NCT07266519
Not yet recruiting
Phase 2
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
Philipps University Marburg0 sites25 target enrollmentStarted: July 1, 2026Last updated:
Overview
- Phase
- Phase 2
- Status
- Not yet recruiting
- Sponsor
- Philipps University Marburg
- Enrollment
- 25
- Primary Endpoint
- Assessment of the effect on pulmonary vascular resistance (PVR)
Overview
Brief 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.
Study Design
- Study Type
- Interventional
- Allocation
- Na
- Intervention Model
- Single Group
- Primary Purpose
- Treatment
- Masking
- None
Eligibility Criteria
- Ages
- 18 Years to 70 Years (Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- No
Inclusion Criteria
- •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
- •Normal blood counts for platelets and erythrocytes
- •Women of childbearing potential must be willing to remain abstinent or use adequate and reliable contraception throughout the study and for at least 4 months after the last dose of study medication has been received.
- •WOCBP must use one of the following highly effective methods of birth control that result in a low failure rate of less than 1% per year when used consistently and correctly - according to recommendations by the European Heads of Medicines Agencies - from at least 14 days before the first administration of study medication until 4 month after the last administration of study medication:
- •combined (estrogen and progestogen containing) hor-monal contraception associated with inhibition of ovulation:
- •intravaginal
- •transdermal
Exclusion Criteria
- •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.
- •History of severe bleeding/haemorrhage
- •Therapy with prostacyclin and/or antithrombotic agents
- •eGFR \<30 ml/min/m
- •Pregnancy or breastfeeding
- •Hypersensitivity to the active substance or to any of the excipients
Arms & Interventions
Single arm
Experimental
Treatment arm
Intervention: Sotatercept 0.3 mg/kg escalating to 0.7 mg/kg (Drug)
Single arm
Experimental
Treatment arm
Intervention: Sotatercept (Drug)
Outcomes
Primary Outcomes
Assessment of the effect on pulmonary vascular resistance (PVR)
Time Frame: 24 weeks
Change in PVR from screening at week 24 after 6-months of treatment with triple combination therapy in PAH patients
Secondary Outcomes
No secondary outcomes reported
Investigators
Similar Trials
Enrolling By Invitation
Phase 2
A Study to Evaluate the Safety,Tolerability,Pharmacokinetics and Pharmacodynamics of Cenerimod in Adult Chinese Participants With Moderate-to-severe Systemic Lupus Erythematosus (SLE)NCT07266090Viatris Pharmaceuticals Co., Ltd.15
Completed
Not Applicable
This is a study to evaluate dose-flexibility of Upadacitinib in Adult Subjects with Moderate to Severe Atopic Dermatitis2023-504869-23-00AbbVie Deutschland GmbH & Co. KG272
Recruiting
Phase 3
A Study to Select a Dose Regimen (Part A) and to Investigate Overall Survival (Part B) With Nanvuranlat Compared With Physician's Best Choice in Participants Aged 18 Years or Older With Biliary Tract CancerNCT07265674J-Pharma Co., Ltd.480
Recruiting
Phase 2
A Trial of SHR-7787 Injection Combined With Other Anti-tumor Drugs in Patients With Malignant Solid TumorsNCT07268040Shanghai Hengrui Pharmaceutical Co., Ltd.400
Not yet recruiting
Not Applicable
A Study of HY001N in the Treatment of Relapsed or Refractory Neurological Autoimmune DiseasesNCT07265206Juventas Cell Therapy Ltd.15