Pulmonary HypertensionMUSCLE UP-PHNCT07132788
Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass
An observational study of Pulmonary Hypertension, run by University of Pennsylvania at 2 sites in United States.
Trial summary
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… Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Pulmonary Hypertension
- What is being tested?
- blood draw, Short Performance Physical Battery and Grip strength test
- Type of treatment
- Surgery or procedure, Lifestyle or therapy programme
- How many people
- 150 participants
- Where
- 2 sitesUnited States
- Study dates
- 29 Jul 2024to 1 Jul 2029
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: The study population will include Pulmonary Hypertension patients from the University of Pennsylvania and Rhode Island Hospital.
Who may be able to join
- 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:
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- 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
Who cannot join
- 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
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- 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
Trial locations and contacts
2 sites in 1 country. Contact the study team or a site near you to ask about taking part.
Rebecca Gallagher Research Project Manager, MSEd contact
United States2 sites, 2 recruiting
- Recruiting
University of Pennsylvania Hospital
Philadelphia, Pennsylvania, 19104
Nicole Denney, MS, Nicole.denney@Pennmedicine.upenn.edu
Nadine Al-Naamani, MD
- Recruiting
Rhode Island Hospital
Providence, Rhode Island, 02903
Allyson Sherman-Roe Senior Clincial Research Program Coordinator, 401-444-2733 ext. 401-444-2733, ashermanroe1@brownhealth.org
Ashley Dennett Senior Clinical Research Assistant, 401-606-4745, adennett@brownhealth.org
About this study
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.
Subjects will be asked to attend a baseline visit where the following will be performed:
- Measure your vital signs
- Undergo a research blood draw, less than 4 tablespoons
- Provide a urine pregnancy test (if applicable)
- Review demographics, personal history, and medical history
- Review current PAH medications
- Complete questionnaires on how your PAH affects you
- Complete a test of physical performance
- Complete a grip strength test
- Undergo an echocardiogram (Echo)
- Complete a six-minute walk test
- Undergo a Chest CT Scan
- Undergo a scan of your body composition (DXA scan)
- Obtain a weight and body composition measurement on the InBody Scale Subjects will also complete activity moniotring, two 24-hour diet recalls, and participate in remote follow-up visits every 6 months
- Conditions
- Pulmonary Hypertension
- Being tested
- blood draw, Short Performance Physical Battery, Grip strength test, Six minute walk test, Chest CT scan
- Keywords
- pulmonary hypertension, body composition
- Medical subject headings
- Hypertension, Pulmonary
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
blood drawDiagnostic test
Research blood draw
Short Performance Physical BatteryDiagnostic test
test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance
Grip strength testDiagnostic test
Measures grip strength
Six minute walk testDiagnostic test
Measures distance walked in 6 minutes
Chest CT scanRadiation
X-ray and computer technology takes detailed pictures of the organs and structures inside your chest
Dual-Energy X-ray Absorptiometry ScanRadiation
Also called DXA Scan, DEXA 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
Activity monitoringBehavioural
A device worn on your non-dominant wrist which measures motion/ movement/ activity
24 hour diet recallBehavioural
A research dietitian will contact you and ask you to provide details of what you ate over the previous 24 hours
EchocardiogramDiagnostic test
Also called Echo, Cardiac Ultrasound
Ultrasound technology to create moving images of your heart, valves, and chambers
Quality of Life Questionnaire (emPHasis 10)Behavioural
Also called 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.)
