Feasibility and Efficacy of Portable Non-invasive Negative Pressure Ventilation in Fontan Patients

Exclusion Criteria

• •unable to complete MRI (pacemaker/defibrillator/retained wires, pregnancy, claustrophobia);
• •evidence of Fontan failure (requiring ongoing medical / device therapy) protein-losing enteropathy, intracardiac thrombus, anatomical obstruction to the Fontan circuit;
• •patent Fontan fenestration;
• •oxygen saturation \< 90%;
• •ongoing arrhythmia;
• •ejection fraction \< 50% on echocardiography or CMR;
• •moderate or severe valve insufficiency on echocardiography or CMR;
• •Obesity (BMI \>35);
• •severe obstructive sleep apnea (AHI\>20);
• •chronic obstructive lung disease (FEV1/FVC\<60%);
• •severe chest wall deformities (scoliosis, kyphosis, kyphoscoliosis);
• •acute or chronic kidney disease (eGFR\<60)
• •unable to provide informed consent.