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Mechanisms that typically result in increased cardiac output, such as inotropic support, increased heart rate, and decreased afterload, have a blunted effect in Fontan circulation. The "thoracic pump" is a contributor to venous return that has been largely unexplored in patients with Fontan physiology. Inspiratory muscle training can improve the performance of competitive athletes across a range of sports and can improve quality of life and functional capacity in heart failure patients, presumably by reducing inspiratory muscle fatigue and possibly by improving peripheral blood flow during exercise. One could surmise that the effects of these changes would be particularly important in the Fontan population. The investigators propose to study the effects of inspiratory muscle training on exercise and pulmonary function parameters in a cohort of adult Fontan patients.
The investigators hypothesize that a 12-week program of inspiratory muscle training with an inspiratory impedance threshold device will improve inspiratory muscle strength and endurance, and that this will translate into improved exercise performance in patients with Fontan physiology.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER | Device | Subjects will be provided with noseclips and a threshold impedance device set to 40% of their measured maximal inspiratory pressure and will be trained on using the device. Specifically, subjects will be instructed to breathe through the inspiratory muscle training device while wearing noseclips at a rate of 12 to 16 breaths per minute for 30 minutes a day, 5 days a week for 12 consecutive weeks. Each subject will be provided a customized schedule for increasing resistance by 2 cm H2O every 2 weeks to a maximum resistance of 41 cm H2O. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Peak VO2 Between Baseline and Post-inspiratory Muscle Training Measurements. | Baseline and after 12 weeks of training |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Innocor Measurement (Inert Gas Rebreathing Method) of Cardiac Output at Peak Exercise | 12 weeks | |
| Change in SF-36 Health Survey Score (Physical Component Summary) | The 36-Item Short Form (SF-36) Health Survey is a disease-generic survey of an individual's perceived health status and health-related quality of life. There are eight scaled scores, which are the weighted sums of the questions in their section. Each scale is transformed into a 0-100 scale on the assumption that each question carries equal weight. The lower the score the more disability. The higher the score the less disability. These eight scales are then used to calculate (using a proprietary formula) two summary scores: the physical component score (PCS), representing the four physical health scales (physical functioning, role physical, bodily pain, general health), and the mental component score (MCS), representing the four mental health scales (mental health, role emotional, vitality, social functioning). Component scores are norm-based with a mean of 50 (standardized to the US population) and a standard deviation of 10. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Maximum Voluntary Ventilation | 12 weeks | |
| Change in Maximal Inspiratory Pressure | Baseline and after 12 weeks of training | |
| Change in Oxygen Pulse at Peak Exercise |
Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Fred Wu, MD | Boston Children's Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital, Boston | Boston | Massachusetts | 02115 | United States |
One subject who was enrolled and consented dropped out during the initial assessment due to inability to tolerate the metabolic cart during baseline exercise testing.
Consecutive adult patients (≥18years old) with Fontan physiology were recruited through the outpatient cardiology clinic at Boston Children's Hospital between April 2014 and December 2014.
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| ID | Title | Description |
|---|---|---|
| FG000 | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER: Subjects will be provided with noseclips and a threshold impedance device set to 40% of their measured maximal inspiratory pressure and will be trained on using the device. Specifically, subjects will be instructed to breathe through the inspiratory muscle training device while wearing noseclips at a rate of 12 to 16 breaths per minute for 30 minutes a day, 5 days a week for 12 consecutive weeks. Each subject will be provided a customized schedule for increasing resistance by 2 cm H2O every 2 weeks to a maximum resistance of 41 cm H2O. |
| Title | Milestones | Reasons Not Completed | ||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
|
Only subjects who were subject to the intervention (inspiratory muscle training) are included
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| ID | Title | Description |
|---|---|---|
| BG000 | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER: Subjects will be provided with noseclips and a threshold impedance device set to 40% of their measured maximal inspiratory pressure and will be trained on using the device. Specifically, subjects will be instructed to breathe through the inspiratory muscle training device while wearing noseclips at a rate of 12 to 16 breaths per minute for 30 minutes a day, 5 days a week for 12 consecutive weeks. Each subject will be provided a customized schedule for increasing resistance by 2 cm H2O every 2 weeks to a maximum resistance of 41 cm H2O. |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Continuous | Mean |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Change in Peak VO2 Between Baseline and Post-inspiratory Muscle Training Measurements. | Posted | Mean | Standard Deviation | ml/kg/min | Baseline and after 12 weeks of training |
|
Through completion of follow-up testing (completion of study), typically 12 weeks but no more than 15 weeks.
