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

NCT03251742 | Completed Phase 1

• 1 other identifier: Fontan0615
• Study Type: interventional
• Target: 20
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Fontan Physiology

Keywords

Fontan operation; Negative pressure ventilation; MRI

Outcome Measures

Primary Outcomes (2)

• 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 Outcomes (1)

• Tolerability and safety of negative pressure ventilation.

  6-8 months

Study Arms (2)

Fontan patient population

ACTIVE COMPARATOR

Device: Hayek RTX ventilator

Healthy volunteers

SHAM COMPARATOR

Device: Hayek RTX ventilator

Interventions

Hayek RTX ventilator DEVICE

Negative pressure ventilation in Fontan patients.

Also known as: Cuirass, Negative pressure ventilation

Fontan patient population; Healthy volunteers

Eligibility Criteria

• Age: 13 Years - 55 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• Fontan patients age \> 13 years.

You may not qualify if:

• 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.

Sponsors & Collaborators

• University Health Network, Toronto: lead
• Hayek Medical: collaborator

Study Sites (1)

Toronto General Hospital/University of Toronto.

Toronto, Ontario, M5G2N2, Canada

Location

Related Publications (1)

• Shaji S, Zafar MA, Christopher A, Saraf A, Hoskoppal A, Lanford L, Kreutzer J, Olivieri L, Alsaied T. Augmented Biphasic Breathing Using Sniff and an Oral Positive Expiratory Pressure Device (Sniff-PEP) in Fontan Patients. Pediatr Cardiol. 2025 Aug;46(6):1725-1732. doi: 10.1007/s00246-024-03598-3. Epub 2024 Jul 19.

  PMID: 39028352 DERIVED

Study Officials

• Rachel Wald, MD, MS

  Toronto General Hospital/University Health Network

  PRINCIPAL INVESTIGATOR

• Pradeepkumar Charla, MD, MS

  Toronto General Hospital/University Health Network

  STUDY DIRECTOR

Study Design

• Study Type: interventional
• Phase: phase 1
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: OTHER
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Clinical Fellow

Model Details: This is a cross-sectional study designed to evaluate the immediate impact of negative pressure ventilation (NPV) in an ambulatory Fontan population using cardiovascular magnetic resonance imaging (CMR) for assessment of cardiovascular flows.

Study Record Dates

• First Submitted: April 4, 2017
• First Posted: August 16, 2017
• Study Start: November 17, 2017
• Primary Completion: May 1, 2018
• Study Completion: May 1, 2018
• Last Updated: June 1, 2018

Record last verified: 2018-05

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1)