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Physical Activity in Pediatric Pulmonary Hypertension


Study First Submitted Date 2022-06-24
Study First Posted Date 2022-07-05
Last Update Posted Date 2023-03-27
Start Month Year February 1, 2024
Primary Completion Month Year March 1, 2026
Verification Month Year March 2023
Verification Date 2023-03-31
Last Update Posted Date 2023-03-27

Detailed Descriptions

Sequence: 20620581
Description Pediatric pulmonary hypertension (PH) is a rare disease, affecting 2-16 per million children. Without treatment, pulmonary vascular disease leads to right ventricular cardiac failure and death. Survival has improved with pharmacologic advances, however, 5-year survival from diagnosis is still only about 75%. Even with increased survival, pediatric PH is a severe, chronic illness with physical and psychosocial burdens affecting the quality of life of children and families. Exercise intolerance is nearly universal and adversely affects quality of life. While exercise physiology is complex, skeletal muscle deficits and dysfunction are important factors associated with exercise intolerance in adults with PH. The contraction of the skeletal muscle pump helps increase pulmonary blood flow at the initiation of upright exercise in the normal circulation. Low skeletal muscle mass and muscle dysfunction may impair this mechanism in PH. Investigators previously demonstrated low skeletal muscle mass in association with worse exercise performance in children with PH and identified this as an intervention target. As children with PH engage in less moderate and vigorous physical activity compared to healthy peers, we hypothesize that increasing physical activity could increase skeletal muscle mass and lead to improved exercise performance. Recent trials have demonstrated improved exercise performance and functional class in adults with PH undertaking prescribed exercise training. Pediatric data are limited to a single, small study in which exercise performance improved after a 16-week home exercise program. That intervention did not include skeletal muscle assessment, wearable activity monitors, or specified behavioral theory, as does the current proposal. The characteristics of the optimal PH exercise program are not clear as prior studies often failed to provide sufficient detail to enable reproducibility. Most programs have been hospital-based, some with additional at-home components. Duration has varied from 4-15 weeks. Programs have included aerobic, resistance, and respiratory exercise. Patient adherence has been inconsistently assessed. While wearable activity monitors have been used to assess activities of daily living of PH patients, they have not been employed in PH exercise programs. In youth, practical concerns of developmental differences, access to facilities or equipment, and parental time commitment must be considered, and home programs may be critical to avoiding missed school and work. Newer technologic advances enable remote monitoring of physical activity in the home setting. Wearable accelerometer sensors with wireless transmission capabilities allow us to measure relevant endpoints from a patient's daily life. In this study, participants will undergo baseline assessment of muscle mass and strength, 6-minute walk test distance, and quality of life prior to a 16-week home exercise program, enriched by mobile health technology. Investigators will use a wrist-worn accelerometer paired with a custom mobile device application on a smart device to measure activity, obtain daily downloads, and engage patients to promote adherence. Assessment of muscle mass and strength, 6-minute walk test distance, and quality of life will be repeated after the intervention. This real-time monitoring of adherence and feedback to the patient represent fundamental changes in PH treatment paradigms that can significantly enhance efficacy.


Sequence: 51912791
Name Pulmonary Hypertension
Downcase Name pulmonary hypertension

Id Information

Sequence: 39956016 Sequence: 39956017
Id Source org_study_id Id Source secondary_id
Id Value 20-018168 Id Value 1K23HL150337-01A1
Id Type U.S. NIH Grant/Contract
Id Link

Design Groups

Sequence: 55319960
Group Type Other
Title Home exercise intervention
Description Participants will be instructed in performing aerobic exercise for 20 minutes per day, 5 days per week, and light resistance exercises 2 days per week. Aerobic sessions will start with stretching followed by walking, biking, or light jogging, depending on access to facilities/equipment and weather. Participants will increase their heart rate on the smartwatch to a goal of 80% that achieved on their 6-minute walk test (at most 150 beats/min). Participants will perform light resistance exercises using resistance bands. Participants will be advised to avoid heavy exercise. Participants will wear the smartwatch for the length of the intervention. This will synch with a mobile device application to support at-home symptom assessment, recording of resistance exercise, and protocol adherence. There will be multiple ways (videochat, telephone ChatBot) by which participants can contact the study team with questions or concerns.


