title: Serial evaluation of hepatic function profile after Fontan operation.
abstract: 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.
authors: Division Pediatric Cardiology and Pediatric Intensive Care Medicine, Tuebingen University Children's Hospital, Hoppe-Seyler Str. 1, 72076, Tuebingen, Germany, renate.kaulitz@med.uni-tuebingen.de.
authors: R
authors: R
authors: Kaulitz
authors: P
authors: P
authors: Haber
authors: E
authors: E
authors: Sturm
authors: J
authors: J
authors: Schäfer
authors: M
authors: M
authors: Hofbeck
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008099:Liver / Q000503:physiopathology*; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D008111:Liver Function Tests; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
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