Feasibility and Efficacy of Portable Non-invasive Negative Pressure Ventilation in Fontan Patients (FONTAN-CMR)

Feasibility and Efficacy Of Negative Pressure Ventilation in Fontan populatioN: Cardiovascular Flow Assessment by Magnetic Resonance Imaging

The human heart has 4 chambers: 2 collecting chambers (atria) and 2 pumping chambers (ventricles) to allow blood flow within two distinct circuits: "pulmonary" and "systemic". The pulmonary circuit directs the blood to the lungs to receive oxygen and the systemic circuit delivers oxygen-rich blood throughout the body. In children born with a single ventricle, blood from these two circuits mixes within the heart resulting in lower oxygen levels in the blood delivered to the body (cyanosis).

The Fontan procedure is a palliative surgery which bypasses the need for a ventricle to deliver blood to the lungs, as blood from the body flows passively to the lungs by a man-made connection (whereby two large body veins [cavae] are sewn to the pulmonary arteries), thereby preventing mixing of blood through restoration of two distinct circuits without mixing of blood. Although the Fontan operation effectively eliminates cyanosis and enables survival into adulthood, increased systemic venous pressure is an unavoidable systemic complication and low cardiac output (CO) is pervasive finding.

Despite excellent pediatric surgical results, the risk of late complications and death dramatically increases in the decades following Fontan surgery. A chronically low CO state secondary to decreased forward flow of blood to the lungs can result in end-organ dysfunction and shortened life expectancy. Short of heart transplantation, deemed suitable only for a minority of patients, effective therapies for low CO are largely absent. The investigators aim to investigate a novel, non-invasive, ambulatory therapy which can augment CO. Specifically, external suction is applied intermittently to the chest wall, much like a vacuum, to increase CO, called negative pressure ventilation (NPV) using a Cuirass® ventilator (Hayek Medical). Although used in patients with lung disease, the investigators' proposal is to evaluate this novel, portable, ventilation system would be the first study of its kind in adults with congenital heart disease, specifically those with a Fontan palliation.

Study Overview

• Status: Completed
• Conditions: Fontan Physiology
• Intervention / Treatment: Device: Hayek RTX ventilator

• Study Type: Interventional
• Enrollment (Actual): 20
• Phase: Phase 1

Contacts and Locations

Study Locations

• Canada
  • Ontario
    • Toronto, Ontario, Canada, M5G2N2
      • Toronto General Hospital/University of Toronto.

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 13 years to 55 years (ADULT, CHILD)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Fontan patients age > 13 years.

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.

Study Plan

How is the study designed?

Design Details

• Primary Purpose: OTHER
• Allocation: NON_RANDOMIZED
• Interventional Model: PARALLEL
• Masking: NONE

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
ACTIVE_COMPARATOR: Fontan patient population	Device: Hayek RTX ventilator; Negative pressure ventilation in Fontan patients.; Other Names: Cuirass; Negative pressure ventilation
SHAM_COMPARATOR: Healthy volunteers	Device: Hayek RTX ventilator; Negative pressure ventilation in Fontan patients.; Other Names: Cuirass; Negative pressure ventilation

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Efficacy of the NPV on cardiac output	Cardiac output will be measured in L/min/m2 using phase contrast MRI. Flow measurements will be made at baseline and with application of the device.	6-8 months
Efficacy of the NPV on organ perfusion	Multi organ perfusion will be measured in L/min/m2 using phase contrast MRI. Flow measurements will be made at baseline and with application of the device.	6-8 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Tolerability and safety of negative pressure ventilation.	Assessed by participant questionnaire rating tolerance, safety, overall satisfaction with the device.	6-8 months

Collaborators and Investigators

• Sponsor: University Health Network, Toronto
• Collaborators: Hayek Medical
• Investigators: Principal Investigator: Rachel Wald, MD, MS, Toronto General Hospital/University Health Network; Study Director: Pradeepkumar Charla, MD, MS, Toronto General Hospital/University Health Network

Study record dates

Study Major Dates

• Study Start (ACTUAL): November 17, 2017
• Primary Completion (ACTUAL): May 1, 2018
• Study Completion (ACTUAL): May 1, 2018

Study Registration Dates

• First Submitted: April 4, 2017
• First Submitted That Met QC Criteria: August 15, 2017
• First Posted (ACTUAL): August 16, 2017

Study Record Updates

• Last Update Posted (ACTUAL): June 1, 2018
• Last Update Submitted That Met QC Criteria: May 30, 2018
• Last Verified: May 1, 2018

More Information

Terms related to this study

• Keywords: MRI; Fontan operation; Negative pressure ventilation
• Other Study ID Numbers: Fontan0615

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No