improves Right Heart Mechanics Despite Reduced Myocardial Contractility in Pulmonary Arterial Hypertension: A Prospective Exercise Hemodynamic Study. (PubMed, Circulation) - P4 | "These data suggest that sotatercept causes an energetically favorable reduction in resting RV contractility to match the lower afterload, which does not appear physiologically detrimental when considering the concurrent improvement in RV free wall contractile mechanics and exertional right heart reserve. URL: https://www.clinicaltrials.gov; Unique identifier: NCT06409026."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Merck…announced the U.S. Food and Drug Administration (FDA) has approved an update to the U.S. product label for WINREVAIR (sotatercept-csrk) for injection, 45mg, 60mg, based on the Phase 3 HYPERION trial. (Merck (MSD) Press Release) - "Today’s approval updates the WINREVAIR label to include efficacy and safety data from the HYPERION trial evaluating adults newly diagnosed with PAH (WHO FC II or III, diagnosed within 12 months of study screening) at intermediate to high risk of disease progression...The ERS guidelines provide a 'strong' recommendation for the use of WINREVAIR in adult patients with PAH on background therapy who have not achieved low-risk status at follow up, including patients at intermediate-low, intermediate-high, or high risk, and grade the certainty of evidence for WINREVAIR for this recommendation as 'high,' out of four possible evidence scores (very low, low, moderate, or high)."

FDA approval • Pulmonary Arterial Hypertension

Sotatercept in Pediatric Pulmonary Hypertension: Promising Hemodynamic Findings, but Are We Extrapolating Too Far? (PubMed, Pediatr Cardiol) - "However, the small and heterogeneous patient cohort-which also included patients with Group 3 pulmonary hypertension and uncorrected Eisenmenger physiology-limits the generalizability of these findings. These points should be taken into account when evaluating the potential benefits of sotatercept in a broader pediatric population with pulmonary arterial hypertension."

Journal • Cardiovascular • Hypertension • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Late Breaking Abstract - Echocardiographic (ECHO) parameters with sotatercept in pulmonary arterial hypertension (PAH): a pooled long-term follow-up analysis (ERS 2026) - P2, P3 | "Sotatercept showed continued benefit in ECHO measures of RV size and function over 3 yrs in a broad range of participants, further supporting long-term benefit of sotatercept in PAH."

Late-breaking abstract • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Late Breaking Abstract - Development of intrapulmonary shunts in patients with pulmonary arterial hypertension treated with long-term sotatercept (ERS 2026) - "Moderate to severe pulmonary capillary dilatations may occur in PAH patients receiving long-term sotatercept and are associated with impaired oxygenation and reduced functional capacity."

Clinical • Late-breaking abstract • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Clinical Data Supporting Earlier Initiation of Sotatercept in PAH Patients on Top of Double Background Therapy (ERS 2026) - "Sponsored by (Merck) MSD"

Clinical data

Percutaneous Transluminal Peripheral Pulmonary Artery Biopsy (PT-PPAB): A Feasible and Safe Technique for the Diagnosis of Unidentified Pulmonary Artery Diseases (ERS 2026) - P=N/A | "PT-PPAB safely provides in vivo pulmonary arterial tissue, enabling histopathological assessment and supporting a histology-guided approach to PAH."

Biopsy • Cardiovascular • SMAD2 • TGFB1

Early Hemodynamic, Functional, and Regional Quantitative CT Response to Sotatercept in Pulmonary Hypertension With COPD and Systemic-Sclerosis: A Paired Case Analysis (ERS 2026) - "Sotatercept was associated with substantial hemodynamic and imaging improvement in COPD- and SSc-associated PH. Quantitative CT suggests upper-lobe–predominant vascular recruitment, supporting regional reversal of pulmonary vascular remodeling and restoration of physiologic perfusion gradients."

Clinical • Cardiovascular • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis • TGFB1

ERS clinical practice guidelines on the treatment of pulmonary arterial hypertension: new recommendations for medical therapy (ERS 2026) - "In this presentation, the updated evidence-based recommendations for the treatment of pulmonary arterial hypertension (PAH) will be discussed, focusing on the latest scientific evidence on sotatercept, an activin-signalling inhibitor targeting a novel pathway for the treatment of PAH."

Clinical • Clinical guideline • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Sotatercept in PAH: 12-Month Real-World Outcomes and Safety (ERS 2026) - "Most patients (93%) were receiving triple combination therapy with systemic prostacyclinsFollowing initiation of sotatercept, a progressive and sustained improvement in clinical, functional, and hemodynamic parameters was observed. In our real-world, sotatercept demonstrated a consistent efficacy and safety profile, in patients with advanced PAH receiving combination therapy."

Clinical • Real-world • Real-world evidence • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Treatment response to sotatercept in participants with pulmonary arterial hypertension at high risk of death: subgroup analysis by cardiometabolic comorbidity burden (ERS 2026) - P3 | "Sotatercept reduced the risk of major morbidity and mortality events vs placebo and had a consistent safety profile across the 0 and ≥1 CMC subgroups in the ZENITH trial."

