Derek L Tran1, Cameron Rodrigues2, Karin du Plessis3, Diana Zannino2, Glen M Davis4, David S Celermajer5, Yves d'Udekem6, Rachael Cordina7. 1. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney School of Health Sciences, University of Sydney, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; Heart Research Institute, Sydney, NSW, Australia. 2. Murdoch Children's Research Institute, Melbourne, Vic, Australia. 3. Murdoch Children's Research Institute, Melbourne, Vic, Australia; Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, Vic, Australia. 4. Sydney School of Health Sciences, University of Sydney, Sydney, NSW, Australia. 5. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; Heart Research Institute, Sydney, NSW, Australia. 6. Murdoch Children's Research Institute, Melbourne, Vic, Australia; Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, Vic, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Melbourne, Vic, Australia. 7. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; Heart Research Institute, Sydney, NSW, Australia; Murdoch Children's Research Institute, Melbourne, Vic, Australia. Electronic address: rachael.cordina@sydney.edu.au.
Abstract
BACKGROUND: In people with a Fontan circulation, serial cardiopulmonary exercise testing (CPET) to evaluate change in peak exercise capacity has been increasingly recognised as a useful prognostic tool; a decline is associated with adverse clinical outcomes. The aim of this study is to describe the "natural" history of exercise capacity in the Australian and New Zealand (ANZ) Fontan cohort and to identify factors associated with a decline. METHODS: The ANZ Fontan registry was retrospectively reviewed for adolescent and adult patients (≥16 years) with serial CPET results performed on a cycle ergometer ≥6 months apart. Patients were excluded if they underwent a surgical procedure or fenestration closure in-between tests or if the tests were considered as submaximal effort. Exercise capacity trajectory was defined as the change in percentage of predicted peak oxygen uptake (% pred VO2peak) points per year. RESULTS: Thirty-seven (37) patients (59.5% male, mean age 24±7 years) were eligible. Average duration between CPET was 5.3±3.9 years. At baseline, % pred VO2peak was 61.3±14.5%. Thirteen (13) (35%) had a systemic right ventricle, and 14 (38%) had an atriopulmonary type Fontan circulation. Average change in % pred VO2peak overall was +1.3±6.4 percentage points per year. Sixteen (16) had a negative exercise capacity trajectory, and the average decline in that group was -2.7±3.4 percentage points per year. There was no association between exercise capacity trajectory and clinical characteristics. Of the 18 patients with physical activity levels recorded, 12 (67%) were physically active and % pred VO2peak in that group increased by 2.7±4.0 percentage points per year compared with the physically inactive group who fell by 0.5±0.8 percentage points per year. CONCLUSIONS: In this ANZ series of Fontan patients, over half of our cohort had stable, or an increase, in peak exercise capacity. Regular participation in physical activity was common in patients with a positive exercise capacity trajectory. Clinical characteristics were not associated with exercise capacity trajectory.
BACKGROUND: In people with a Fontan circulation, serial cardiopulmonary exercise testing (CPET) to evaluate change in peak exercise capacity has been increasingly recognised as a useful prognostic tool; a decline is associated with adverse clinical outcomes. The aim of this study is to describe the "natural" history of exercise capacity in the Australian and New Zealand (ANZ) Fontan cohort and to identify factors associated with a decline. METHODS: The ANZ Fontan registry was retrospectively reviewed for adolescent and adult patients (≥16 years) with serial CPET results performed on a cycle ergometer ≥6 months apart. Patients were excluded if they underwent a surgical procedure or fenestration closure in-between tests or if the tests were considered as submaximal effort. Exercise capacity trajectory was defined as the change in percentage of predicted peak oxygen uptake (% pred VO2peak) points per year. RESULTS: Thirty-seven (37) patients (59.5% male, mean age 24±7 years) were eligible. Average duration between CPET was 5.3±3.9 years. At baseline, % pred VO2peak was 61.3±14.5%. Thirteen (13) (35%) had a systemic right ventricle, and 14 (38%) had an atriopulmonary type Fontan circulation. Average change in % pred VO2peak overall was +1.3±6.4 percentage points per year. Sixteen (16) had a negative exercise capacity trajectory, and the average decline in that group was -2.7±3.4 percentage points per year. There was no association between exercise capacity trajectory and clinical characteristics. Of the 18 patients with physical activity levels recorded, 12 (67%) were physically active and % pred VO2peak in that group increased by 2.7±4.0 percentage points per year compared with the physically inactive group who fell by 0.5±0.8 percentage points per year. CONCLUSIONS: In this ANZ series of Fontan patients, over half of our cohort had stable, or an increase, in peak exercise capacity. Regular participation in physical activity was common in patients with a positive exercise capacity trajectory. Clinical characteristics were not associated with exercise capacity trajectory.
Authors: Derek L Tran; Hannah Gibson; Andrew J Maiorana; Charlotte E Verrall; 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; Glen M Davis; Robert Weintraub; Anthony C Keech; Rajesh Puranik; Martin Ugander; Robert Justo; Dominica Zentner; Avik Majumdar; Leeanne Grigg; Jeff S Coombes; Yves d'Udekem; Norman R Morris; Julian Ayer; David S Celermajer; Rachael Cordina Journal: Front Pediatr Date: 2022-01-06 Impact factor: 3.418
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 Journal: Front Cardiovasc Med Date: 2021-12-07