Pulmonary Hypertension
Conditions
Keywords
pulmonary hypertension, body composition
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
Interventions
DIAGNOSTIC_TESTblood draw
Research blood draw
DIAGNOSTIC_TESTShort Performance Physical Battery
test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance
DIAGNOSTIC_TESTGrip strength test
Measures grip strength
DIAGNOSTIC_TESTSix minute walk test
Measures distance walked in 6 minutes
RADIATIONChest CT scan
X-ray and computer technology takes detailed pictures of the organs and structures inside your chest
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
BEHAVIORALActivity monitoring
A device worn on your non-dominant wrist which measures motion/ movement/ activity
BEHAVIORAL24 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_TESTEchocardiogram
Ultrasound technology to create moving images of your heart, valves, and chambers
BEHAVIORALQuality 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.)
BEHAVIORALInternational Physical Activity Questionnaire Short Form (IPAQ-SF)
The IPAQ-SF is a small set of questions to obtain comparable estimates of physical activity.
Sponsors
National Heart, Lung, and Blood Institute (NHLBI)
University of Pennsylvania
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Health-Related Quality of Life (HRQOL) | One time at Baseline | 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. |
| Intake of food and nutrients | One time at Baseline | Adequacy of micronutrient and protein intake are derived from the two 24 hour diets recalls. |
| Epicardial adipose tissue (EAT) volume | One time at Baseline | Derived from chest CT |
| Metabolomics | One time at Baseline | 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. |
| Right ventricular (RV) function | One time at Baseline | Determined from 2D echocardiography |
| Adiponectin | One time at Baseline | 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%. |
| Low lean mass | One time at Baseline | 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. |
| Accelerometry measures | One time at Baseline | Step counts and activity intensity (sedentary time, time spent doing light, moderate, and vigorous activity) will be derived. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Chest Computed Tomographic (CT) scan | One time at Baseline | The primary variables of interest will be total thoracic muscle volume measured on non-contrast chest CT scans |
Countries
United States
Outcome results
None listed