Minimally invasive procedures on the upper urinary tract in children younger than 1 year are technically challenging, and require expertise of the surgeon and the entire team. Given these assumptions, such procedures can be safely performed with excellent functional outcomes.
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.
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