Impact of Exercise Training on Single Ventricle Function in Paediatric Fontan Patients

A Single-centre, Prospective Case Control Study Evaluating the Impact of a Standardised Aerobic and Strength Exercise Regime on Exercise Capacity, Ventricular Function, and Perceived Quality of Life in Paediatric Fontan Patients

The goal of this observational study is to learn about how a prescribed Exercise Training Program would affect children living with a Fontan circulation.

From birth, the children who are born with single functioning ventricle will undergo 3-staged Fontan surgery to achieve a unique Fontan circulation, supported by one functioning ventricle. With advancement in surgical technique and medical care, there are increasing number of children living with Fontan circulating surviving into adulthood.

A significant number of these individuals develop complications in their adulthood due to problems related to this unique circulation, including reduced exercise tolerance, heart failure, abnormal heart rhythm or premature death. Many of these children have acquired sedentary lifestyle, and may have lower self-perceived health related quality of life.

Exercise training is a well-established therapy used in adult population with heart failure. It has been shown to be safe in children with congenital heart disease and with Fontan circulation, it improves their aerobic exercise capacity which is associated with better outcomes.

The main question[s] it aims to answer are the impact of a Exercise Training program on:

1. Single ventricle function
2. Exercise capacity
3. Self-perceived quality of life
4. Skeletal muscle mass

Participants will be given a 20-weeks exercise program, 3 sessions of 30 minutes workout weekly, to carry out at home. The following tests will be performed prior to starting and upon completion of the exercise program:

• Echocardiogram
• Cardiopulmonary exercise test
• Body composition scan using a bio-impedance analyser
• Quality of life questionnaires

Study Overview

• Status: Not yet recruiting
• Conditions: Univentricular Heart

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

• Study Contact: Name: Li Yen Ng, MB BCh BAO; Phone Number: +353862341835; Email: ngliyen@gmail.com
• Study Contact Backup: Name: Adam James, MD; Phone Number: +353871925275; Email: adamoj@gmail.com

Study Locations

• Ireland
  • Dublin, Ireland, D12 N512
    • Children's Health Ireland
    • Contact: Li Yen Ng, MB BCh BAO; Phone Number: +353862341835; Email: ngliyen@gmail.com
    • Contact: Adam James, MD; Phone Number: +353871925275; Email: adamoj@gmail.com
    • Principal Investigator: Adam James, MD
    • Sub-Investigator: Li Yen Ng, MB BCh BAO

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 4 years to 14 years (Child, Adult)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Children aged 8 to 18 years old, who are living with a Fontan circulation, who have met the eligibility criteria listed above.

Description

Inclusion Criteria:

• Patients with Fontan circulation, age range 8 to 18 years
• Ability to perform a cardiopulmonary exercise stress test (CPET)
• Free from acute or chronic respiratory illness and/or conditions that might affect exercise performance
• Who have had a Fontan operation at least six months prior.

Exclusion Criteria:

• Existing neuro-disability i.e. with paresis post cerebrovascular accident
• Neurodevelopmental issue with inability to comply with commands.
• Documented life-threatening arrhythmia without implantable cardioverter defibrillator
• Moderate or severe atrio-ventricular valve regurgitation
• Moderate or severe ventricular dysfunction by echocardiography exams
• Acute inflammatory cardiac disease
• documented coronary artery stenosis and/or myocardial ischaemia
• Heart failure requiring hospitalisation in the past year
• Evidence of systemic venous pathway obstruction by echocardiography or catheterisation
• Resting oxygen saturation < 90%
• Systemic ventricular outflow tract stenosis with peak resting systolic gradient > 50mmHg

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Single ventricle Function	By implementing a prescribed exercise training regime over a 20 week period, a change will be noted in ventricular function at the subclinical state using Echocardiography parameters.	After a 20 week Exercise training program

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Exercise Capacity	By implementing a prescribed exercise training regime over a 20 week period, an improvement will be noted in functional exercise capacity, which will be objectively assessed using cardiopulmonary exercise test.	After a 20 week Exercise training program
Self-perceived Quality of life - Generic	By implementing a prescribed exercise training regime over a 20 week period, an improvement will be noted in self-reported quality of life, which will be assessed using validated QOL questionnaire - Using Paediatric Quality of Life Inventory, PedsQL v4.0	After a 20 week Exercise training program
Self-perceived Quality of life - Cardiac module	By implementing a prescribed exercise training regime over a 20 week period, an improvement will be noted in self-reported quality of life, which will be assessed using validated QOL questionnaire - Using PedsQL Cardiac Module v3.0	After a 20 week Exercise training program
Skeletal muscle mass	By implementing a prescribed exercise training regime over a 20 week period, an improvement will be noted in the skeletal muscle mass, which will be assessed using a non-invasive bio-impedance analyser.	After a 20 week Exercise training program

Collaborators and Investigators

• Sponsor: Children's Health Ireland at Crumlin
• Collaborators: CHI Research & Innovation Office
• Investigators: Principal Investigator: Adam James, MD, Children's Health Ireland at Crumlin

Study record dates

Study Major Dates

• Study Start (Anticipated): January 10, 2023
• Primary Completion (Anticipated): December 31, 2023
• Study Completion (Anticipated): December 31, 2023

Study Registration Dates

• First Submitted: October 24, 2022
• First Submitted That Met QC Criteria: November 14, 2022
• First Posted (Actual): November 15, 2022

Study Record Updates

• Last Update Posted (Estimate): December 21, 2022
• Last Update Submitted That Met QC Criteria: December 20, 2022
• Last Verified: December 1, 2022

More Information

Terms related to this study

• Keywords: Quality of Life; Exercise Training; Fontan; Ventricular Function
• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Heart Defects, Congenital; Cardiovascular Abnormalities; Univentricular Heart
• Other Study ID Numbers: RSFG-21-ACC13

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