NCT07132788 · University of Pennsylvania
Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass
(MUSCLE UP-PH)
What this study is about
Patients with pulmonary arterial hypertension (PAH) are at increased risk of muscle loss and decreased physical activity. This study will aim to (1) understand the way in which muscle loss occurs in PAH, particularly the role of fat surrounding the heart, and (2) look at the impact muscle loss has on quality of life, daily physical activity, and hospitalizations in patients with PAH.
View original scientific description
Patients with pulmonary arterial hypertension (PAH) are at increased risk of muscle loss and decreased physical activity. This study will aim to (1) understand the way in which muscle loss occurs in PAH, particularly the role of fat surrounding the heart, and (2) look at the impact muscle loss has on quality of life, daily physical activity, and hospitalizations in patients with PAH. The findings from this study could help identify potentially treatable factors that may improve the overall quality of life and physical functioning of patients with PAH.
Interventions
DIAGNOSTIC_TEST
blood draw
Research blood draw
DIAGNOSTIC_TEST
Short Performance Physical Battery
test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance
DIAGNOSTIC_TEST
Grip strength test
Measures grip strength
DIAGNOSTIC_TEST
Six minute walk test
Measures distance walked in 6 minutes
RADIATION
Chest CT scan
X-ray and computer technology takes detailed pictures of the organs and structures inside your chest
RADIATION
Dual-Energy X-ray Absorptiometry Scan
Uses X-ray technology to measure the mineral density of your bones, and provides a ratio of fat tissue to lean mass (muscle) and bone
BEHAVIORAL
Activity monitoring
A device worn on your non-dominant wrist which measures motion/ movement/ activity
BEHAVIORAL
24 hour diet recall
A research dietitian will contact you and ask you to provide details of what you ate over the previous 24 hours
DIAGNOSTIC_TEST
Echocardiogram
Ultrasound technology to create moving images of your heart, valves, and chambers
BEHAVIORAL
Quality of Life Questionnaire (emPHasis 10)
The emPHasis-10 is a pulmonary hypertension-specific questionnaire which is scored from 0-50 (with higher scores indicating worse quality of life.)
BEHAVIORAL
International Physical Activity Questionnaire Short Form (IPAQ-SF)
The IPAQ-SF is a small set of questions to obtain comparable estimates of physical activity.
BEHAVIORAL
Low resistance exercise intervention
Participants will be asked to perform a series of exercises recommend by the National Institute on Aging Go4Life campaign, namely hand grip, overhead arm raise, arm curl, wall push-ups, and chair dips.
Primary outcome measures
Intake of food and nutrients
Time frame: One time at Baseline
Adequacy of micronutrient and protein intake are derived from the two 24 hour diets recalls.
Epicardial adipose tissue (EAT) volume
Time frame: One time at Baseline
Derived from chest CT
Metabolomics
Time frame: One time at Baseline
Circulating proteins will be measured in plasma samples using the Olink Proteomics (Waltham, MA). In this assay, Olink uses a proprietary proximity extension assay technology to combine a detection step involving oligonucleotide-labeled antibodies with a proximity-dependent DNA polymerization step and a real-time quantitative PCR amplification step to measure relative levels of multiple biomarkers simultaneously. Two different panels will be used: Olink Explore 384 Cardiometabolic and Olink Explore 384 Inflammation.
Right ventricular (RV) function
Time frame: One time at Baseline
Determined from 2D echocardiography
Adiponectin
Time frame: One time at Baseline
Measured using a solid phase sandwich ELISA (R\&D Systems, Inc., Minneapolis, MN) with a minimum detectable dose of 0.246 ng/mL and intra-assay variations (CV) 2.5%-4.7%.
Low lean mass
Time frame: One time at Baseline
Using the data from the dual-energy X-ray absorptiometry, we will generate fat mass index-adjusted appendicular lean mass index (ALMIFMI) Z-Scores derived from age, sex, and race/ethnicity specific models.16 Low lean mass will be defined as ALMIFMI ZScore less than or equal to -1.
Accelerometry measures
Time frame: One time at Baseline
Step counts and activity intensity (sedentary time, time spent doing light, moderate, and vigorous activity) will be derived.
Health-Related Quality of Life (HRQOL)
Time frame: One time at Baseline
The emPHasis-10 is a questionnaire used to assess the impact of pulmonary hypertension (PH) on a person's life. It consists of 10 items, each scored on a scale of 0 to 5, resulting in a total score ranging from 0 to 50. Higher scores indicate a greater symptom burden and poorer quality of life.
Measurement of adiponectin levels pre- and post-exercise at baseline and at Week 12
Time frame: 12 Weeks
We will measure adiponectin levels using a solid phase sandwich ELISA (R\&D Systems, Inc., Minneapolis, MN) with a minimum detectable dose of 0.246 ng/mL and intra-assay variations (CV) 2.5%-4.7%.
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.
Time frame: 12 weeks
We will measure oxy-hemoglobin and deoxyhemoglobin concentration using NIRS applied to the biceps and calf pre- and post- exercise at baseline and Week 12. Moreover, we will measure time to recovery of oxy-hemglobin post-exercise at baseline and at Week 12.
Who can participate
This study lists these criteria on ClinicalTrials.gov. A study coordinator reviews eligibility during screening — this page does not determine whether you qualify.
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
Where
- Philadelphia, Pennsylvania
Collaborators
National Heart, Lung, and Blood Institute (NHLBI)
Related conditions & keywords
Frequently asked questions
What is a clinical trial?
A clinical trial is a research study that tests new medical treatments, drugs, devices, or procedures to determine their safety and effectiveness. Trials are carefully designed and monitored to protect participants while advancing medical knowledge.
Is it safe to participate?
Clinical trials follow strict safety guidelines and ethical standards. Trials must be reviewed and approved, and participants are closely monitored by medical professionals throughout the study. You can withdraw at any time if you choose.
Will I be compensated?
Many clinical trials offer compensation for your time, travel expenses, and inconvenience. The specific compensation varies by study and will be discussed during the screening process. All study-related medical care is typically provided at no cost to participants.
Will I receive a placebo instead of treatment?
When effective treatment exists, participants typically receive either the standard treatment plus the study intervention, or the standard treatment plus placebo. You would not be denied effective care. Placebos are primarily used when no proven treatment is available, or in addition to standard care. Your trial consent form will clearly explain what treatments you may receive.
Can I leave a trial if I change my mind?
Absolutely. Participation in clinical trials is completely voluntary. You have the right to withdraw from the study at any time, for any reason, without penalty or loss of benefits to which you are otherwise entitled.
How long does a clinical trial last?
Trial duration varies widely depending on the study design and purpose. Some trials last just a few weeks, while others may continue for months or years. The study coordinator will provide specific timeline information during your screening call.
Data: ClinicalTrials.gov · synced Jul 6, 2026 · Source of record for eligibility and locations