Adding sotatercept to usual therapy for adults with Eisenmenger syndrome or unrepaired shunt-related pulmonary arterial hypertension

Is this trial a fit for you?

A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.

What this trial studies

This randomized phase 4 study enrolls about 36 adults in Japan whose pulmonary arterial hypertension from unrepaired atrial/ventricular septal defects or patent ductus arteriosus remains insufficiently controlled on vasodilator therapy. Participants are randomized 2:1 to receive sotatercept plus their usual vasodilator-based PAH care or to continue vasodilator-based care alone for 24 weeks. Sotatercept is given by injection every three weeks and all participants have clinic visits with tests at baseline, week 12, and week 24. The study is limited to ambulatory adults without very severe functional limitation (WHO class IV) or major comorbid organ disease.

Why it matters

Who can join: the full rules

Inclusion Criteria:

* adults (≥18 years)
* unrepaired ASD, VSD or PDA
* ≥90 days of pulmonary vasodilator therapy; and either (i) pulmonary vascular resistance (PVR) ≥5 Wood units and mean pulmonary arterial pressure (mPAP) \> 20 mm Hg on right heart catheterization within 180 days, or (ii) echocardiographic tricuspid regurgitation velocity \>3.4 m/s with right-to-left/bidirectional shunt plus resting SpO₂ ≤92% consistent with cyanosis
* baseline 6MWD ≥100 m
* ability to complete questionnaires

Exclusion Criteria:

* WHO functional class IV; other unrepaired intracardiac shunts
* severe renal/hepatic/parenchymal lung disease or LVEF \<40%
* prior sotatercept use
* contraindication to sotatercept per label
* investigator-judged unsuitability

Where it's running

• Kyushu University Hospital
  Fukuoka, Not Required For This Country, Japan Recruiting
• The Second Department of Internal Medicine, University of Occupational and Environmental Health
  Fukuoka, Japan Recruiting
• Division of Cardiovascular Medicine, Kobe University Hospital
  Kobe, Japan Recruiting
• Department of Cardiovascular Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine
  Kyoto, Japan Recruiting
• Department of Cardiology, Nagoya University Hospital
  Nagoya, Japan Recruiting
• Department of Cardiovascular Medicine, Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University
  Okayama, Japan Recruiting

Study contacts

• Study coordinator: Kazuya Hosokawa, MD, PhD
• Email: hosokawa.kazuya.712@m.kyushu-u.ac.jp
• Phone: +81-92-642-5360

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How to take part

1. Review the eligibility criteria above with your treating physician.
2. Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
3. Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.