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NCT Number: NCT07357974

Impact of Sotatercept on Pulmonary Artery and Right Ventricle Remodeling Imaging Assessed With 68Ga-FAPI PET/CT in Patients With PAH

Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterized by structural changes in the pulmonary arteries, leading to increased pulmonary vascular resistance and elevated pulmonary arterial pressure and, if untreated, right heart failure. Diagnosis requires a comprehensive evaluation, including right heart catheterization performed in specialized centers.

Despite advances in the understanding and management of the disease, PAH remains a severe condition. Current approved therapies primarily target three key pathways involved in endothelial dysfunction: the endothelin, nitric oxide, and prostacyclin pathways. Pulmonary arterial remodeling is characterized by alterations in endothelial cells, smooth muscle cells, and fibroblasts, with fibroblast activation and macrophage involvement contributing to disease progression.

Two positron emission tomography/computed tomography (PET/CT) imaging approaches are currently under investigation in PAH. [⁶⁸Ga]Ga-FAPI PET/CT targets activated fibroblasts and enables noninvasive assessment of fibroblast activity and tissue remodeling. [⁶⁸Ga]Ga-MAA lung perfusion PET/CT is an emerging imaging technique that provides higher spatial resolution and sensitivity than conventional lung perfusion imaging and allows evaluation of regional pulmonary perfusion.

Sotatercept is a novel fusion protein that modulates signaling within the transforming growth factor-beta (TGF-β) superfamily by binding select ligands involved in vascular remodeling. Its mechanism of action is distinct from that of currently approved PAH therapies. Sotatercept has been evaluated in clinical development programs, including the PULSAR and STELLAR studies. Reported adverse events include epistaxis, dizziness, increased hemoglobin levels, and changes in blood pressure.

This study is designed with the following objectives:

Primary objective: To assess pulmonary vascular remodeling in patients with PAH using [⁶⁸Ga]Ga-FAPI PET/CT imaging.

Secondary objectives: To evaluate [⁶⁸Ga]Ga-FAPI uptake and regional lung perfusion using [⁶⁸Ga]Ga-MAA lung perfusion PET/CT imaging at predefined study time points.

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Key information

Conditions

Pulmonary Arterial Hypertension (PAH) Hypertension, Pulmonary Lung Diseases Pulmonary Arterial Hypertension Respiratory Tract Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 1 / Phase 2

Primary location

Chu Brest

Brest, Finistere, 29200, France

Location contact

cecile Tromeur

CONTACT

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Age ≥ 18 years
  • Documented diagnostic right heart catheterization (RHC) within 12 months of screening documenting a minimum PVR of ≥ 4 Wood units and pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤ 15 mmHg, with the diagnosis of WHO PAH Group 1 in any of the following subtypes:
  • Idiopathic PAH
  • Heritable PAH
  • Drug/toxine-induced PAH
  • PAH associated with connective tissue disease
  • PAH associated with simple, congenital systemic-to- pulmonary shunts at least 1year following repair
  • Patients under bi or tri-background-therapy
  • Symptomatic PAH classified WHO FC II or III
  • Patients will be started on Sotatercept
  • Ability to adhere to study visit schedule and understand and comply with all the protocol requirement.
  • Ability to understand and provide written informed consent

Exclusion criteria

  • Diagnosis of PH WHO Groups 2, 3, 4, or 5
  • Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis associated PAH, pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis.
  • Hemoglobin at screening above gender-specific ULN, per local laboratory test
  • Pregnant or breastfeeding women
  • Any of the following clinical laboratory values at the Screening visit:
  • eGFR < 30 mL/min/1.73 m2 (as defined by MDRD equation)
  • Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), or total bilirubin levels > 3 × ULN
  • Platelet count < 50,000/mm3 (< 50.0 × 109/L)
  • Known allergic reaction to sotatercept (ACE-011), its excipients, or luspatercept

Treatment and study plan

[68Ga]Ga-FAPI PET/CT

Diagnostic Test

[68Ga]Ga-FAPI PET/CT

[68Ga]Ga-MAA PET/CT

Diagnostic Test

[68Ga]Ga-MAA PET/CT

Primary outcomes

  1. Vascular remodeling as assessed by [68Ga] Ga-FAPI uptake of pulmonary arteries on PET/CT imaging.

    Time frame: at baseline

    A positive uptake will be defined as a ratio of the Standardized uptake value (SUV) max of the pulmonary arteries and the SUVmean of the vascular background activity measured at the center of the left ventricle greater than 1.5.

Secondary outcomes

  1. Change from baseline in [68Ga] Ga-FAPI uptake on pulmonary arteries as assessed by SUVmax at 24 weeks.

    Time frame: At baseline and at 24 weeks.

