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.
Age 18- 70 years
Treatment-naïve patients diagnosed within 12 weeks prior to screening with I/H/D-PAH or PAH associated with corrected congenital heart disease (disease (≥12 months after correction). Patients who have received treatment with PDE5is and/or ERAs for up to 6 weeks after diagnosis are eligible provided that state-of-the-art right heart catheterization has been performed at PAH diagnosis.
PVR >5 WU, mPAP ≥35 mmHg and PAWP or LVEDP ≤15 mmHg at PAH diagnosis.
Signed written informed consent
Other Forms of PH or PAH
Use of PAH medications for more than 6 weeks prior to screening
Symptoms or signs of clinically relevant lung disease, including TLC < 70%, FEV1/FVC <60%, and DLCO <45%, respectively
Signs of left heart failure with reduced or preserved ejection fraction, including LVEF <50%, LAVI >34 ml/m2, E/é ≥15, or permanent atrial fibrillation, respectively.
Single group
Sotatercept 0.3 mg/kg escalating to 0.7 mg/kg
Drugsubcutaneously administration in 3-weekly intervals
Sotatercept
DrugPatients 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
Assessment of the effect on pulmonary vascular resistance (PVR)
Change in PVR from screening at week 24 after 6-months of treatment with triple combination therapy in PAH patients
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Philipps University Marburg
Lead sponsor
MSD Sharp & Dohme GmbH, Germany
Collaborator
This trial is not recruiting at the moment. You can still explore other options:
