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Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass-Substudy

Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass-Penn Pilot Low Load Resistance Exercise Training Substudy

Who can join

Ages 18 and older · All sexes

Inclusion Criteria: Participating the main MUSCLE UP Study:

* Signed informed consent prior to initiation of any study mandated procedure.
* Male and female patient's ≥ 18 years of age.
* 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
* More than moderate aortic or mitral valve disease
* LVEF \< 40% within 1 year of screening
* Pregnancy

About the study

After completion of the Baseline study visit for the main study (MUSCLE UP-PH), participants at the University of Pennsylvania location will be invited to participate in an optional substudy. The substudy will implement a low-load resistance exercise training for the lower extremities, focusing primarily on the gastrocnemius muscles. The goal of this intervention is to understand how low-load resistance training will impact blood flow and oxygen tissue extraction on the lower legs.

What is being tested

• Measurement of oxyhemoglobin and deoxyhemoglobin concentration pre and post- exercise at baseline and Week 12. Measurement of time to recovery of oxyhemglobin post-exercise at baseline and at Week 12. (device)
• Blood draw (diagnostic test)

Sponsor: University of Pennsylvania · Participants: 20 · Started: Nov 2, 2026

Contact the study team

• Rebecca Gallagher Research Project Manager, MSEd · Phone: 215-662-4088
• Nicole Denney, MS · Phone: 215-615-4156

Official record on ClinicalTrials.gov — NCT07781709

Locations in the U.S.

Conditions

• Pulmonary Arterial Hypertension

From ClinicalTrials.gov, data retrieved Nov 15, 2026. Each study sets its own eligibility; the study team decides who can join.