The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

Background: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance (“Super-Fontan”), which may represent a low-risk phenotype.Methods: People with a “Super-Fontan” phenotype were defined as achieving normal exercise pe...

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Main Authors: Derek L. Tran, David S. Celermajer, Julian Ayer, Leeanne Grigg, Carley Clendenning, Tim Hornung, Robert Justo, Glen M. Davis, Yves d'Udekem, Rachael Cordina
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-12-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2021.764273/full
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author Derek L. Tran
Derek L. Tran
Derek L. Tran
David S. Celermajer
David S. Celermajer
David S. Celermajer
Julian Ayer
Julian Ayer
Leeanne Grigg
Leeanne Grigg
Carley Clendenning
Tim Hornung
Robert Justo
Robert Justo
Glen M. Davis
Yves d'Udekem
Yves d'Udekem
Rachael Cordina
Rachael Cordina
Rachael Cordina
Rachael Cordina
author_facet Derek L. Tran
Derek L. Tran
Derek L. Tran
David S. Celermajer
David S. Celermajer
David S. Celermajer
Julian Ayer
Julian Ayer
Leeanne Grigg
Leeanne Grigg
Carley Clendenning
Tim Hornung
Robert Justo
Robert Justo
Glen M. Davis
Yves d'Udekem
Yves d'Udekem
Rachael Cordina
Rachael Cordina
Rachael Cordina
Rachael Cordina
author_sort Derek L. Tran
collection DOAJ
description Background: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance (“Super-Fontan”), which may represent a low-risk phenotype.Methods: People with a “Super-Fontan” phenotype were defined as achieving normal exercise performance [≥80% predicted peak oxygen uptake (VO2) and work rate] during cardiopulmonary exercise testing (CPET) and were identified from the Australian and New Zealand Fontan Registry. A Fontan control group that included people with impaired exercise performance (<80% predicted VO2 or work rate) was also identified based on a 1:3 allocation ratio. A subset of participants were prospectively recruited and completed a series of physical activity, exercise self-efficacy, and health-related quality of life questionnaires.Results: Sixty CPETs (“Super-Fontan”, n = 15; control, n = 45) were included. A subset (“Super-Fontan”, n = 10; control, n = 13) completed a series of questionnaires. Average age was 29 ± 8 years; 48% were males. Exercise capacity reflected by percent predicted VO2 was 67 ± 17% in the entire cohort. Compared to the “Super-Fontan” phenotype, age at Fontan completion was higher in controls (4.0 ± 2.9 vs. 7.2 ± 5.3 years, p = 0.002). Only one (7%) person in the “Super-Fontan” group had a dominant right ventricle compared to 15 (33%) controls (p = 0.043). None of those in the “Super-Fontan” group were obese, while almost a quarter (22%) of controls were obese based on body mass index (p = 0.046). Lung function abnormalities were less prevalent in the “Super-Fontan” group (20 vs. 70%, p = 0.006). Exercise self-efficacy was greater in the “Super-Fontan” group (34.2 ± 3.6 vs. 27.9 ± 7.2, p = 0.02). Self-reported sports participation and physical activity levels during childhood and early adulthood were higher in the “Super-Fontan” group (p < 0.05). The total average time spent participating in structured sports and physical activity was 4.3 ± 2.6 h/wk in the “Super-Fontan” group compared to 2.0 ± 3.0 h/wk in controls, p = 0.003. There were no differences in self-reported current total physical activity score or health-related quality of life between groups (p ≥ 0.05).Conclusions: The “Super-Fontan” phenotype is associated with a healthy weight, lower age at Fontan completion, better exercise self-efficacy, and higher overall levels of sport and physical activity participation during physical development.
