Re-Energize Fontan

NCT04195451 | Completed DMC

• 3 other identifiers: 46606R33HL1467751R61HL146775-01A1
• Study Type: interventional
• Target: 150
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Single-ventricle

Keywords

Fontan palliation

Outcome Measures

Primary Outcomes (1)

• 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

Study Arms (2)

Live Video-Supervised Exercise Intervention Arm

EXPERIMENTAL

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

Live-Video-Supervised Exercise Control Arm

EXPERIMENTAL

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

Interventions

Usual Care then Live-Video Supervised Exercise Intervention OTHER

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.

Live-Video-Supervised Exercise Control Arm

Live-Video-Supervised Exercise Intervention OTHER

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.

Live Video-Supervised Exercise Intervention Arm

Eligibility Criteria

• Age: 8 Years - 19 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• 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.

You may not qualify if:

• 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

Sponsors & Collaborators

• Stanford University: lead
• The Methodist Hospital Research Institute: collaborator
• National Heart, Lung, and Blood Institute (NHLBI): collaborator

Study Sites (1)

Stanford University, Lucile Packard Children Hospital

Palo Alto, California, 94304, United States

Location

Related Publications (2)

• Gallegos FN, Bernstein D, Punn R, Long J, Stauffer KJ, Thorson K, Chen S, Lui MC, Olson I, Myers J, Palaniappan L, Tierney S. Systolic performance of the single ventricle, exercise capacity, and endothelial function in pediatric Fontan patients. Am Heart J. 2025 Dec;290:58-68. doi: 10.1016/j.ahj.2025.05.015. Epub 2025 May 31.

  PMID: 40456431 DERIVED

• Selamet Tierney ES, Palaniappan L, Leonard M, Long J, Myers J, Davila T, Lui MC, Kogan F, Olson I, Punn R, Desai M, Schneider LM, Wang CH, Cooke JP, Bernstein D. Design and rationale of re-energize fontan: Randomized exercise intervention designed to maximize fitness in fontan patients. Am Heart J. 2023 May;259:68-78. doi: 10.1016/j.ahj.2023.02.006. Epub 2023 Feb 14.

  PMID: 36796574 DERIVED

MeSH Terms

Conditions

Univentricular Heart

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: DOUBLE
• Who Masked: INVESTIGATOR, OUTCOMES ASSESSOR
• Purpose: PREVENTION
• Intervention Model: CROSSOVER
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Associate Professor, Pediatrics

Study Record Dates

• First Submitted: December 6, 2019
• First Posted: December 11, 2019
• Study Start: August 6, 2020
• Primary Completion: March 1, 2026
• Study Completion: March 1, 2026
• Last Updated: April 24, 2026

Record last verified: 2025-01

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)