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

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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

NCT07266519•Philipps University Marburg

View on ClinicalTrials.gov

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.

Eligibility

Age

18 - 70 years

Sex

ALL

Healthy Volunteers

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:
•* oral
•* intravaginal
+15 more criteria

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/m2.
•Pregnancy or breastfeeding
•Hypersensitivity to the active substance or to any of the excipients

Key Dates

Start Date

July 1, 2026

Primary Completion Date

October 1, 2028

Completion Date

October 1, 2028

Last Updated

December 5, 2025

Enrollment

ESTIMATED participants

Conditions

Pulmonary Arterial Hypertension (PAH)

Interventions

Sotatercept 0.3 mg/kg escalating to 0.7 mg/kg

DRUG

Sotatercept

DRUG

Contacts

SIRIUS Study Team

CONTACT

+4915114980915 sirius@kks.uni-marburg.de

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Data Source & Attribution

This clinical trial information is sourced from ClinicalTrials.gov, a service of the U.S. National Institutes of Health.

ClinicalTrials.gov last update: December 5, 2025Data synced to Clareo: March 29, 2026

Modifications: This data has been reformatted for display purposes. Eligibility criteria have been parsed into inclusion/exclusion sections. Location data has been geocoded to enable distance-based search. For the authoritative and most current information, please visit ClinicalTrials.gov.

Neither the United States Government nor Clareo Health make any warranties regarding the data. Check ClinicalTrials.gov frequently for updates.

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

Inclusion Criteria

Exclusion Criteria

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