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Purpose

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

Condition

Eligibility

Eligible Ages
Over 18 Years
Eligible Sex
All
Accepts Healthy Volunteers
No

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

Study Design

Phase
N/A
Study Type
Interventional
Allocation
N/A
Intervention Model
Single Group Assignment
Primary Purpose
Treatment
Masking
None (Open Label)

Arm Groups

ArmDescriptionAssigned Intervention
Other
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.
  • 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
    Other names:
    • DXA Scan
    • DEXA Scan
  • 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
    Other names:
    • Echo
    • Cardiac Ultrasound
  • 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.)
    Other names:
    • emPHasis 10
  • 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.
    Other names:
    • IPAQ-SF
  • 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.

Recruiting Locations

University of Pennsylvania Hospital
Philadelphia, Pennsylvania 19104
Contact:
Nicole Denney, MS
Nicole.denney@Pennmedicine.upenn.edu

More Details

Status
Recruiting
Sponsor
University of Pennsylvania

Study Contact

Rebecca Gallagher
215-294-9755
Rebecca.lang@Pennmedicine.upenn.edu

Detailed Description

In a subset of patients, after obtaining informed consent, patients will be scheduled for an in-person visit with a physical therapist. The subject will have their vital signs checked, blood drawn and then will have the PortaMon device applied to their biceps and their calf muscles. The PortaMon is a wireless NIRS device designed to measure muscle oxygenation non-invasively. It has one channel to measure absolute oxygenated hemoglobin and three channels to measure relative concentrations of oxy-, deoxy-, total hemoglobin concentration changes, and tissue saturation index. Following application and activation of the biosensor and the NIRS device, the physical therapist will demonstrate the exercises to the participant and will observe them performing the exercises. After the conclusion of the session (~1.5 hours), the subject's vital signs will be reassessed and blood drawn. The second study visit will be at Week 12.

Notice

Study information shown on this site is derived from ClinicalTrials.gov (a public registry operated by the National Institutes of Health). The listing of studies provided is not certain to be all studies for which you might be eligible. Furthermore, study eligibility requirements can be difficult to understand and may change over time, so it is wise to speak with your medical care provider and individual research study teams when making decisions related to participation.