Congenital Heart Disease (CHD)Phase 2CHASENCT07498803
Sota-ES - Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome
A Phase 2 study testing Sotatercept in people with Congenital Heart Disease (CHD) and Eisenmenger Syndrome, run by Philipps University Marburg.
Trial summary
The present study seeks to provide pilot data on the safety and efficacy of medical therapy with sotatercept in patients with an established diagnosis of congenital heart disease and Eisenmenger syndrome. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Congenital Heart Disease (CHD), Eisenmenger Syndrome, PAH
- What is being tested?
- Sotatercept
- Type of treatment
- Injection or infusion
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 40 participants
- Where
- No sites listed yet
- Study dates
- 1 Sep 2026to 31 Dec 2028
- Phase
- Phase 2
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- Age ≥18 years
- Congenital heart disease with Eisenmenger syndrome (known unrepaired atrial septal defect, and/or ventricular septal defect, and/or patent ductus arteriosus; patients with anomalous pulmonary venous drainage will not be considered)
- Eisenmenger syndrome defined as right-to-left or bi-directional shunt with a mPAP >25 mmHg, PAWP < 15 mmHg, and PVR >5 WU
- In patients with pre-tricuspid shunt, the consideration of Eisenmenger syndrome requires one of the following: Systemic arterial O2 saturation (SaO2) at rest <88% and more than 70%, and/or SaO2 <80% during 6MWT, and secondary erythrocytosis (Hb > 15.0 g/dl for females and 16.0 g/dl for males)
- On stable doses of background PAH therapy* and diuretics (i.e., patient-individual dose goal for each therapy achieved) for ≥30 days
- 6-minute walking distance >100 m
- WHO-FC II or III
- Written informed consent
Who cannot join
- Age <18 years
- Diagnosis of pulmonary hypertension groups 2, 3, 4, or 5
- Hospitalization or change in PAH background therapies within 30 days prior to screening (changes in dose of diuretics or parenteral prostanoids [<10% change in infusion rate over the preceding 3 months] are allowed)
- Uncontrolled systemic hypertension as evidenced by sitting systolic blood pressure >160 mmHg or sitting diastolic blood pressure >100 mmHg during screening visit after a period of rest
- Baseline systolic blood pressure <90 mmHg at screening
- Left ventricular systolic dysfunction (LVEF 40%)
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- Restrictive lung disease with a TLC < 60% AND demonstration of more than mild fibrosis on chest CT prior to enrolment (note that patients with congenital heart disease may have thoracic cage deformities [e.g. pectus] that may lead to thoracic cage restriction in the absence of parenchymal lung disease).
- Obstructive lung disease (FEV1 < 60% pred. and FEV1/FVC <60%)
- Chronic thromboembolic disease (intermediate or high probability) on V/Q scan and evidence of distal thromboembolism on CT angiography (note that proximal in-situ thrombosis is a known complication of Eisenmenger's and will not be considered an exclusion criterion - such patients may be reviewed centrally for inclusion)
- Significant liver disease (Child II or III)
- Any of the following clinical laboratory values at the screening visit:
- Estimated glomerular filtration rate (eGFR) <30 mL/min/m2 (as defined by the Modification of Diet in Renal Disease [MDRD] equation)
- Serum alanine aminotransferase, aspartate aminotransferase, or total bilirubin levels >3 × ULN (bilirubin criterion waived if there is a documented history of Gilbert's syndrome)
- Baseline platelet count <50,000/µl (<50.0 x 109/L) at screening
- Documented episodes of previous repetitive hyperviscosity syndrome
- History of haemoptysis within 12 months prior to screening, and/or repeated severe epistaxis (≥ 1 episode per month)
- Uncorrected iron deficiency (iron stores must be normal defined as a Ferritin > 100 and transferrin saturation [TSAT] ≥ 20%)
- Recent (< 3 months) enrolment into a rehabilitation program
- Untreated sleep disordered breathing with an AHI > 10. Patients with sleep apnea will need to have been on CPAP or BiPAP for 3 months prior to enrolment.
- Prior or current exposure to sotatercept or luspatercept
- Treatment by phlebotomy within 1 month prior to screening
- Participation in another interventional study
- Pregnant (serum B-HCG) or lactating women
Trial locations and contacts
No sites are listed yet. Contact the study team or a site near you to ask about taking part.
About this study
Official title: Sota-ES - A Prospective, Non-randomized, Open-label, Multi-center Study of the Activin Signaling Inhibitor Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome
The present study seeks to provide pilot data on the safety and efficacy of medical therapy with sotatercept in patients with an established diagnosis of congenital heart disease and Eisenmenger syndrome.
CHASE is an interventional, single-arm, open-label study, that will enroll 40 patients with an established diagnosis of CHD and Eisenmenger syndrome. PAH background therapy may be present at the discretion of the investigators at the time of enrolment. CHASE will be performed only in countries where standard PAH therapies are available and reimbursed. At the end of the 24-week patient period, PAH treatment is left to the investigator's discretion.
- Conditions
- Congenital Heart Disease (CHD), Eisenmenger Syndrome, PAH
- Being tested
- Sotatercept
- Keywords
- Congenital heart disease with Eisenmenger syndrome, WHO-FC II or III, PAH therapy
- Medical subject headings
- Heart Defects, Congenital, Eisenmenger Complex
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
SotaterceptDrug
Sotatercept will be administered subcutaneously in 3-weekly intervals at study sites beginning at a dose of 0.3 mg/kg body weight followed by up titration to the target dose of 0.7 mg/kg body weight.
| Study group | Role | What they receive |
|---|---|---|
| Single arm | Experimental | Sotatercept will be administered subcutaneously in 3-weekly intervals at study sites beginning at a dose of 0.3 mg/kg body weight followed by up titration to the target dose of 0.7 mg/kg body weight Receives: Drug: Sotatercept |
What is being measured
The primary outcome is the main question the study is designed to answer.
