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The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan

William N Evans1,2, Ruben J Acherman3,4, Gary A Mayman3,4

  • 1Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA. wnevans50@aol.com.

Pediatric Cardiology
|March 4, 2020
PubMed

Insights

Patients with extracardiac Fontan surgery show a faster hepatic fibrosis progression rate compared to those with lateral tunnel Fontan surgery. Further research is needed to confirm these findings in post-Fontan patients.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Surgery

Background:

  • The Fontan procedure is a palliative surgery for complex congenital heart disease.
  • Hepatic fibrosis is a known complication in post-Fontan patients.
  • Different surgical techniques for the Fontan procedure may influence long-term outcomes.

Purpose of the Study:

  • To analyze and compare the rate of hepatic fibrosis progression in patients who underwent lateral tunnel Fontan versus extracardiac Fontan procedures.
  • To identify potential differences in liver health outcomes based on Fontan surgical type.

Main Methods:

  • Retrospective analysis of 126 post-Fontan patients who underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019.
  • Patients were categorized into lateral tunnel Fontan (n=27) and extracardiac Fontan (n=99) groups.
  • Hepatic fibrosis was assessed using total fibrosis scores and rates of progression per year.

Main Results:

  • No significant difference in age at Fontan surgery or average total fibrosis score between the two groups.
  • Extracardiac Fontan patients had a significantly shorter average Fontan duration (11±5 years) compared to lateral tunnel Fontan patients (20±6 years).
  • The average rate of hepatic fibrosis progression was significantly faster in the extracardiac Fontan group (0.30±0.23/year) than in the lateral tunnel Fontan group (0.16±0.10/year).

Conclusions:

  • Extracardiac Fontan procedures are associated with a faster rate of hepatic fibrosis progression compared to lateral tunnel Fontan procedures.
  • These findings suggest a potential difference in liver disease development based on Fontan surgical technique.
  • Larger, multi-institutional studies are recommended to validate these results and determine clinical significance.

Keywords:
Cardiac catheterizationFontanHepatic biopsyHepatic fibrosis

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