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Hepatic dysfunction following the Fontan procedure

Michael R Narkewicz1, Henry M Sondheimer, James W Ziegler

  • 1Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, Denver, 80218, USA. narkewicz.michael@tchden.org

Journal of Pediatric Gastroenterology and Nutrition
|February 27, 2003
PubMed

Insights

Children undergoing the Fontan procedure show abnormal liver function, indicated by prolonged prothrombin time and altered galactose elimination. These markers are crucial for monitoring hepatic health over time in Fontan patients.

Area of Science:

  • Pediatric Cardiology
  • Hepatology
  • Congenital Heart Disease

Background:

  • The Fontan procedure is a palliative treatment for single-ventricle congenital heart defects.
  • It can lead to elevated systemic venous pressure and hepatic congestion.
  • Long-term hepatic function in these patients requires characterization.

Purpose of the Study:

  • To assess hepatic function in children post-Fontan procedure.
  • To investigate the relationship between cardiac and hepatic function in this cohort.

Main Methods:

  • Cross-sectional study of 11 children (38-216 months old) post-Fontan procedure.
  • Evaluated hepatic function using galactose clearance, Doppler ultrasonography, synthetic function, and liver injury markers.
  • Assessed cardiac function indices.

Main Results:

  • Prolonged prothrombin time and low factor V levels were common biochemical abnormalities.
  • A trend toward worsening prothrombin time with longer intervals post-Fontan was observed.
  • Galactose elimination capacity correlated inversely with time since Fontan (R2=0.65, P=0.004).
  • No significant relationship was found between cardiac function and liver function.

Conclusions:

  • Children post-Fontan procedure exhibit abnormal liver function.
  • Prothrombin time and galactose elimination half-life serve as valuable longitudinal markers for hepatic function assessment in Fontan patients.
Abstract

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