Understanding the Effects of Pulmonary Arterial Hypertension on Lean Muscle Mass

Обзор

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

Подробное описание

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.

Вмешательства

• Диагностический тест blood draw
  Research blood draw
• Диагностический тест Short Performance Physical Battery
  test of physical performance, which involves measuring movement from sitting to standing, walking speed, and balance
• Диагностический тест Grip strength test
  Measures grip strength
• Диагностический тест Six minute walk test
  Measures distance walked in 6 minutes
• Лучевая терапия Chest CT scan
  X-ray and computer technology takes detailed pictures of the organs and structures inside your chest
• Лучевая терапия 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
• Поведенческое Activity monitoring
  A device worn on your non-dominant wrist which measures motion/ movement/ activity
• Поведенческое 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
• Диагностический тест Echocardiogram
  Ultrasound technology to create moving images of your heart, valves, and chambers
• Поведенческое 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.)

Первичные конечные точки

• Intake of food and nutrients [Срок оценки: One time at Baseline]
• Epicardial adipose tissue (EAT) volume [Срок оценки: One time at Baseline]
• Metabolomics [Срок оценки: One time at Baseline]
• Right ventricular (RV) function [Срок оценки: One time at Baseline]
• Adiponectin [Срок оценки: One time at Baseline]
• Low lean mass [Срок оценки: One time at Baseline]
• Accelerometry measures [Срок оценки: One time at Baseline]
• Health-Related Quality of Life (HRQOL) [Срок оценки: One time at Baseline]
• Measurement of adiponectin levels pre- and post-exercise at baseline and at Week 12 [Срок оценки: 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. [Срок оценки: 12 weeks]

Вторичные конечные точки (1)

• Chest Computed Tomographic (CT) scan [Срок оценки: One time at Baseline]

Критерии участия

Критерии включения

• 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

Критерии исключения

• 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

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Распределение

Не применимо

Модель

Одна группа

Маскирование

Открытое

Основная цель

Лечение

Центры проведения

США · 1 центр

• University of Pennsylvania Hospital — Philadelphia

Идентификаторы

NCT: NCT07132788 · 856202 · R01HL173533

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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