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| ID | Type | Description | Link |
|---|---|---|---|
| R01HL173533 | U.S. NIH Grant/Contract | View source |
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| Name | Class |
|---|---|
| National Heart, Lung, and Blood Institute (NHLBI) | NIH |
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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:
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Low resistance exercise intervention | Other | 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. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| blood draw | Diagnostic Test | Research blood draw |
| |
| Measure | Description | Time Frame |
|---|---|---|
| Intake of food and nutrients | Adequacy of micronutrient and protein intake are derived from the two 24 hour diets recalls. | One time at Baseline |
| Epicardial adipose tissue (EAT) volume | Derived from chest CT | One time at Baseline |
| Metabolomics | 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. | One time at Baseline |
| Right ventricular (RV) function | Determined from 2D echocardiography | One time at Baseline |
| Adiponectin | 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%. | One time at Baseline |
| Low lean mass | 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. | One time at Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Chest Computed Tomographic (CT) scan | The primary variables of interest will be total thoracic muscle volume measured on non-contrast chest CT scans | One time at Baseline |
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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:
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:
Exclusion Criteria:
Prior to enrollment, evidence of moderately severe obstructive ventilator defect with:
Prior to enrollment, evidence of severe restrictive defect with
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
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Rebecca Gallagher | Contact | 215-294-9755 | Rebecca.lang@Pennmedicine.upenn.edu | |
| Nicole Denney, MS | Contact | 215-615-4156 | nicole.denney@pennmedicine.upenn.edu |
| Name | Affiliation | Role |
|---|---|---|
| Nadine Al-Naamani, MD | University of Pennsylvania | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University of Pennsylvania Hospital | Recruiting | Philadelphia | Pennsylvania | 19104 | United States |
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| Short Performance Physical Battery |
| Diagnostic Test |
test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance |
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| Grip strength test | Diagnostic Test | Measures grip strength |
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| Six minute walk test | Diagnostic Test | Measures distance walked in 6 minutes |
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| Chest CT scan | Radiation | X-ray and computer technology takes detailed pictures of the organs and structures inside your chest |
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| Dual-Energy X-ray Absorptiometry Scan | Radiation | 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 |
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| Activity monitoring | Behavioral | A device worn on your non-dominant wrist which measures motion/ movement/ activity |
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| 24 hour diet recall | Behavioral | A research dietitian will contact you and ask you to provide details of what you ate over the previous 24 hours |
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| Echocardiogram | Diagnostic Test | Ultrasound technology to create moving images of your heart, valves, and chambers |
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| 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.) |
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| 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. |
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| Low resistance exercise intervention | Behavioral | 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. |
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| Accelerometry measures | Step counts and activity intensity (sedentary time, time spent doing light, moderate, and vigorous activity) will be derived. | One time at Baseline |
| Health-Related Quality of Life (HRQOL) | 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. | One time at Baseline |
| Measurement of adiponectin levels pre- and post-exercise at baseline and at Week 12 | We will measure adiponectin levels 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%. | 12 Weeks |
| Measurement of oxyhemoglobin and deoxyhemoglobin concentration pre and post- exercise at baseline and Week 12. Measurement of time to recovery of oxyhemglobin post-exercise at baseline and at Week 12. | We will measure oxy-hemoglobin and deoxyhemoglobin concentration using NIRS applied to the biceps and calf pre- and post- exercise at baseline and Week 12. Moreover, we will measure time to recovery of oxy-hemglobin post-exercise at baseline and at Week 12. | 12 weeks |
| ID | Term |
|---|---|
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| D000070857 | Walk Test |
| D015502 | Absorptiometry, Photon |
| D004452 | Echocardiography |
| D059552 | Caves |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |
| D005080 | Exercise Test |
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D011859 | Radiography |
| D003952 | Diagnostic Imaging |
| D003720 | Densitometry |
| D010783 | Photometry |
| D002623 | Chemistry Techniques, Analytical |
| D057791 | Cardiac Imaging Techniques |
| D014463 | Ultrasonography |
| D055593 | Geological Phenomena |
| D055585 | Physical Phenomena |
| D004777 | Environment |
| D055669 | Ecological and Environmental Phenomena |
| D001686 | Biological Phenomena |
| D004778 | Environment and Public Health |
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