Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass

NCT ID: NCT07132788

Last Updated: 2025-08-20

Basic Information

• Recruitment Status: ACTIVE_NOT_RECRUITING
• Total Enrollment: 150 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2025-05-01
• Study Completion Date: 2029-09-30

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

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Interventions

blood draw

Research blood draw

Intervention Type: DIAGNOSTIC_TEST

Short Performance Physical Battery

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

Intervention Type: DIAGNOSTIC_TEST

Grip strength test

Measures grip strength

Intervention Type: DIAGNOSTIC_TEST

Six minute walk test

Measures distance walked in 6 minutes

Intervention Type: DIAGNOSTIC_TEST

Chest CT scan

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

Intervention Type: 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

Intervention Type: RADIATION

Activity monitoring

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

Intervention Type: 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

Intervention Type: BEHAVIORAL

Echocardiogram

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

Intervention Type: DIAGNOSTIC_TEST

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.)

Intervention Type: 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.

Intervention Type: BEHAVIORAL

Other Intervention Names

DXA Scan; DEXA Scan; Echo; Cardiac Ultrasound; emPHasis 10; IPAQ-SF

Eligibility Criteria

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

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

National Heart, Lung, and Blood Institute (NHLBI)

NIH

Sponsor Role: collaborator

University of Pennsylvania

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Nadine Al-Naamani, MD

Role: PRINCIPAL_INVESTIGATOR

University of Pennsylvania

Locations

University of Pennsylvania Hospital

Philadelphia, Pennsylvania, United States

Countries

United States

Other Identifiers

R01HL173533

Identifier Type: NIH

Identifier Source: secondary_id

View Link

856202

Identifier Type: -

Identifier Source: org_study_id