Simple Non-invasive Breathing Device to Improve Pulmonary Flow Pusatility in Single Ventricle Post Fontan

Study Overview
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Brief Summary
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This is a pilot study using a novel, minimal risk, portable, hands-free oral-positive pressure device (oPEP) in patients with Fontan palliation that will examine whether using this device in both the acute and chronic phase will alter Fontan hemodynamics and create pulsatility in the Fontan circuit and thereby increasing cardiac output. This device is easy to use and poses no significant risk to human subjects. The investigators will measure this through echocardiographic measures including pulsatility in different aspects of the Fontan circuit including IVC, hepatic veins, the Fontan conduit, and pulmonary arteries and aortic blood flow measurements. After demonstration of how to use the device appropriately, the investigators will have patients use the device after their clinical echocardiogram for their clinic appointment. The investigators will ask them to use the device at home 3-4 times a day for 10-15 mins and have them return in approximately 4 weeks to have another echocardiogram done with the same measurements.

Official Title
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Oral Positive Pressure Device (oPEP) Effect on Flow Pulsatility in the Fontan Circuit

Conditions
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Single-ventricle

Intervention / Treatment
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* Device: Hands-free oral-positive pressure device (oPEP)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with single ventricle with Fontan palliation Over the age of 8 years who would be cooperative with breathing through the oPEP device Exclusion Criteria: Patients with Fontan palliation under the age of 8 years Patients who have interrupted inferior vena cava Patients with abnormal pulmonary artery anatomy

Ages Eligible for Study
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Minimum Age: 8 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Prevention
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Hands-free oral-positive pressure device (oPEP)<br> | Device: Hands-free oral-positive pressure device (oPEP)<br>* This is a pilot study using a novel, minimal risk, portable, hands-free oral-positive pressure device (oPEP) in patients with Fontan palliation that will examine whether using this device in both the acute and chronic phase will alter Fontan hemodynamics and create pulsatility in the Fontan circuit and thereby increasing cardiac output. This device is easy to use and poses no significant risk to human subjects. We will measure this through echocardiographic measures including pulsatility in different aspects of the Fontan circuit including IVC, hepatic veins, the Fontan conduit, and pulmonary arteries and aortic blood flow measurements. After demonstration of how to use the device appropriately, we will have patients use the device after their clinical echocardiogram for their clinic appointment. We will ask them to use the device at home 3-4 times a day for 10-15 mins and have them return in approximately 4 weeks to have another echocardiogram done with the same measurements.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in Pulmonary pulsatility index | This will be measured by echocardiogram | The change is between enrollment echocardiogram and echocardiogram after 4 week use of the device |