International Physical Activity Questionnaire Short Form (IPAQ-SF)Behavioural
Also called IPAQ-SF
The IPAQ-SF is a small set of questions to obtain comparable estimates of physical activity.
| Study group | Role | What they receive |
|---|---|---|
| PAH cohort at Penn and Brown | Participants will be asked to:
Complete a research blood draw Review of demographics, personal history, and medical history Review of current PAH medications Physical performance test (SPPB) Grip strength test Echocardiogram (Echo) Six-minute walk test Chest CT Scan Scan of your body composition (DXA scan - Penn only) and In-Body Scale (Penn and Brown) Wear an activity monitoring watch Complete 24-hour diet recall Receives: Diagnostic Test: blood draw; Diagnostic Test: Short Performance Physical Battery; Diagnostic Test: Grip strength test; Diagnostic Test: Six minute walk test; Radiation: Chest CT scan; Radiation: Dual-Energy X-ray Absorptiometry Scan; Behavioral: Activity monitoring; Behavioral: 24 hour diet recall; Diagnostic Test: Echocardiogram; Behavioral: Quality of Life Questionnaire (emPHasis 10); Behavioral: International Physical Activity Questionnaire Short Form (IPAQ-SF) |
What is being measured
The primary outcome is the main question the study is designed to answer.
Intake of food and nutrients
Adequacy of micronutrient and protein intake are derived from the two 24 hour diets recalls.
Measured: One time at Baseline
Chest Computed Tomographic (CT) scan
The primary variables of interest will be total thoracic muscle volume measured on non-contrast chest CT scans
Measured: One time at Baseline
Epicardial adipose tissue (EAT) volume
Derived from chest CT
Measured: One time at Baseline
Metabolomics
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.
Measured: One time at Baseline
Right ventricular (RV) function
Determined from 2D echocardiography
Measured: One time at Baseline
Adiponectin
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%.
Measured: One time at Baseline
Low lean mass
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.
Measured: One time at Baseline
Accelerometry measures
Step counts and activity intensity (sedentary time, time spent doing light, moderate, and vigorous activity) will be derived.
Measured: One time at Baseline
Health-Related Quality of Life (HRQOL)
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.
Measured: Baseline and every 6 months up to 5 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 Aug 2025
- Study start29 Jul 2024
- Primary completion1 Jul 2029 (estimated)
- Study completion1 Jul 2029 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary HypertensionStudies of the same condition, open and under way.
- More trials run by University of PennsylvaniaThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 150 participants (estimated)
Sponsor and investigators
- Lead sponsor
- University of Pennsylvania
- Sponsor type
- University, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI), Rhode Island Hospital
- Responsible party
- Sponsor
- Investigators
- Nadine Al-Naamani, MD, Principal investigator, University of Pennsylvania
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07132788
- Sponsor's protocol ID
- 856202
- Other IDs
- R01HL173533
- Acronym
- MUSCLE UP-PH
- Status last verified
- Oct 2026
- First submitted
- 1 Jul 2025
- Last updated
- 15 Oct 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 15 Oct 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass
- ClinicalTrials.gov ID
- NCT07132788
- Acronym / sponsor ID
- MUSCLE UP-PH / 856202
- Conditions
- Pulmonary Hypertension
- Keywords
- pulmonary hypertension, body composition
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 150 participants (estimated)
- Lead sponsor
- University of PennsylvaniaUniversity, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI), Rhode Island Hospital
- Study start
- 29 Jul 2024 (actual)
- Primary completion
- 1 Jul 2029 (estimated)
- Study completion
- 1 Jul 2029 (estimated)
- First posted
- 20 Aug 2025 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Subjects will be asked to attend a baseline visit where the following will be performed:
- Measure your vital signs
- Undergo a research blood draw, less than 4 tablespoons
- Provide a urine pregnancy test (if applicable)
- Review demographics, personal history, and medical history
- Review current PAH medications
- Complete questionnaires on how your PAH affects you
- Complete a test of physical performance
- Complete a grip strength test
- Undergo an echocardiogram (Echo)
- Complete a six-minute walk test
- Undergo a Chest CT Scan
- Undergo a scan of your body composition (DXA scan)
- Obtain a weight and body composition measurement on the InBody Scale Subjects will also complete activity moniotring, two 24-hour diet recalls, and participate in remote follow-up visits every 6 months
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| PAH cohort at Penn and Brown | – | Diagnostic Test: blood draw, Diagnostic Test: Short Performance Physical Battery, Diagnostic Test: Grip strength test, Diagnostic Test: Six minute walk test, Radiation: Chest CT scan, Radiation: Dual-Energy X-ray Absorptiometry Scan, Behavioral: Activity monitoring, Behavioral: 24 hour diet recall, Diagnostic Test: Echocardiogram, Behavioral: Quality of Life Questionnaire (emPHasis 10), Behavioral: International Physical Activity Questionnaire Short Form (IPAQ-SF)Participants will be asked to: Complete a research blood draw Review of demographics, personal history, and medical history Review of current PAH medications Physical performance test (SPPB) Grip strength test Echocardiogram (Echo) Six-minute walk test Chest CT Scan Scan of your body composition (DXA scan - Penn only) and In-Body Scale (Penn and Brown) Wear an activity monitoring watch Complete 24-hour diet recall |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- The study population will include Pulmonary Hypertension patients from the University of Pennsylvania and Rhode Island Hospital.
