NCT07266519

SIRIUS - Initial Combination Therapy With an Endothelin Receptor Antagonist, a Phosphodiesterase-5 Inhibitor and Sotatercept in Patients With Newly Diagnosed Pulmonary Arterial Hypertension

SIRIUS - A Prospective, Non-randomized, Open-label, Proof-of-concept Study of Initial Combination Therapy With an Endothelin Receptor Antagonist, a Phosphodiesterase-5 Inhibitor and Sotatercept in Patients With Newly Diagnosed Pulmonary Arterial Hypertension A Single-arm, Multi-center, Interventional Phase II Study

• Status: Not Yet Recruiting
• Phase: Phase 2
• Study type: Interventional
• Enrollment: 25 (estimated)
• Sponsor: Philipps University Marburg · Academic / Other
• Sex: All
• Age: 18 Years – 70 Years
• Healthy volunteers: Not accepted

Summary

The study aims to see how 24 weeks of triple therapy-an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor (PDE5i), and sotatercept-affects pulmonary vascular resistance (PVR) in patients with newly diagnosed pulmonary arterial hypertension (PAH). SIRIUS is a 24-week, single-arm, open-label study with up to 42 days of screening and a 28-day safety follow-up. It will enroll 25 patients and will be conducted only in countries where all treatments are available and covered. After 24 weeks, PAH treatment is decided by the doctor.

Conditions

• Pulmonary Arterial Hypertension (PAH)

Interventions

Type	Name	Description
DRUG	Sotatercept 0.3 mg/kg escalating to 0.7 mg/kg	subcutaneously administration in 3-weekly intervals
DRUG	Sotatercept	Patients are traitment-naiive before start of study. Therapy will be a triple combination therapy with an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor (PDE5i), and Sotatercept

Timeline

• Start date: 2026-07-01
• Primary completion: 2028-10-01
• Completion: 2028-10-01
• First posted: 2025-12-05
• Last updated: 2025-12-05