Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass

University of Pennsylvania150 enrolled

Overview

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 Type

OBSERVATIONAL

Enrollment

150

Conditions

Pulmonary Hypertension

Interventions

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

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 monitoringBEHAVIORAL

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

24 hour diet recallBEHAVIORAL

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

EchocardiogramDIAGNOSTIC_TEST

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

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.)

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.

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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

Locations (2)

University of Pennsylvania Hospital

Philadelphia, Pennsylvania, United States

RECRUITING

Rhode Island Hospital

Providence, Rhode Island, United States

RECRUITING