Assessment of the effect on pulmonary vascular resistance (PVR)
Change in PVR from screening at week 24 after 24 weeks of treatment with Sotatercept
Measured: 24weeks
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted27 Mar 2026
- Study start1 Sep 2026 (estimated)
- Primary completion31 Mar 2028 (estimated)
- Study completion31 Dec 2028 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Congenital Heart Disease (CHD)Studies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase 2
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Model description
- This is an interventional, prospective, non-randomized, single-arm, open-label, multi-center, phase II study.
- Masking
- None (open label)
- Enrolment
- 40 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Philipps University Marburg
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Stephan Rosenkranz, Prof. Dr., Principal investigator, University Hospital Cologne Department of Cardiology, Pulmonology, and Intensive Care Medicine Heart Center Cologne Cardiovascular Research Center (CCRC) Center for Molecular Medicine Cologne (CMMC)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07498803
- Sponsor's protocol ID
- KKS-344
- Other IDs
- 2026-525914-68-00
- Acronym
- CHASE
- Status last verified
- Mar 2026
- First submitted
- 19 Mar 2026
- Last updated
- 27 Mar 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 27 Mar 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Sota-ES - A Prospective, Non-randomized, Open-label, Multi-center Study of the Activin Signaling Inhibitor Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome
- ClinicalTrials.gov ID
- NCT07498803
- Acronym / sponsor ID
- CHASE / KKS-344
- Conditions
- Congenital Heart Disease (CHD), Eisenmenger Syndrome, PAH
- Keywords
- Congenital heart disease with Eisenmenger syndrome, WHO-FC II or III, PAH therapy
- Phase
- Phase 2
- Study type
- Interventional
- Recruitment status
- Not yet recruiting
- Enrolment
- 40 participants (estimated)
- Lead sponsor
- Philipps University MarburgUniversity, hospital or other
- Study start
- 1 Sep 2026 (estimated)
- Primary completion
- 31 Mar 2028 (estimated)
- Study completion
- 31 Dec 2028 (estimated)
- First posted
- 27 Mar 2026 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
The present study seeks to provide pilot data on the safety and efficacy of medical therapy with sotatercept in patients with an established diagnosis of congenital heart disease and Eisenmenger syndrome.
CHASE is an interventional, single-arm, open-label study, that will enroll 40 patients with an established diagnosis of CHD and Eisenmenger syndrome. PAH background therapy may be present at the discretion of the investigators at the time of enrolment. CHASE will be performed only in countries where standard PAH therapies are available and reimbursed. At the end of the 24-week patient period, PAH treatment is left to the investigator's discretion.
Study design
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
- Model description
- This is an interventional, prospective, non-randomized, single-arm, open-label, multi-center, phase II study.
| Arm | Type | Receives |
|---|---|---|
| Single arm | Experimental | Drug: SotaterceptSotatercept will be administered subcutaneously in 3-weekly intervals at study sites beginning at a dose of 0.3 mg/kg body weight followed by up titration to the target dose of 0.7 mg/kg body weight |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
Key inclusion criteria (8 listed)
- Age ≥18 years
- Congenital heart disease with Eisenmenger syndrome (known unrepaired atrial septal defect, and/or ventricular septal defect, and/or patent ductus arteriosus; patients with anomalous pulmonary venous drainage will not be considered)
- Eisenmenger syndrome defined as right-to-left or bi-directional shunt with a mPAP >25 mmHg, PAWP < 15 mmHg, and PVR >5 WU
- In patients with pre-tricuspid shunt, the consideration of Eisenmenger syndrome requires one of the following: Systemic arterial O2 saturation (SaO2) at rest <88% and more than 70%, and/or SaO2 <80% during 6MWT, and secondary erythrocytosis (Hb > 15.0 g/dl for females and 16.0 g/dl for males)
- On stable doses of background PAH therapy* and diuretics (i.e., patient-individual dose goal for each therapy achieved) for ≥30 days
Key exclusion criteria (23 listed)
- Age <18 years
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| SotaterceptSotatercept will be administered subcutaneously in 3-weekly intervals at study sites beginning at a dose of 0.3 mg/kg body weight followed by up titration to the target dose of 0.7 mg/kg body weight. | Drug | Single arm |
Primary outcome
Assessment of the effect on pulmonary vascular resistance (PVR)
Time frame: 24weeks
Change in PVR from screening at week 24 after 24 weeks of treatment with Sotatercept
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 27 Mar 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Stephan Rosenkranz, Prof. Dr. principal investigator
University Hospital Cologne Department of Cardiology, Pulmonology, and Intensive Care Medicine Heart Center Cologne Cardiovascular Research Center (CCRC) Center for Molecular Medicine Cologne (CMMC)
Questions about this trial
What is this clinical trial studying?
A Phase 2 study testing Sotatercept in people with Congenital Heart Disease (CHD) and Eisenmenger Syndrome, run by Philipps University Marburg.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 8 inclusion and 23 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: opening soon, as of the sponsor's last update on 27 Mar 2026. Status can change between updates, so confirm with the study contact before making plans.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact CHASE Study Team (+4915114980915, chase@kks.uni-marburg.de), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Assessment of the effect on pulmonary vascular resistance (PVR) (time frame: 24weeks). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 27 Mar 2026.