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

Tarek Alsaied15 enrolled

Overview

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.

The aortic VTI secondary outcome was inadvertently left out of the initial registration and is now reported

Study Type

INTERVENTIONAL

Allocation

Purpose

PREVENTION

Masking

NONE

Enrollment

Conditions

Single-ventricle

Interventions

Hands-free oral-positive pressure device (oPEP)DEVICE

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.

Eligibility

Sex: ALLMin age: 8 Years

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

Change in Pulmonary Pulsatility Index

This will be measured by echocardiogram using Doppler evaluation of the pulmonary artery The Pulmonary Artery Pulsatility Index (PAPI) is calculated using Doppler-derived pulmonary artery velocities: PAPI= (Systolic Velocity - Diastolic Velocity) /Mean Velocity

Time frame: The change is between enrollment echocardiogram and echocardiogram after 4 week use of the device

Secondary Outcomes

Aortic Valve VTI

This was measured by echocardiography Aortic Valve Velocity Time Integral (VTI) Definition: Aortic VTI is a key echocardiographic measurement reflecting stroke distance and cardiac output. Measurement: Obtained using pulsed-wave Doppler at the left ventricular outflow tract (LVOT) just below the aortic valve. The VTI is measured by tracing the Doppler envelope of the LVOT flow during systole. Clinical Importance: Stroke Volume Calculation: Stroke Volume = LVOT VTI × LVOT Area

Time frame: Baseline, 4 weeks