21 YEARS FOLLOW-UP OF LIVER FUNCTION IN PATIENTS AFTER FONTAN OPERATION

Introduction
Fontan circulation is associated with suboptimal outcome due to chronic venous congestion. The aim of this longitudinal study was to analyse the influence of long-term venous congestion on liver function in patients with univentricular circulation after total cavopulmonary connection (TCPC).
Methods
47 patients (22 women), who underwent TCPC at median (IQR) age of 4 (3-6) years, were repeatedly examined during subsequent follow-up Studies 1-3 at 5 (3-6), 13 (11-14) and 21 (20-22) years after TCPC, respectively. Cardiac function was semiquantitatively evaluated by echocardiography, liver function was examined by a panel of biochemical and haematological tests. Moreover, liver elastography, enhanced liver fibrosis test (ELF) and quality of life were evaluated in Study 3.
Results
Progressive impairment of liver function was found between the Studies. There was a significant increase in levels of gamma glutamyl transferase (p<0.001), bilirubin (p=0.001), the FIB-4 (p<0.001) and Forns index (p <0.001); and decrease in levels of prealbumin (p<0.001). In Study 3 abnormal ELF score (>7.7) was found in 95% of patients and abnormal elastography (>7.2 kPa) in all subjects. Good systolic ventricular function was present in 83% subjects. 17% of patients rated their health as excellent, 51% as very good, 23% as good, 9% as fair and none as poor. There was no correlation between the liver tests and ventricular function nor the subjective quality of life.
Conclusion
Progressive impairment of liver function occurs during long term follow-up majority of patients after TCPC despite preserved single ventricle function and good quality of life.