Abstract
BACKGROUND: Exercise capacity is reduced in patients with a Fontan circulation compared to their healthy peers secondary to altered haemodynamics of the Fontan circulation. Cardiopulmonary exercise testing is routinely used in Fontan patients to assess their cardiopulmonary response to exercise and help guide management decisions, however their results are routinely compared to normative data derived from healthy individuals. METHOD: Using data available from the Pediatric Heart Network, we performed a retrospective study to derive Fontan-specific normative values in a subgroup of well-functioning adolescent patients. We utilised results from the Congenital Heart Adolescent and Teenager (CHAT) Questionnaire to classify patients into well-functioning and lower-functioning groups. RESULTS: We generated Fontan-specific regression equations in adolescent male and females for normative values of peak oxygen consumption (VO(2)), maximal workload and VO(2) at anaerobic threshold. CONCLUSION: We propose that Fontan-specific normative values are a more useful comparison than healthy normative data due to the known circulatory limitations of the Fontan circulation and will assist in management decision and prognostication.