Pulmonary HypertensioniTONENCT05442671
iTONE Trial (exercIse Training in pulmONary hypertEnsion) Exercise Program for Pediatric PH
A study testing Exercise intervention in people with Pulmonary Hypertension, run by Children's Hospital of Philadelphia at 1 site in United States.
Trial summary
Children with pulmonary hypertension (PH) engage in less physical activity than their peers. This is a concern since adult data support exercise as a non-pharmacologic treatment for PH. Despite adult data, therapeutic exercise has not been widely adopted in pediatric PH. Read more about this study
- Who is it for?
- Ages 8 to 18 years
- Conditions the study is for
- Pulmonary Hypertension
- What is being tested?
- Exercise intervention
- Type of treatment
- Lifestyle or therapy programme
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 25 participants
- Where
- 1 siteUnited States
- Study dates
- 3 Apr 2024to 31 Dec 2026
- Phase
- Interventional
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 8 to 18 years
- All sexes
- No healthy volunteers
- Child, Adult
Who may be able to join
- Age 8-18 years
- PH World Health Organization (WHO) diagnostic groups 1, 2, 3, or 4 (pulmonary arterial hypertension, PH due to left heart disease, PH due to lung disease, chronic thromboembolic PH)
- WHO functional class I or II
- Ambulatory status
- Mean pulmonary to systemic arterial pressure ratio <0.75 if the patient has not undergone a pulmonary to systemic artery shunt ("Potts" shunt) OR the placement of a Potts shunt ≥ 6 months prior to study enrollment
- Stable PH medication regimen for 3 months prior to the intervention
- Home Wifi connection
- Mobile device in family capable of receiving text messages
Who cannot join
- 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
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, 19104
Catherine Avitabile, MD, avitabilec@chop.edu
About this study
Official title: Home Exercise Training in Pediatric Pulmonary Hypertension
Children with pulmonary hypertension (PH) engage in less physical activity than their peers. This is a concern since adult data support exercise as a non-pharmacologic treatment for PH. Despite adult data, therapeutic exercise has not been widely adopted in pediatric PH. Investigators have previously demonstrated that children with PH have less skeletal muscle mass in association with worse exercise performance. Interventions to increase physical activity and skeletal muscle mass may improve exercise performance and quality of life in children with PH. This study will use wearable activity monitoring devices to promote physical activity in a 16-week pilot intervention in children and teenager with PH.
Read the detailed 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 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 periodic text messages to measure activity, obtain daily downloads, and engage patients to promote adherence. Assessment of muscle mass and strength 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.
- Conditions
- Pulmonary Hypertension
- Being tested
- Exercise intervention
- Keywords
- Actigraphy
- Medical subject headings
- Hypertension, Pulmonary
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Exercise interventionBehavioural
16-week home exercise intervention, enriched by mobile health technology
| Study group | Role | What they receive |
|---|---|---|
| Home exercise intervention | Other | * Personalized 16 week home exercise program - aerobic exercise for 20 minutes per day/4 days per week and light resistance exercise using resistance bands 3 days per week.
* Aerobic sessions will include walking, biking, or light jogging, depending on access to facilities/equipment and weather.
* Smartwatch for the length of the intervention and a heart rate monitor during exercise sessions.
* Max heart rate prescribed will be 80% of that on recent cardiopulmonary exercise test (at most 150 beats/min).
* Heart rate monitor will sync with the smartwatch.
* Activity and heart rate data will be transmitted to the study team via a data hub connected to the participant's home internet modem several times per week.
* Periodic text messaging to remind participants to wear the watch, sync the data, or adhere to heart rate goals, to ask about symptoms, or to support activity progress.
* Multiple ways to contact the study team with questions or concerns. Receives: Behavioral: Exercise intervention |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in moderate to vigorous physical activity (MVPA)
Change in MVPA (min/day) will be measured by smartwatch accelerometer from baseline over the course of the intervention
Measured: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Secondary outcomes (3)
Change in leg lean mass Z-score (LLMZ)
LLMZ will be measured by densitometry. LLMZ is a percentile score with scores -2 to 2 considered typical
Measured: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Change in muscle strength
Upper extremity strength will be measured by handgrip dynamometer. Lower extremity strength will be measured by Biodex across the knee and ankle
Measured: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Change in quality of life score
Score measured by completion of PedsQL questionnaire. Scores range from 0-100 with higher scores indicating better quality of life
Measured: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted5 Jul 2022
- Study start3 Apr 2024
- Primary completion31 Dec 2026 (estimated)
- Study completion31 Dec 2026 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary HypertensionStudies of the same condition, open and under way.
