Noninvasive Negative Pressure Ventilation to Support Failing Fontan Physiology

Study Overview
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Brief Summary
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The purpose of this study is to assess for acute hemodynamic changes after implementation of noninvasive negative pressure ventilation (as compared with spontaneous respiration) in pediatric patients with Fontan failure using modern negative pressure ventilators

Official Title
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Noninvasive Negative Pressure Ventilation to Support Failing Fontan Physiology

Conditions
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Failing Fontan Physiology, Pediatric Congenital Heart Disease

Intervention / Treatment
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* Device: Hayek RTX ventilator


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 1. Patients with Fontan physiology with clinical evidence of Fontan failure as defined by those with one of the following in addition to Fontan circulation: New York Heart Association Class II, fluid retention, cyanosis, protein losing enteropathy, renal/hepatic dysfunction and/or subjectively qualify for hemodynamic assessment (by primary cardiologist). Exclusion Criteria: Patients which will likely require intubation for catheterization Severely depressed systemic ventricular systolic function Baseline airway obstruction Plastic bronchitis Significant OUTFLOW obstruction

Ages Eligible for Study
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Minimum Age: 10 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: Device Feasibility
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Hayek RTX ventilator<br>Participants will receive noninvasive negative pressure ventilation. | Device: Hayek RTX ventilator<br>* Participants will receive Noninvasive negative pressure from the Hayek RTX ventilator for 30 minutes during diagnostic catheterization procedure.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Hemodynamics as characterized by change in pulmonary artery pressure | Pulmonary artery wedge pressure will be measured in millimeters of mercury (mmHg) at baseline and completion of noninvasive negative pressure ventilation. | Baseline, 45 Minutes | 
| Hemodynamics as characterized by change in pulmonary blood flow | Change in pulmonary blood flow will be measured at baseline and completion of noninvasive negative pressure ventilation. | Baseline, 45 Minutes | 
| Hemodynamics as characterized by change in systemic arterial saturation | Systemic arterial saturation measures the amount of oxygen in the blood stream. Systemic arterial saturation will be registered at baseline and completion of noninvasive negative pressure ventilation. | Baseline, 45 Minutes | 
| Hemodynamics as characterized by change in cardiac output | Cardiac output will be measured in liters per minute at baseline and completion of noninvasive negative pressure ventilation | Baseline, 45 Minutes |