Background
A chronic imbalance of the autonomic nervous system(ANS) may contribute to long term complications in different congenital heart diseases. The purpose of this study was to determine whether the ANS plays a role in the long-term outcome of patients with Transposition of great arteries(TGA) after arterial switch operation(ASO) as its contribution is as yet not clear.
Methods
The ANS activity was evaluated non-invasively in 26 TGA patients and 52 age-appropriate healthy subjects combining impedance cardiography and electrocardiography. Heart rate, pre-ejection period(sympathetic activity parameter) and respiratory sinus arrhythmia and the root of the mean square of successive normal-to-normal interval differences(parasympathetic activity parameter) were measured during 5 different daily activities(sleep, sitting, active sitting, light and moderate/vigorous physical activity). Whether the ANS activity was related to ventricular function, exercise test performance or clinical outcome in the patient group was also analyzed.
Results
Compared to healthy subjects: heart rate was significantly lower in TGA patients at rest and during quiet and active sitting; sympathetic activity was significantly reduced in patients during physical activity; and the parasympathetic activity was higher in TGA patients while quiet and active sitting. In the patient group a significant positive correlation between 4-chamber longitudinal strain and parasympathetic activity during 3 different daily activities was found.
Conclusions
The sympathetic nervous system response to physical activity is reduced in TGA patients after ASO. Additionally, we observed a positive correlation between better left ventricular function and higher parasympathetic activity that could be in line with the known protective effect of a higher vagal activity.

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 stiffness.