Key inclusion criteria (15 listed)
- 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]
- 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:
Key exclusion criteria (10 listed)
- Prior to enrollment, evidence of moderately severe obstructive ventilator defect with:
- Prior to enrollment, evidence of severe restrictive defect with
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| blood drawResearch blood draw | Diagnostic test | PAH cohort at Penn and Brown |
| Short Performance Physical Batterytest of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance | Diagnostic test | PAH cohort at Penn and Brown |
| Grip strength testMeasures grip strength | Diagnostic test | PAH cohort at Penn and Brown |
| Six minute walk testMeasures distance walked in 6 minutes | Diagnostic test | PAH cohort at Penn and Brown |
| Chest CT scanX-ray and computer technology takes detailed pictures of the organs and structures inside your chest | Radiation | PAH cohort at Penn and Brown |
| Dual-Energy X-ray Absorptiometry ScanAlso: DXA Scan, DEXA ScanUses X-ray technology to measure the mineral density of your bones, and provides a ratio of fat tissue to lean mass (muscle) and bone | Radiation | PAH cohort at Penn and Brown |
| Activity monitoringA device worn on your non-dominant wrist which measures motion/ movement/ activity | Behavioural | PAH cohort at Penn and Brown |
| 24 hour diet recallA research dietitian will contact you and ask you to provide details of what you ate over the previous 24 hours | Behavioural | PAH cohort at Penn and Brown |
| EchocardiogramAlso: Echo, Cardiac UltrasoundUltrasound technology to create moving images of your heart, valves, and chambers | Diagnostic test | PAH cohort at Penn and Brown |
| Quality of Life Questionnaire (emPHasis 10)Also: emPHasis 10The emPHasis-10 is a pulmonary hypertension-specific questionnaire which is scored from 0-50 (with higher scores indicating worse quality of life.) | Behavioural | PAH cohort at Penn and Brown |
| International Physical Activity Questionnaire Short Form (IPAQ-SF)Also: IPAQ-SFThe IPAQ-SF is a small set of questions to obtain comparable estimates of physical activity. | Behavioural | PAH cohort at Penn and Brown |
Primary outcome
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.
Chest Computed Tomographic (CT) scan
Time frame: One time at Baseline
The primary variables of interest will be total thoracic muscle volume measured on non-contrast chest CT scans
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: Baseline and every 6 months up to 5 years
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.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 15 Oct 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Rebecca Gallagher Research Project Manager, MSEd contact
Nadine Al-Naamani, MD principal investigator
University of Pennsylvania
2 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Hypertension, run by University of Pennsylvania at 2 sites in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 15 inclusion and 10 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 15 Oct 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 2 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Rebecca Gallagher Research Project Manager, MSEd (215-294-9755, Rebecca.lang@Pennmedicine.upenn.edu), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Intake of food and nutrients (time frame: One time at Baseline). The study lists 9 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 15 Oct 2026.