    [68Ga] Ga-FAPI uptake on pulmonary arteries. This will be assessed using a visual scale and the Standardized uptake value (SUVmax).

  2. Visual assessment of pulmonary artery [68Ga]Ga-FAPI uptake

    Time frame: At baseline and at 24 weeks.

    Change from baseline in regional lung perfusion as assessed by the Pulmonary vascular obstruction index (PVOI) (in %) on [68Ga]Ga-MAA lung perfusion PET/CT imaging at 24 weeks.

  3. [68Ga] Ga-FAPI uptake on the right ventricule (RV) assessed by SUVmax at 24 weeks.

    Time frame: At week 24

  4. RV ventricule dysfunction based on Tricuspid annular plane systolic excursion (TAPSE)<17mm at 24 weeks.

    Time frame: At 24 weeks.

  5. Change from baseline in [68Ga] Ga-FAPI uptake on the RV as assessed by SUVmax at 24 weeks.

    Time frame: At baseline and at 24 weeks.

  6. Mean pulmonary arterial pressure (mPAP) (in mmHg) at baseline.

    Time frame: At baseline.

    mPAP will be measured by right Heart catheterism (RHC).

  7. Cardiac output (CO) (l/mn) at baseline.

    Time frame: At baseline.

    CO will be measured by RHC.

  8. Right atrial pressure (RAP) (mmHg) at baseline.

    Time frame: at baseline

    RAP will be measured by RHC.

  9. Right ventricular pressure (RVP) (mmHg) at baseline.

    Time frame: At baseline.

    CO will be measured by RHC.

  10. Pulmonary vascular resistance (PVR) (Wood unit) at baseline.

    Time frame: At baseline.

    CO will be measured by RHC.

  11. Mean pulmonary arterial pressure (mPAP) (mmHg) at 24 weeks.

    Time frame: At 24 weeks.

    mPAP will be measured by right Heart catheterism (RHC).

  12. Cardiac output (CO) (l/mn) at 24 weeks.

    Time frame: At 24 weeks.

    CO will be measured by RHC.

  13. Right atrial pressure (RAP) (mmHg) at 24 weeks.

    Time frame: At 24 weeks.

    RAP will be measured by RHC.

  14. Right ventricular pressure (RVP) (mmHg) at 24 weeks.

    Time frame: at baseline

    CO will be measured by RHC.

  15. 6 minutes walking test result (6MWT) (meters) at baseline.

    Time frame: At baseline.

  16. Vascular remodeling on [68Ga]Ga-FAPI PET/CT is correlated with Nt-proBNP measure in PAH patients

    Time frame: at baseline

    [68Ga] Ga-FAPI uptake on pulmonary arteries (SUVmax) will be correlated with Nt-proBNP measure in PAH patients

  17. 6 minutes walking test result (6MWT) (meters) at 24 weeks.

    Time frame: At 24 weeks.

  18. NYHA functional class at baseline.

    Time frame: At baseline.

    The New York heart association (NYHA) scale will be used to classify the severity of the heart failure impact. Classes are defined as I, II, III, IV, with IV indicating severe limitations

  19. NYHA functional class at 24 weeks.

    Time frame: At 24 weeks.

    The New York heart association (NYHA) scale will be used to classify the severity of the heart failure impact extent of heart failure. Classes are defined as I, II, III, IV, with IV indicating severe limitations.

  20. N-terminal pro B natriuretic peptide (NT-pro-BNP) (pg/ml) at baseline

    Time frame: At baseline.

    biological mark indicating heart failure and impact on PH prognosis

  21. N-terminal pro B natriuretic peptide (NT-pro-BNP) (pg/ml) at 24 weeks.

    Time frame: At 24 weeks.

    biological mark indicating heart failure and impact on PH prognosis

  22. Quality of life (QOL) assessed by emPHasis-10 scale at baseline.

    Time frame: At baseline.

    The emPHasis-10 ranges from 0 to 50, with a higher score indicating a poorer health status and QOL.

  23. QOL assessed by emPHasis-10 scale at 24 weeks.

    Time frame: At 24 weeks.

    The emPHasis-10 ranges from 0 to 50, with a higher score indicating a poorer health status and QOL.

Study contacts

Contact information is provided by the study sponsor or research team.

Cécile Tromeur

CONTACT

[email protected]

+33230337602

Sponsors and collaborators

Lead sponsor

University Hospital, Brest

Other

Registry information

Official study title

Impact of Sotatercept on Pulmonary Artery and Right Ventricle Remodeling Imaging Assessed With 68Ga-FAPI PET/CT in Patients With PAH: a Pilot Study

Acronym: SOFAPI

Important dates

Study start
2026
Primary completion
2028
Study completion
2028
First posted
Jan 22, 2026
Registry last updated
Jun 16, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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