Re-Energize Fontan

RE-ENERGIZE FONTAN: A RandomizEd Exercise INtERvention DesiGned to MaximIZE Fitness in Pediatric FONTAN Patients

Survival of children with single ventricles ("half a heart") beyond the neonatal period has increased dramatically with the staged Fontan palliation. Yet, long-term morbidity remains high. By the age of 40, 50% of Fontan patients will have died or undergone heart transplantation. With >1,000 Fontan palliations performed in the US annually, there is a burgeoning population of Fontan patients at risk for progressive heart failure and death. Factors that contribute to onset and progression of heart failure in Fontan patients remain incompletely understood. However, it is established that Fontan patients have poor exercise capacity, associated with a greater risk of morbidity and mortality, in addition to decreased muscle mass, abnormal muscle function, and endothelial dysfunction contributing to disease progression. In adult patients with two ventricles and heart failure, reduced exercise capacity, muscle mass, and muscle strength are powerful predictors of poor outcomes, and exercise interventions can not only improve exercise capacity and muscle mass, but also reverse endothelial dysfunction. Limited exercise interventions in children with congenital heart disease have demonstrated that exercise is safe and effective; however, these studies have been conducted in small, heterogeneous groups, and most had few Fontan patients. Furthermore, none of these interventions have studied the impact of exercise on muscle mass or mitochondrial function, or endothelial function. The investigators propose a milestone-driven, randomized controlled trial in pediatric Fontan patients to test the hypothesis that a live-video-supervised exercise (aerobic + resistance) intervention will improve cardiac and physical capacity; muscle mass, strength and function; and endothelial function. The investigators' ultimate goal is the translation of this model to clinical application as an "exercise prescription" to intervene early in pediatric Fontan patients and decrease long-term morbidity and mortality.

Study Overview

• Status: Recruiting
• Conditions: Single-ventricle
• Intervention / Treatment: Other: Live-Video-Supervised Exercise Intervention; Other: Usual Care then Live-Video Supervised Exercise Intervention

• Study Type: Interventional
• Enrollment (Estimated): 150
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Seda Tierney; Phone Number: 650-334-7156; Email: tierneys@stanford.edu

Study Locations

• United States
  • California
    • Palo Alto, California, United States, 94304
      • Recruiting
      • Stanford University, Lucile Packard Children Hospital
      • Contact: Seda Tierney, MD; Phone Number: 650-736-7747; Email: fontanstudy@stanford.edu

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 8 years to 19 years (Child, Adult)
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• 8-19 years of age
• Fontan palliation
• Ability to fast overnight
• Cardiac clearance to exercise by primary cardiologist
• Presence of an adult at home during exercise sessions for patients <14 years old
• English-speaking patient.

Exclusion Criteria:

• NYHA Class IV (severe heart failure)
• Acute illness within the past three months
• Active protein losing enteropathy (albumin <2.5 mg/dL)
• Implanted pacemaker
• Cognitive delay deemed severe enough to inhibit the ability to follow the exercise

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Prevention
• Allocation: Randomized
• Interventional Model: Crossover Assignment
• Masking: Double

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Live Video-Supervised Exercise Intervention Arm; Patients randomized to exercise intervention at baseline will participate in live-video-supervised exercise sessions x3/week for 3 months, and then will follow a maintenance regimen for 6 months. During maintenance, patients will continue live-video-supervised exercise sessions, only x1/week, and will be instructed to exercise on their own x2/week following an individualized prescribed exercise program and use their heart rate monitor as an activity tracker.	Other: Live-Video-Supervised Exercise Intervention; The live-video-supervised exercise intervention includes exercise sessions x3/week for 3 months, and then maintenance regimen for 6 months. Maintenance includes live-video-supervised exercise sessions, only x1/week, and self-exercise x2/week following an individualized prescribed exercise program and use their heart rate monitor as an activity tracker.
Experimental: Live-Video-Supervised Exercise Control Arm; Patients randomized to usual care at baseline will receive usual care for 9 months and will then start the 3-month exercise intervention of live-video-supervised exercise sessions x3/week for 3 months.	Other: Usual Care then Live-Video Supervised Exercise Intervention; Patients randomized to usual care at baseline will receive usual care for 9 months and will then start the live-video-supervised exercise intervention that includes exercise sessions x3/week for 3 months.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in volume of Oxygen Consumed at Maximal Exertion	In Fontan patients, peak VO2 is a powerful predictor of poor outcomes. The subjects will undergo progressive cardiopulmonary exercise testing with continuous monitoring by 12-lead EKG and gas exchange by measuring breath-to-breath ventilation. Exercise capacity will be measured by a cardiopulmonary exercise test to obtain the peak VO2	Baseline, 3 months, 9 months, 12 months

Collaborators and Investigators

• Sponsor: Stanford University
• Collaborators: National Heart, Lung, and Blood Institute (NHLBI); The Methodist Hospital Research Institute

Study record dates

Study Major Dates

• Study Start (Actual): August 6, 2020
• Primary Completion (Estimated): October 6, 2024
• Study Completion (Estimated): January 6, 2025

Study Registration Dates

• First Submitted: December 6, 2019
• First Submitted That Met QC Criteria: December 9, 2019
• First Posted (Actual): December 11, 2019

Study Record Updates

• Last Update Posted (Actual): December 28, 2023
• Last Update Submitted That Met QC Criteria: December 20, 2023
• Last Verified: December 1, 2023

More Information

Terms related to this study

• Keywords: Fontan palliation
• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Heart Defects, Congenital; Cardiovascular Abnormalities; Univentricular Heart
• Other Study ID Numbers: 46606; R33HL146775 (U.S. NIH Grant/Contract); 1R61HL146775-01A1 (U.S. NIH Grant/Contract)

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