Cardiovascular • Coronary Artery Disease • Diabetes • Hypertension • Metabolic Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases

Sotatercept for Pulmonary Arterial Hypertension on Background Therapy: An Updated Systematic Review and Meta-Analysis of Randomized Controlled Trials (ERS 2026) - "Sotatercept improves pulmonary hemodynamics, functional capacity, and clinical risk in PAH on background therapy, with predictable mechanism-related adverse effects."

Retrospective data • Review • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases • ACVR2A • TGFB1

Clinical Outcomes and Safety of Sotatercept in Pulmonary Arterial Hypertension: A Real- World Propensity-Matched Analysis (ERS 2026) - "In routine clinical practice, sotatercept was associated with improved survival and cardiovascular outcomes in PAH, with an overall acceptable safety profile despite increased mucosal bleeding."

Clinical • Clinical data • Real-world • Cardiovascular • Gastroenterology • Hematological Disorders • Hypertension • Pulmonary Arterial Hypertension • Respiratory Diseases • Thrombocytopenia

Haemodynamic Effect of Sotatercept and Parenteral Prostacyclins in Pulmonary Arterial Hypertension Patients (ERS 2026) - "Conclusions. The different haemodynamic effects observed with prostacyclins, with or without other approved PAH therapies, compared with sotatercept may be largely driven by differences in patients' baseline haemodynamic characteristics at treatment initiation and possibly differences in treatments' effect."

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

When Better Isn't Hemodynamically Better: Paradoxical Pulmonary Vascular Resistance Response Following Sotatercept Therapy (ERS 2026) - "In 20% of PAH patients treated with sotatercept, PVR increased despite improvement in mPAP and correction for Hct. This was largely driven by decreases in CO and PAWP."

Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Predictive value of early NTproBNP decline for hemodynamic improvement in sotatercept-treated patients with pulmonary arterial hypertension (ERS 2026) - "Conclusion While patients treated with sotatercept had similar reductions in mPAP independent of initial NTproBNP response, those with 30% response had improved PVR with nonsignificant increase in CI."

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Sotatercept as Add-On Therapy to Triple Combination Therapy with Parenteral Prostacyclin in Pulmonary Arterial Hypertension: Insights from the French Early Access Program (ERS 2026) - "This nationwide real-world experience confirms the effectiveness of sotatercept in PAH patients on background triple therapy including parenteral prostacyclin, with prostacyclin weaning undertaken in selected patients, while clinically relevant adverse events underscore the importance of careful monitoring."

Combination therapy • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Sotatercept in High-Risk PAH Patients Under Triple Combination Therapy, in a Greek PAH centre (ERS 2026) - "This real-world experience suggests sotatercept may provide additional clinical benefit in high-risk PAH patients unresponsive to maximal therapy, with early improvements in biomarkers, right ventricular performance, and exercise tolerance. Larger studies are needed to confirm safety and long-term efficacy."

Clinical • Combination therapy • Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Long-term safety and efficacy of sotatercept treatment for patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) (ERS 2026) - P2, P3 | "Sotatercept was generally well-tolerated with sustained efficacy in CTD-PAH over 3 yrs f/u."

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

Sotatercept Versus Other Add-on Therapies in Pulmonary Arterial Hypertension: a Bayesian Network Meta-Analysis (ERS 2026) - "Oral Treprostinil (MD 17m), Inhaled Iloprost (MD 26.0m), Selexipag (MD 8.3m), Ralinepag (MD 7.3m), and MK-5475 (MD -5.7m) showed non-significant effects (Figure 1). Sotatercept demonstrated the highest probability of being the most effective add-on therapy for improving 6MWD in PAH, supporting its positioning as preferred add-on for patients not achieving treatment goals."

Retrospective data • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

TGF-beta pathway: activin-signalling inhibitors (ERS 2026) - "Sotatercept is a first-in-class therapy targeting activin signalling that has been demonstrated to be highly effective in treating pulmonary arterial hypertension and was recommended for use across a wide spectrum of disease severity at the most recent World Symposium on Pulmonary Hypertension. The presentation will describe the transforming growth factor (TGF)-beta therapeutic pathway and the evidence base for sotatercept use."

Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • TGFB1

Specific BMPR2 variants may predict response to sotatercept in patients with heritable pulmonary arterial hypertension (ERS 2026) - "Our observations support the notion that BMPR2 mutation carriers are a heterogeneous population with varying clinical courses and varying responses to sotatercept treatment. We therefore speculate that mutations affecting different functional domains of BMPR2 may exhibit distinct features of pulmonary vascular remodeling, which may affect the response to therapy."

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Treatment response to sotatercept within 1 year of diagnosis of pulmonary arterial hypertension: subgroup analysis by cardiometabolic comorbidity burden (ERS 2026) - P3 | "Time to a first clinical worsening event was longer with sotatercept (vs placebo), with a generally well tolerated and consistent safety profile across CMC subgroups in HYPERION."

Cardiovascular • Coronary Artery Disease • Diabetes • Hypertension • Metabolic Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases