SPC365

Early Real-World Use of Sotatercept in Pulmonary Arterial Hypertension

Session: Sessão de Posters 57 - Hipertensão arterial pulmonar: estratificação de risco, hemodinâmica e prognóstico
Speaker: Tiago Miguel Peixoto

Congress: CPC 2026
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme: 21.4 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure - Treatment
Session Type: Posters Eletrónicos
FP Number: ---
Authors: Tiago Miguel Oliveira Peixoto; Bruno Bragança; Fabienne Gonçalves; Luísa Carvalho; Inês Furtado; José Moura Meireles; Alzira Melo; Joana Alves; Mário Santos

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong><span style="font-family:&quot;Times New Roman&quot;,serif">Introduction: </span></strong><span style="font-family:&quot;Times New Roman&quot;,serif">Pulmonary arterial hypertension (PAH) is a progressive disease marked by pulmonary vascular remodeling and right heart failure. Although combination therapy targeting the endothelin, nitric oxide, and prostacyclin pathways has improved care, outcomes remain suboptimal. Sotatercept, a first-in-class activin-signaling inhibitor that modulates TGF-</span><span style="font-family:&quot;Times New Roman&quot;,serif">&beta;</span><span style="font-family:&quot;Times New Roman&quot;,serif"> pathways, has shown marked benefits in trials - improving hemodynamics, exercise capacity, and clinical outcomes. We report real-world data from the first eight patients treated with sotatercept at a PAH expert center.</span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong><span style="font-family:&quot;Times New Roman&quot;,serif">Methods: </span></strong><span style="font-family:&quot;Times New Roman&quot;,serif">We conducted a retrospective, longitudinal, single-centre analysis of all patients with PAH that started sotatercept treatment between January and December 2025. All patients fulfilled diagnostic and hemodynamic criteria as per 2022 ESC/ERS Guidelines for group 1 pulmonary hypertension. Baseline assessment included demographical and clinical data, NT-proBNP, echocardiography, and right heart catheterization. Sotatercept was administered subcutaneously every 3 weeks (starting dose 0.3 mg/kg, titrated to 0.7 mg/kg) in addition to previous background PAH therapy. Follow-up evaluations at 6 months included clinical, laboratory, and hemodynamic reassessment, as well as systematic safety monitoring for adverse events.</span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong><span style="font-family:&quot;Times New Roman&quot;,serif">Results: </span></strong><span style="font-family:&quot;Times New Roman&quot;,serif">Eight patients were included (75% female, mean age 46.5 &plusmn; 13.7 years). Etiologies consisted of connective tissue disease&ndash;associated PAH, idiopathic PAH, pulmonary veno-oclusive disease, and congenital heart disease-associated PH. Disease duration averaged 7 years. </span><span style="font-family:&quot;Times New Roman&quot;,serif">At baseline, all patients were receiving an endothelin receptor antagonist (ERA), 7 were on PDE5 inhibitors, 5 on selexipag and 3 on prostacyclin analogues as background PAH therapy. </span><span style="font-family:&quot;Times New Roman&quot;,serif">Paired invasive hemodynamics were available in 4 patients. PVR decreased significantly from 14.2 to 8.1 Wood units (mean change &minus;6.03 WU; 95% CI &minus;10.38 to &minus;1.67; p = 0.02). Mean pulmonary artery pressure declined from 69.3 &plusmn; 18.0 to 50.8 &plusmn; 22.3 mmHg (&minus;18.5 mmHg; p = 0.12). Cardiac index increased from 2.64 to 3.03 L/min/m<sup>2</sup> (+0.39; p = 0.11). Functional outcomes also trended positively: 6MWD improved by 86 m (p=0.18). WHO functional class improved in all patients that completed the 6-month follow-up evaluation (figure). Echocardiographic follow-up data trended toward higher TAPSE and higher FAC. Sotatercept was well tolerated: Hemoglobin rose by 1.6 g/dL (p = 0.08). One patient reported minor epistaxis and another one developed mild pericardial effusion</span><span style="font-size:8.0pt">&nbsp;</span><span style="font-family:&quot;Times New Roman&quot;,serif">, with no severe adverse effects. </span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong><span style="font-family:&quot;Times New Roman&quot;,serif">Discussion/Conclusions:</span></strong> <span style="font-family:&quot;Times New Roman&quot;,serif">In this early real-world cohort, sotatercept effects on functional class, 6MWD, cardiac function, and hemodynamics mirrored those reported in trials. Hemoglobin increased as anticipated, and bleeding events were minor. These results align with trial evidence and support sotatercept use in clinical practice.</span></span></span></p>