Patients were contacted regularly throughout the training period by phone or email to evaluate for potential adverse events.
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER | THRESHOLD(TM) INSPIRATORY MUSCLE TRAINER: Subjects will be provided with noseclips and a threshold impedance device set to 40% of their measured maximal inspiratory pressure and will be trained on using the device. Specifically, subjects will be instructed to breathe through the inspiratory muscle training device while wearing noseclips at a rate of 12 to 16 breaths per minute for 30 minutes a day, 5 days a week for 12 consecutive weeks. Each subject will be provided a customized schedule for increasing resistance by 2 cm H2O every 2 weeks to a maximum resistance of 41 cm H2O. |
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This pilot study was based on a limited number of patients. In addition, there was incomplete compliance with the prescribed inspiratory muscle training regimen. Finally, our subjects were relatively healthy and mostly had normal MIP at baseline.
| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Dr. Fred Wu | Boston Children's Hospital | 6173556508 | fred.wu@cardio.chboston.org |
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| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D000080039 | Univentricular Heart |
| ID | Term |
|---|---|
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
| D000013 | Congenital Abnormalities |
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|
| 12 weeks |
| 12 weeks |
| Change in Peak Work Rate During Exercise | Baseline and after 12 weeks of training |
| Change in VE/VCO2 Slope (Ratio) During Exercise | Baseline and after 12 weeks of training |
| years |
|
| Sex: Female, Male | Count of Participants | Participants |
|
| Age at Fontan completion | Mean | Standard Deviation | years |
|
| Fontan type | Count of Participants | Participants |
|
| Maximum inspiratory pressure | Mean | Standard Deviation | cmH2O |
|
| Peak work rate | Mean | Standard Deviation | Watts |
|
| Peak relative VO2 | Mean | Standard Deviation | ml/kg/min |
|
| Peak relative VO2 | Mean | Standard Deviation | % predicted |
|
| VE/VCO2 slope (ratio) | Mean | Standard Deviation | ratio |
|
| Peak VE | Mean | Standard Deviation | L/min |
|
| Peak heart rate | Mean | Standard Deviation | beats per minute |
|
| Peak tidal volume | Mean | Standard Deviation | L |
|
| Units | Counts |
|---|
| Participants |
|
|
|
| Secondary | Change in Innocor Measurement (Inert Gas Rebreathing Method) of Cardiac Output at Peak Exercise | One individual failed to sustain maximal exercise long enough to complete measurement of cardiac output on her post-intervention study. That individual was therefore left out of the data analysis for cardiac output at peak exercise ONLY. | Posted | Mean | Standard Deviation | L/min | 12 weeks |
|
|
|
|
| Secondary | Change in SF-36 Health Survey Score (Physical Component Summary) | The 36-Item Short Form (SF-36) Health Survey is a disease-generic survey of an individual's perceived health status and health-related quality of life. There are eight scaled scores, which are the weighted sums of the questions in their section. Each scale is transformed into a 0-100 scale on the assumption that each question carries equal weight. The lower the score the more disability. The higher the score the less disability. These eight scales are then used to calculate (using a proprietary formula) two summary scores: the physical component score (PCS), representing the four physical health scales (physical functioning, role physical, bodily pain, general health), and the mental component score (MCS), representing the four mental health scales (mental health, role emotional, vitality, social functioning). Component scores are norm-based with a mean of 50 (standardized to the US population) and a standard deviation of 10. | Posted | Mean | Standard Deviation | units on a scale | 12 weeks |
|
|
|
|
| Other Pre-specified | Change in Maximum Voluntary Ventilation | Posted | Mean | Standard Deviation | L/min | 12 weeks |
|
|
|
|
| Other Pre-specified | Change in Maximal Inspiratory Pressure | Posted | Mean | Standard Deviation | cmH2O | Baseline and after 12 weeks of training |
|
|
|
|
| Other Pre-specified | Change in Oxygen Pulse at Peak Exercise | Posted | Mean | Standard Deviation | mL/beat | 12 weeks |
|
|
|
|
| Other Pre-specified | Change in Peak Work Rate During Exercise | Posted | Mean | Standard Deviation | Watts | Baseline and after 12 weeks of training |
|
|
|
|
| Other Pre-specified | Change in VE/VCO2 Slope (Ratio) During Exercise | Posted | Mean | Standard Deviation | ratio | Baseline and after 12 weeks of training |
|
|
|
|
| 0 |
| 11 |
| 0 |
| 11 |
| 0 |
| 11 |
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| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |