Frontiers in Pediatrics (Jan 2022)
Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)—Rationale and Design
- Derek L. Tran,
- Derek L. Tran,
- Derek L. Tran,
- Derek L. Tran,
- Hannah Gibson,
- Andrew J. Maiorana,
- Andrew J. Maiorana,
- Charlotte E. Verrall,
- Charlotte E. Verrall,
- David W. Baker,
- David W. Baker,
- Melanie Clode,
- David R. Lubans,
- Diana Zannino,
- Andrew Bullock,
- Suzie Ferrie,
- Julie Briody,
- Peter Simm,
- Vishva Wijesekera,
- Michelle D'Almeida,
- Sally E. Gosbell,
- Sally E. Gosbell,
- Sally E. Gosbell,
- Glen M. Davis,
- Robert Weintraub,
- Robert Weintraub,
- Anthony C. Keech,
- Anthony C. Keech,
- Anthony C. Keech,
- Rajesh Puranik,
- Rajesh Puranik,
- Martin Ugander,
- Robert Justo,
- Dominica Zentner,
- Dominica Zentner,
- Avik Majumdar,
- Avik Majumdar,
- Leeanne Grigg,
- Leeanne Grigg,
- Jeff S. Coombes,
- Yves d'Udekem,
- Norman R. Morris,
- Norman R. Morris,
- Julian Ayer,
- Julian Ayer,
- David S. Celermajer,
- David S. Celermajer,
- David S. Celermajer,
- Rachael Cordina,
- Rachael Cordina,
- Rachael Cordina,
- Rachael Cordina
Affiliations
- Derek L. Tran
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- Derek L. Tran
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Derek L. Tran
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Derek L. Tran
- Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia
- Hannah Gibson
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Andrew J. Maiorana
- School of Allied Health, Curtin University, Perth, WA, Australia
- Andrew J. Maiorana
- Allied Health Department, Fiona Stanley Hospital, Perth, WA, Australia
- Charlotte E. Verrall
- The University of Sydney Westmead Clinical School, Sydney, NSW, Australia
- Charlotte E. Verrall
- Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
- David W. Baker
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- David W. Baker
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Melanie Clode
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
- David R. Lubans
- 0School of Education, Priority Research Centre for Physical Activity and Nutrition, The University of Newcastle, Newcastle, NSW, Australia
- Diana Zannino
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
- Andrew Bullock
- 1Paediatric and Adult Congenital Cardiology, Perth Children's Hospital, Perth, WA, Australia
- Suzie Ferrie
- 2Department of Nutrition and Dietetics, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- Julie Briody
- 3Department of Nuclear Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia
- Peter Simm
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
- Vishva Wijesekera
- 4Department of Cardiology, The Prince Charles Hospital, Brisbane, QLD, Australia
- Michelle D'Almeida
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Sally E. Gosbell
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Sally E. Gosbell
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Sally E. Gosbell
- Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
- Glen M. Davis
- Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia
- Robert Weintraub
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
- Robert Weintraub
- 5Department of Cardiology, The Royal Children's Hospital, Melbourne, VIC, Australia
- Anthony C. Keech
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- Anthony C. Keech
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Anthony C. Keech
- 6NHMRC Clinical Trials Centre, The University of Sydney, Sydney, NSW, Australia
- Rajesh Puranik
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- Rajesh Puranik
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Martin Ugander
- 7Royal North Shore Hospital, The Kolling Institute, Sydney, NSW, Australia
- Robert Justo
- 8Paediatric Cardiac Service, Queensland Children's Hospital, Brisbane, QLD, Australia
- Dominica Zentner
- 9The University of Melbourne Medical School, Melbourne, VIC, Australia
- Dominica Zentner
- 0Department of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, Australia
- Avik Majumdar
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Avik Majumdar
- 1Australian National Liver Transplant Unit, AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
- Leeanne Grigg
- 9The University of Melbourne Medical School, Melbourne, VIC, Australia
- Leeanne Grigg
- 0Department of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, Australia
- Jeff S. Coombes
- 2School of Human Movement and Nutrition Sciences, Centre for Research on Exercise, Physical Activity, and Health, The University of Queensland, Brisbane, QLD, Australia
- Yves d'Udekem
- 3Division of Cardiac Surgery, Children's National Hospital, Washington, DC, United States
- Norman R. Morris
- 4Allied Health Collaborative and Queensland Lung Transplant Service, The Prince Charles Hospital, Brisbane, QLD, Australia
- Norman R. Morris
- 5School of Health Sciences and Social Work, Griffith University, Gold Coast, QLD, Australia
- Julian Ayer
- The University of Sydney Westmead Clinical School, Sydney, NSW, Australia
- Julian Ayer
- Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
- David S. Celermajer
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- David S. Celermajer
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- David S. Celermajer
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Rachael Cordina
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
- Rachael Cordina
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
- Rachael Cordina
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
- Rachael Cordina
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
- DOI
- https://doi.org/10.3389/fped.2021.799125
- Journal volume & issue
-
Vol. 9
Abstract
Background: Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe—and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. The mechanisms underlying improvements in aerobic exercise capacity and the effects of exercise training on circulatory and end-organ function remain incompletely understood. Furthermore, the optimal methods of exercise prescription are poorly characterized. This highlights the need for large, well-designed, multi-center, randomized, controlled trials.Aims and Methods: The Fontan Fitness Intervention Trial (F-FIT)—a phase III clinical trial—aims to optimize exercise prescription and delivery in people with a Fontan circulation. In this multi-center, randomized, controlled study, eligible Fontan participants will be randomized to either a 4-month supervised aerobic and resistance exercise training program of moderate-to-vigorous intensity followed by an 8-month maintenance phase; or usual care (control group). Adolescent and adult (≥16 years) Fontan participants will be randomized to either traditional face-to-face exercise training, telehealth exercise training, or usual care in a three-arm trial with an allocation of 2:2:1 (traditional:telehealth:control). Children (<16 years) will be randomized to either a physical activity and exercise program of moderate-to-vigorous intensity or usual care in a two-arm trial with a 1:1 allocation. The primary outcome is a change in aerobic exercise capacity (peak oxygen uptake) at 4-months. Secondary outcomes include safety, and changes in cardiopulmonary exercise testing measures, peripheral venous pressure, respiratory muscle and lung function, body composition, liver stiffness, neuropsychological and neurocognitive function, physical activity levels, dietary and nutritional status, vascular function, neurohormonal activation, metabolites, cardiac function, quality of life, musculoskeletal fitness, and health care utilization. Outcome measures will be assessed at baseline, 4-months, and 12-months. This manuscript will describe the pathophysiology of exercise intolerance in the Fontan circulation and the rationale and protocol for the F-FIT.
Keywords
- aerobic exercise
- cardiac rehabilitation
- single ventricle
- congenital heart disease
- telehealth
- exercise intolerance
