Late outcome and variables of cardiopulmonary exercise testing at baseline
| Author/study | Number of deaths/transplantations | Morbidity type reported | HRR | Peak heart rate | Peak VO2 (mL/kg/min) | Peak VO2 Per cent of predicted | VE/VCO2 | RER |
| Diller et al 22 | 22 deaths 6 transplantations | 41% hospitalisation (heart failure, arrhythmia and complications characteristic of the Fontan circulation). | 63±25 | 146±28 | 22.8±7.4 | 51.7±15.4 | – | – |
| Fernandes et al 23 | 16 deaths | 37% combined end point (mortality or a new morbidity: hospitalisation, CHF, thrombosis and protein-losing enteropathy). | – | – | 21.2±6.2 | 57.1±14.1 | – | >1.05 |
| Ohuchi et al 24 | 24 deaths | 19% hospitalisations (arrhythmias, heart failure, hemostatic complications, catheterisation and/or surgical intervention and sudden death). | 56±24 | 145±25 | 27.1±7.4 | 61±15 | 40±7.7 | 1.12±0.07 |
| Nathan et al 26 | 21 deaths 3 transplantations | 26% combined outcome (death, transplantation, hospitalisation for cardiac or Fontan-related events). | – | 74.7±14 % of predicted | 23.5±6.9 | 59.7±14.3 | 36.8±6.9 | 1.13 ±0.11 |
| Egbe et al 25 | 22 deaths 45 cardiac surgery (1 transplantation) | – | – | 135±31 | 22.7±5.4 | 63±11 | 3 5±4 | >1.10 |
| Atz et al 27 | 31 deaths 23 transplantations | Additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%) and protein-losing enteropathy (8%).* | – | – | – | 61±16 | – | >1.10 |
| Cunningham et al 28 | 13 deaths 2 transplantations | 24% combined outcome (death, transplantation or non-elective hospitalisation for heart failure). 38% combined outcome (death, transplantation or hospitalisation for any cardiovascular or Fontan-related cause). | – | 79.3±13.0 % of predicted | 22.0±5.7 | 60.9±13.7 | 33.0±6.7 | 1.20 |
*Cumulative complications since the Fontan procedure.
HHR, heart rate reserve; RER, respiratory exchange ratio; VO2, oxygen uptake; VE/VCO2, minute ventilation/carbon dioxide production; CHF, congestive heart failure.