Sequence: 52227198
Intervention Type Behavioral
Name Exercise intervention
Description 16-week home exercise intervention, enriched by mobile health technology

Design Outcomes

Sequence: 176490491 Sequence: 176490492 Sequence: 176490493 Sequence: 176490494 Sequence: 176490495
Outcome Type primary Outcome Type secondary Outcome Type secondary Outcome Type secondary Outcome Type secondary
Measure Change in moderate to vigorous physical activity (MVPA) Measure Change in leg lean mass Z-score (LLMZ) Measure Change in muscle strength Measure Change in 6-minute walk test distance Measure Change in quality of life score
Time Frame Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention) Time Frame Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention) Time Frame Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention) Time Frame Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention) Time Frame Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Description Change in MVPA (min/day) will be measured by accelerometer from baseline over the course of the intervention Description LLMZ will be measured by densitometry. LLMZ is a percentile score with scores -2 to 2 considered typical Description Upper extremity strength will be measured by handgrip dynamometer. Lower extremity strength will be measured by Biodex across the knee and ankle Description 6-minute walk distance (in meters) will be measured per standard American Thoracic Society guidelines Description Score measured by completion of PedsQL questionnaire. Scores range from 0-100 with higher scores indicating better quality of life

Browse Conditions

Sequence: 192448716 Sequence: 192448717 Sequence: 192448718 Sequence: 192448719 Sequence: 192448720 Sequence: 192448721
Mesh Term Hypertension, Pulmonary Mesh Term Hypertension Mesh Term Vascular Diseases Mesh Term Cardiovascular Diseases Mesh Term Lung Diseases Mesh Term Respiratory Tract Diseases
Downcase Mesh Term hypertension, pulmonary Downcase Mesh Term hypertension Downcase Mesh Term vascular diseases Downcase Mesh Term cardiovascular diseases Downcase Mesh Term lung diseases Downcase Mesh Term respiratory tract diseases
Mesh Type mesh-list Mesh Type mesh-list Mesh Type mesh-ancestor Mesh Type mesh-ancestor Mesh Type mesh-ancestor Mesh Type mesh-ancestor


Sequence: 48075856 Sequence: 48075857
Agency Class OTHER Agency Class NIH
Lead Or Collaborator lead Lead Or Collaborator collaborator
Name Children's Hospital of Philadelphia Name National Heart, Lung, and Blood Institute (NHLBI)

Design Group Interventions

Sequence: 67818909
Design Group Id 55319960
Intervention Id 52227198


Sequence: 30611819
Gender All
Minimum Age 8 Years
Maximum Age 18 Years
Healthy Volunteers No
Criteria Inclusion Criteria: Age 8-18 years PH World Health Organization (WHO) diagnostic groups 1, 2, or 3 (pulmonary arterial hypertension, PH due to left heart disease, PH due to lung disease) WHO functional class I or II Ambulatory status Mean pulmonary to systemic arterial pressure ratio <0.75 on most recent cardiac catheterization Oxygen saturation >85% on 6-minute walk test Stable PH medication regimen for 3 months prior to the intervention, Exclusion Criteria: WHO functional class III or IV Single ventricle physiology Moderate to severe renal disease (>stage 3) Severe hepatic impairment [aspartate aminotransferase (AST)/alanine transaminase (ALT) > 2x upper limit of normal] Current pregnancy Significant developmental delay/inability to comply with verbal instructions to complete the study procedures
Adult True
Child True
Older Adult False

Calculated Values

Sequence: 253943313
Registered In Calendar Year 2022
Were Results Reported False
Has Single Facility False
Minimum Age Num 8
Maximum Age Num 18
Minimum Age Unit Years
Maximum Age Unit Years
Number Of Primary Outcomes To Measure 1
Number Of Secondary Outcomes To Measure 4


Sequence: 30359052
Allocation N/A
Intervention Model Single Group Assignment
Observational Model
Primary Purpose Treatment
Time Perspective
Masking None (Open Label)

Responsible Parties

Sequence: 28730683
Responsible Party Type Sponsor