||Background: Although various determinants of exercise limitation in Fontan patients have been studied, most
research has been performed in patients who underwent different surgical procedures with differing haemodynamic characteristics. The aim of the current study was to evaluate non-invasively measured cardiovascular
parameters and their influence on exercise performance in paediatric Fontan patients with an extracardiac
conduit and moderate-good systolic ventricular function.
Methods: Fontan patients, between 8 and 18 years of age, with moderate to good systolic ventricular function and
an extracardiac conduit were included. Exercise performance and cardiovascular assessment, comprising echocardiography, aortic stiffness measurement and ambulatory measurement of cardiac autonomous nervous activity were performed on the same day. Healthy subjects served as controls.
Results: Thirty-six Fontan patients (age 14.0 years) and thirty-five healthy subjects (age 12.8 years) were
included. Compared to controls, Fontan patients had reduced diastolic ventricular function and increased arterial
stiffness. No differences were found in heart rate (HR) and cardiac parasympathetic nervous activity. In Fontan
patients, maximal as well as submaximal exercise capacity was impaired, with the percentage of predicted capacity ranging between 54 and 72%. Chronotropic competence, however, was good with a peak HR of 174 (94%
of predicted). Lower maximal and submaximal exercise capacity was correlated with a higher HR at rest, higher
pulse wave velocity of the aorta and a lower ratio of early and late diastolic flow velocity.
Conclusion: Contemporary paediatric Fontan patients have an impaired exercise capacity with preserved chronotropic competence. Exercise performance correlates with heart rate at rest, diastolic function and aortic