What is sotatercept studying for pulmonary arterial hypertension due to unrepaired congenital heart defects?

Sotatercept is being studied to see if adding it to usual medicines can improve lung and heart function in adults with PAH caused by unrepaired heart defects, including Eisenmenger syndrome. The research aims to find out if this treatment can help when standard vasodilator therapy alone is not enough. The study is led by Kazuya Hosokawa and conducted in Japan.

Who may be eligible for this pulmonary arterial hypertension clinical trial?

Adults aged 18 years or older with PAH due to unrepaired atrial or ventricular septal defect or patent ductus arteriosus may be eligible. Participants should have been on stable pulmonary vasodilator therapy for at least 90 days and have a walking ability of at least 100 meters. People with very severe symptoms or other major illnesses are excluded. Please review eligibility criteria for more details.

Is there any cost to participate in this pulmonary arterial hypertension clinical trial?

Study-related care, including the study drug and all trial visits, is provided at no cost. However, personal travel expenses may vary and are not guaranteed to be covered. Participants can expect to receive treatments and tests without charge during the study.

Actively Recruiting

Phase 4

Age: 18Years +

All Genders

ID07356778

Evaluating Sotatercept Added to Usual Pulmonary Arterial Hypertension Treatment in Adults with Unrepaired Congenital Heart Defects and Eisenmenger Syndrome

Led by Kazuya Hosokawa · Updated on 2026-01-21

Participants Needed

Research Sites

34 weeks

Total Duration

AI-Summary

What this Trial Is About

Researchers are evaluating whether adding sotatercept to standard medicines can help adults with pulmonary arterial hypertension PAH caused by unrepaired congenital heart defects such as atrial or ventricular septal defects and patent ductus arteriosus, including those with Eisenmenger syndrome. This condition affects lung blood vessels and oxygen levels, and the study focuses on adults whose PAH has not improved enough with current vasodilator treatments. The trial is a randomized Phase 4 study conducted in Japan, aiming to understand sotatercepts effects in this specific group. Participants will be randomly assigned in a 21 ratio to receive either sotatercept plus usual vasodilator therapy or vasodilator therapy alone for 24 weeks. Sotatercept is given as subcutaneous injections every 3 weeks, starting with a lower dose and increasing if safety criteria are met, with dose adjustments based on blood counts. All participants continue their stable PAH therapy, and changes are generally discouraged unless needed for safety. During the study, participants will attend visits at the start, week 12, and week 24 to undergo tests including a 6-minute walk test, blood tests, questionnaires, and heart-lung assessments used in routine PAH care. Researchers will monitor changes in walking distance, clinical events, and heart-lung function. Safety monitoring includes regular blood counts to manage potential side effects. The total participation lasts about six months, with study treatments and tests provided at no cost.

CONDITIONS

Brief Title

A Study of Sotatercept for Patients With Eisenmenger Syndrome or Unrepaired Shunt-Associated Pulmonary Arterial Hypertension Resistant to Vasodilator Therapy

Who Can Participate

Age: 18Years +

All Genders

Eligibility Criteria

You may qualify if you...

Adults aged 18 years or older
Diagnosis of unrepaired atrial septal defect, ventricular septal defect, or patent ductus arteriosus
At least 90 days of pulmonary vasodilator therapy
Pulmonary vascular resistance of 5 or more Wood units and mean pulmonary arterial pressure over 20 mm Hg on right heart catheterization within 180 days, or echocardiographic tricuspid regurgitation velocity over 3.4 m/s with right-to-left or bidirectional shunt plus resting oxygen saturation 92% or less indicating cyanosis
Baseline 6-minute walk distance of at least 100 meters
Ability to complete study questionnaires

You will not qualify if you...

WHO functional class IV symptoms or other unrepaired intracardiac shunts
Severe kidney, liver, or lung disease or left ventricular ejection fraction below 40%
Prior use of sotatercept
Contraindications to sotatercept according to its label
Judged unsuitable for the study by the investigator

Research Team

Kazuya Hosokawa, MD, PhD

Keimei Yoshida, MD, PhD

Not the Right Trial for You?

Explore thousands of other clinical trials that might be a better match.
Sign up to get personalized trial recommendations delivered to your inbox.

Already have an account? Log in here