Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass

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

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.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI)

  collaborator NIH

• University of Pennsylvania

  lead OTHER

Principal Investigators

• Nadine Al-Naamani, MD · University of Pennsylvania

Study Design

Allocation

NA

Purpose

TREATMENT

Masking

NONE

Model

SINGLE_GROUP

Eligibility

Min Age

18 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2025-05-01

Primary Completion

2029-09-30

Completion

2029-09-30

Countries

• United States

Study Locations