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Updated: May 11, 2026

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Serial evaluation of hepatic function profile after Fontan operation.

R Kaulitz1, P Haber, E Sturm

  • 1Division 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.

Herz
|May 8, 2013
PubMed
Summary

Persistent elevation of gamma-glutamyltransferase (γGT) is common in patients after total cavopulmonary connection (TCPC). Liver texture abnormalities and altered hepatic blood flow are frequent, necessitating regular monitoring for cardiac hepatopathy.

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Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Surgery

Background:

  • Patients with functionally univentricular hearts often undergo total cavopulmonary connection (TCPC) for palliation.
  • Long-term survivors of the Fontan procedure face risks of cardiac hepatopathy and cirrhosis.
  • Elevated liver enzymes, particularly gamma-glutamyltransferase (γGT), are frequently observed in this population.

Purpose of the Study:

  • To investigate the prevalence and clinical significance of liver abnormalities in patients with TCPC.
  • To correlate biochemical liver function, hemodynamic parameters, and sonographic findings in long-term TCPC survivors.
  • To assess the need for routine hepatic monitoring in these patients.

Main Methods:

  • Analysis of serial intraindividual data including γGT levels and liver function tests.
  • Correlation of γGT levels with hemodynamic parameters (systemic ventricular end-diastolic pressure, mean pulmonary artery pressure).
  • Sonographic evaluation of liver texture (echogenicity, inhomogeneity, surface nodularity) and Doppler assessment of hepatic and portal vein flow.

Main Results:

  • Over 80% of patients showed a significant increase in γGT levels over a minimum 4-year interval.
  • γGT elevation correlated with hemodynamic parameters but not strongly with follow-up duration or other liver function tests.
  • Sonographic abnormalities, including liver surface nodularity (in all 17 patients with this finding after >10 years follow-up), were common.
  • Inspiratory dependence of hepatic vein flow (>90%) and decreased portal vein flow velocity were frequent Doppler findings.
  • No patients exhibited signs of synthetic liver dysfunction.

Conclusions:

  • Moderate persistent elevation of γGT and abnormal liver texture are common in long-term TCPC survivors.
  • Hemodynamic status significantly influences γGT levels, highlighting the link to cardiac physiology.
  • Routine hepatic morphology investigation and monitoring are crucial for early detection of cardiac hepatopathy in these high-risk patients.