- More trials run by Children's Hospital of PhiladelphiaThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 25 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Children's Hospital of Philadelphia
- Sponsor type
- University, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI)
- Responsible party
- Sponsor
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT05442671
- Sponsor's protocol ID
- 20-018168
- Other IDs
- 1K23HL150337-01A1
- Acronym
- iTONE
- Status last verified
- Apr 2026
- First submitted
- 24 Jun 2022
- Last updated
- 22 Apr 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 22 Apr 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Home Exercise Training in Pediatric Pulmonary Hypertension
- ClinicalTrials.gov ID
- NCT05442671
- Acronym / sponsor ID
- iTONE / 20-018168
- Conditions
- Pulmonary Hypertension
- Keywords
- Actigraphy
- Study type
- Interventional
- Recruitment status
- Recruiting
- Enrolment
- 25 participants (estimated)
- Lead sponsor
- Children's Hospital of PhiladelphiaUniversity, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI)
- Study start
- 3 Apr 2024 (actual)
- Primary completion
- 31 Dec 2026 (estimated)
- Study completion
- 31 Dec 2026 (estimated)
- First posted
- 5 Jul 2022 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Children with pulmonary hypertension (PH) engage in less physical activity than their peers. This is a concern since adult data support exercise as a non-pharmacologic treatment for PH. Despite adult data, therapeutic exercise has not been widely adopted in pediatric PH. Investigators have previously demonstrated that children with PH have less skeletal muscle mass in association with worse exercise performance. Interventions to increase physical activity and skeletal muscle mass may improve exercise performance and quality of life in children with PH. This study will use wearable activity monitoring devices to promote physical activity in a 16-week pilot intervention in children and teenager with PH.
Detailed 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 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 periodic text messages to measure activity, obtain daily downloads, and engage patients to promote adherence. Assessment of muscle mass and strength 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.
Study design
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Home exercise intervention | Other | Behavioral: Exercise intervention* Personalized 16 week home exercise program - aerobic exercise for 20 minutes per day/4 days per week and light resistance exercise using resistance bands 3 days per week. * Aerobic sessions will include walking, biking, or light jogging, depending on access to facilities/equipment and weather. * Smartwatch for the length of the intervention and a heart rate monitor during exercise sessions. * Max heart rate prescribed will be 80% of that on recent cardiopulmonary exercise test (at most 150 beats/min). * Heart rate monitor will sync with the smartwatch. * Activity and heart rate data will be transmitted to the study team via a data hub connected to the participant's home internet modem several times per week. * Periodic text messaging to remind participants to wear the watch, sync the data, or adhere to heart rate goals, to ask about symptoms, or to support activity progress. * Multiple ways to contact the study team with questions or concerns. |
Who may qualify
- Ages
- 8 to 18 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult
Key inclusion criteria (8 listed)
- Age 8-18 years
- PH World Health Organization (WHO) diagnostic groups 1, 2, 3, or 4 (pulmonary arterial hypertension, PH due to left heart disease, PH due to lung disease, chronic thromboembolic PH)
- WHO functional class I or II
- Ambulatory status
- Mean pulmonary to systemic arterial pressure ratio <0.75 if the patient has not undergone a pulmonary to systemic artery shunt ("Potts" shunt) OR the placement of a Potts shunt ≥ 6 months prior to study enrollment
Key exclusion criteria (6 listed)
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Exercise intervention16-week home exercise intervention, enriched by mobile health technology | Behavioural | Home exercise intervention |
Primary outcome
Change in moderate to vigorous physical activity (MVPA)
Time frame: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Change in MVPA (min/day) will be measured by smartwatch accelerometer from baseline over the course of the intervention
3 secondary outcomes
Change in leg lean mass Z-score (LLMZ)
Time frame: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
Change in muscle strength
Time frame: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)
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)
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 22 Apr 2026.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Exercise intervention in people with Pulmonary Hypertension, run by Children's Hospital of Philadelphia at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 8 inclusion and 6 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 22 Apr 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Catherine Avitabile, MD (215-590-4040, avitabilec@chop.edu), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in moderate to vigorous physical activity (MVPA) (time frame: Pre-exercise intervention and Post intervention (within 2 weeks of completion of the 16-week exercise intervention)). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 22 Apr 2026; AzTrials last refreshed its copy on 29 Oct 2026.