Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass

NCT07132788 | Active Not Recruiting DMC

• 2 other identifiers: 856202R01HL173533
• Study Type: observational
• Target: 150
• Locations: 1 country
• Sites: 1

Brief 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, 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

Keywords

pulmonary hypertension; body composition

Outcome Measures

Primary Outcomes (8)

• Intake of food and nutrients

  Adequacy of micronutrient and protein intake are derived from the two 24 hour diets recalls.

  One time at Baseline

• Epicardial adipose tissue (EAT) volume

  Derived from chest CT

  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.

  One time at Baseline

• Right ventricular (RV) function

  Determined from 2D echocardiography

  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%.

  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.

  One time at Baseline

• Accelerometry measures

  Step counts and activity intensity (sedentary time, time spent doing light, moderate, and vigorous activity) will be derived.

  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.

  One time at Baseline

Secondary Outcomes (1)

• Chest Computed Tomographic (CT) scan

  One time at Baseline

Interventions

blood draw DIAGNOSTIC_TEST

Research blood draw

Short Performance Physical Battery DIAGNOSTIC_TEST

test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance

Grip strength test DIAGNOSTIC_TEST

Measures grip strength

Six minute walk test DIAGNOSTIC_TEST

Measures distance walked in 6 minutes

Chest CT scan RADIATION

X-ray and computer technology takes detailed pictures of the organs and structures inside your chest

Dual-Energy X-ray Absorptiometry Scan RADIATION

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

Also known as: DXA Scan, DEXA Scan

Activity monitoring BEHAVIORAL

A device worn on your non-dominant wrist which measures motion/ movement/ activity

24 hour diet recall BEHAVIORAL

A research dietitian will contact you and ask you to provide details of what you ate over the previous 24 hours

Echocardiogram DIAGNOSTIC_TEST

Ultrasound technology to create moving images of your heart, valves, and chambers

Also known as: Echo, Cardiac Ultrasound

Quality of Life Questionnaire (emPHasis 10) BEHAVIORAL

The emPHasis-10 is a pulmonary hypertension-specific questionnaire which is scored from 0-50 (with higher scores indicating worse quality of life.)

Also known as: emPHasis 10

International Physical Activity Questionnaire Short Form (IPAQ-SF) BEHAVIORAL

The IPAQ-SF is a small set of questions to obtain comparable estimates of physical activity.

Also known as: IPAQ-SF

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study population will include Pulmonary Hypertension patients from the University of Pennsylvania and Rhode Island Hospital.

You may qualify if:

• 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

You may not qualify if:

• 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

Sponsors & Collaborators

• University of Pennsylvania: lead
• National Heart, Lung, and Blood Institute (NHLBI): collaborator

Study Sites (1)

University of Pennsylvania Hospital

Philadelphia, Pennsylvania, 19104, United States

Location

Biospecimen

Retention: SAMPLES WITHOUT DNA

Blood samples

MeSH Terms

Conditions

Hypertension, Pulmonary

Interventions

Blood Specimen Collection; Walk Test; Absorptiometry, Photon; Echocardiography; Caves

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Intervention Hierarchy (Ancestors)

Specimen Handling; Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Punctures; Surgical Procedures, Operative; Investigative Techniques; Exercise Test; Heart Function Tests; Diagnostic Techniques, Cardiovascular; Radiography; Diagnostic Imaging; Densitometry; Photometry; Chemistry Techniques, Analytical; Cardiac Imaging Techniques; Ultrasonography; Geological Phenomena; Physical Phenomena; Environment; Ecological and Environmental Phenomena; Biological Phenomena; Environment and Public Health

Study Officials

• Nadine Al-Naamani, MD

  University of Pennsylvania

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 1, 2025
• First Posted: August 20, 2025
• Study Start: May 1, 2025
• Primary Completion (Estimated): September 30, 2029
• Study Completion (Estimated): September 30, 2029
• Last Updated: August 20, 2025

Record last verified: 2025-08

Locations

US (1)