Journal Article

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

Celermajer, David S., Davis, Glen M., d'Udekem, Yves, Clendenning, Carley, Tran, Derek L., Ayer, Julian, Hornung, Tim, Justo, Robert, Cordina, Rachael, Grigg, Leeanne

2021

Overview

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 (VO 2 ) 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 VO 2 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 VO 2 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.

• Publisher: Frontiers Media SA,Frontiers Media S.A
• Subject: Age / Anaerobic threshold / Blood pressure / Body mass index / cardiac rehabilitation / Cardiovascular Medicine / congenital heart disease / Demographics / Exercise / exercise capacity / exercise training / Genotype & phenotype / Oxygen saturation / physical activity / Physical fitness / Quality of life / Questionnaires / Self-efficacy / Spirometry / Statistical analysis