Serial evaluation of hepatic function profile after Fontan operation.

Moderate persistent elevation of the γ-glutamyltransferase (γGT) level is a frequent finding during long-term follow-up of patients with total cavopulmonary connection (TCPC) for palliation of functionally univentricular hearts. Serial intraindividual data revealed a significant increase in the γGT level within a minimum 4-year interval in more than 80 % of cases. The level of γGT elevation showed a significant correlation to hemodynamic parameters such as systemic ventricular end diastolic pressure and mean pulmonary artery pressure, but did not strongly correlate with duration of follow-up or other liver function parameters, which were less frequent and less impressively deranged. None of the patients had signs of synthetic dysfunction. With increasing postoperative follow-up, abnormalities of sonographic hepatic texture including increased echogenicity, inhomogeneity, or liver surface nodularity were found. All 17 patients with liver surface nodularity had a follow-up period of over 10 years. Structural abnormalities did not correlate with biochemical or hemodynamic parameters. Doppler evaluation revealed inspiratory dependence of hepatic vein flow in more than 90 % as a relevant finding after TCPC; a decrease in portal vein flow velocity was observed in many patients. Since long-term survivors after Fontan procedure are at an increased risk of cardiac hepatopathy and cirrhosis, detailed routine investigation and monitoring of hepatic morphology are needed.