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spelling doaj.art-95183216a2e949bb89ffa9d869da0d9a2022-12-21T23:12:06ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2021-12-01810.3389/fcvm.2021.764273764273The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical PerformanceDerek L. Tran0Derek L. Tran1Derek L. Tran2David S. Celermajer3David S. Celermajer4David S. Celermajer5Julian Ayer6Julian Ayer7Leeanne Grigg8Leeanne Grigg9Carley Clendenning10Tim Hornung11Robert Justo12Robert Justo13Glen M. Davis14Yves d'Udekem15Yves d'Udekem16Rachael Cordina17Rachael Cordina18Rachael Cordina19Rachael Cordina20Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, AustraliaCentral Clinical School, The University of Sydney School of Medicine, Sydney, NSW, AustraliaCharles Perkins Centre, Heart Research Institute, Sydney, NSW, AustraliaDepartment of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, AustraliaCentral Clinical School, The University of Sydney School of Medicine, Sydney, NSW, AustraliaCharles Perkins Centre, Heart Research Institute, Sydney, NSW, AustraliaHeart Centre for Children, The Sydney Children's Hospital Network, Sydney, NSW, AustraliaThe Children's Hospital at Westmead Clinical School, Sydney, NSW, AustraliaDepartment of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, AustraliaThe University of Melbourne School of Medicine, Melbourne, VIC, AustraliaMurdoch Children's Research Institute, Melbourne, VIC, AustraliaGreen Lane Paediatric and Congenital Cardiac Service, Starship Hospital, Auckland, New Zealand0Paediatric Cardiac Service, Queensland Children's Hospital, Brisbane, QLD, Australia1The University of Queensland School of Medicine, Brisbane, QLD, Australia2Discipline of Exercise and Sports Science, Sydney School of Health Sciences, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia3The George Washington University School of Medicine and Health Sciences, Washington, DC, United States4Division of Cardiovascular Surgery, Children's National Hospital, Washington, DC, United StatesDepartment of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, AustraliaCentral Clinical School, The University of Sydney School of Medicine, Sydney, NSW, AustraliaCharles Perkins Centre, Heart Research Institute, Sydney, NSW, AustraliaMurdoch Children's Research Institute, Melbourne, VIC, AustraliaBackground: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance (“Super-Fontan”), which may represent a low-risk phenotype.Methods: People with a “Super-Fontan” phenotype were defined as achieving normal exercise performance [≥80% predicted peak oxygen uptake (VO2) and work rate] during cardiopulmonary exercise testing (CPET) and were identified from the Australian and New Zealand Fontan Registry. A Fontan control group that included people with impaired exercise performance (<80% predicted VO2 or work rate) was also identified based on a 1:3 allocation ratio. A subset of participants were prospectively recruited and completed a series of physical activity, exercise self-efficacy, and health-related quality of life questionnaires.Results: Sixty CPETs (“Super-Fontan”, n = 15; control, n = 45) were included. A subset (“Super-Fontan”, n = 10; control, n = 13) completed a series of questionnaires. Average age was 29 ± 8 years; 48% were males. Exercise capacity reflected by percent predicted VO2 was 67 ± 17% in the entire cohort. Compared to the “Super-Fontan” phenotype, age at Fontan completion was higher in controls (4.0 ± 2.9 vs. 7.2 ± 5.3 years, p = 0.002). Only one (7%) person in the “Super-Fontan” group had a dominant right ventricle compared to 15 (33%) controls (p = 0.043). None of those in the “Super-Fontan” group were obese, while almost a quarter (22%) of controls were obese based on body mass index (p = 0.046). Lung function abnormalities were less prevalent in the “Super-Fontan” group (20 vs. 70%, p = 0.006). Exercise self-efficacy was greater in the “Super-Fontan” group (34.2 ± 3.6 vs. 27.9 ± 7.2, p = 0.02). Self-reported sports participation and physical activity levels during childhood and early adulthood were higher in the “Super-Fontan” group (p < 0.05). The total average time spent participating in structured sports and physical activity was 4.3 ± 2.6 h/wk in the “Super-Fontan” group compared to 2.0 ± 3.0 h/wk in controls, p = 0.003. There were no differences in self-reported current total physical activity score or health-related quality of life between groups (p ≥ 0.05).Conclusions: The “Super-Fontan” phenotype is associated with a healthy weight, lower age at Fontan completion, better exercise self-efficacy, and higher overall levels of sport and physical activity participation during physical development.https://www.frontiersin.org/articles/10.3389/fcvm.2021.764273/fullphysical activitycongenital heart diseaseexercise trainingcardiac rehabilitationexercise capacity
spellingShingle Derek L. Tran
Derek L. Tran
Derek L. Tran
David S. Celermajer
David S. Celermajer
David S. Celermajer
Julian Ayer
Julian Ayer
Leeanne Grigg
Leeanne Grigg
Carley Clendenning
Tim Hornung
Robert Justo
Robert Justo
Glen M. Davis
Yves d'Udekem
Yves d'Udekem
Rachael Cordina
Rachael Cordina
Rachael Cordina
Rachael Cordina
The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
Frontiers in Cardiovascular Medicine
physical activity
congenital heart disease
exercise training
cardiac rehabilitation
exercise capacity
title The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
title_full The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
title_fullStr The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
title_full_unstemmed The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
title_short The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance
title_sort super fontan phenotype characterizing factors associated with high physical performance
topic physical activity
congenital heart disease
exercise training
cardiac rehabilitation
exercise capacity
url https://www.frontiersin.org/articles/10.3389/fcvm.2021.764273/full
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