How pulmonary arterial hypertension affects lean muscle mass

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 observational interventional protocol enrolls adults with Group 1 PAH and collects body-composition and functional measures at baseline and Week 12. Participants undergo vitals, blood draws, questionnaires, physical performance tests (SPPB, 6-minute walk, grip strength), and chest CT to measure cardiac and regional fat. In a subset, a physical therapist applies a wireless NIRS device (PortaMon) to measure muscle oxygenation before and after standardized exercises to explore muscle physiology. Outcomes include changes in lean muscle mass, daily physical activity, quality-of-life measures, and hospitalizations.

Why it matters

Who can join: the full rules

Inclusion Criteria:

* Signed informed consent prior to initiation of any study mandated procedure.
* Diagnosis of PAH belonging to one of the following subgroups of Group 1 PH according to the updated clinical classification \[Humbert 2022\]

  * Idiopathic (IPAH)
  * Heritable (HPAH)
  * Drugs or toxins induced
  * Associated (APAH) with one of the following:

    * Connective tissue disease;
    * Human immunodeficiency virus (HIV) infection;
    * Congenital heart disease; or
    * Portopulmonary hypertension
* Diagnosis of PAH within 6 months of enrollment or diagnosis of PAH and on stable therapy for 3 months prior to enrollment
* Documented hemodynamic diagnosis of PAH by right heart catheterization (RHC), prior to enrollment showing:

  * mPAP \> 20 mmHg; and
  * PAWP or LVEDP ≤ 15 mmHg
  * PVR \> 2 Wood units

Exclusion Criteria:

* Prior to enrollment, evidence of moderately severe obstructive ventilator defect with:

  * FEV1/FVC ≤ 5th percentile; and
  * FEV1 z-score \< -2.5
* Prior to enrollment, evidence of severe restrictive defect with

  * TLC \< 5th percentile
  * FEV1 z-score \< -4
* Prior to enrollment, hospitalization (within 1 week) for decompensated right heart failure
* More than moderate aortic or mitral valve disease
* LVEF \< 40% within 1 year of screening
* Pregnancy

Where it's running

• University of Pennsylvania Hospital
  Philadelphia, Pennsylvania, United States Recruiting
• Rhode Island Hospital
  Providence, Rhode Island, United States Recruiting

Study contacts

• Principal investigator: Nadine Al-Naamani, MD — University of Pennsylvania
• Study coordinator: Rebecca Gallagher Research Project Manager, MSEd
• Email: Rebecca.lang@Pennmedicine.upenn.edu
• Phone: 215-294-9755

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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.