Table 2.

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.