Structural and functional uncoupling of liver performance in the Fontan circulation

Indra Neil Guha, Sumit Bokhandi, Zaheer Ahmad, Nick Sheron, Richard Cope, Caroline Marshall, Gruschen Veldtman*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

32 Citations (Scopus)


Background: The liver is known to be structurally abnormal in long-standing Fontan circulation. The degree of liver dysfunction associated with such abnormalities is however largely unknown. We assessed structural changes (serum fibrosis markers) and function (indocyanine green clearance (ICG)) in Fontan patients. Methods: 21 stable Fontan patients were prospectively assessed and compared with 8 histologically proven compensated viral cirrhotic patients. All subjects had standard liver profile, "Enhanced Liver Fibrosis" (ELF) score (including hyaluronic acid, aminoterminal type III procollagen peptide P3NP and tissue inhibitor of metalloproteinase TIMP-1 levels), and ICG using the LiMON Device. Plasma disappearance rate (PDR) and 15-minute retention (R15) were recorded after ICG infusion. Results: Indocyanine clearance and retention (PDR and R15) were similar between Fontan and compensated cirrhotic patients (17 ± 5 vs 18 ± 6 (p = 0.75) and 11 ± 10 vs 10 ± 10 (p = 0.75)), as was degree of fibrosis (7.97 ± 1.16 vs 9.0 ± 1.43, p = NS). There was a positive correlation between PDR and ELF (R = 0.77, p = 0.028) as well as R15 and ELF (R = 0.905, p = 0.002) in the viral cirrhotics but not in the Fontan group. (R = - 0.243, p = 0.302; and R = 0.226, p = 0.338). PDR (17 ± 5) and R15 (11 ± 10) were not significantly different in Fontan as compared with the established cirrhotics. Conclusions: Fontan patients have similar global hepatic function and fibrosis as compared with viral cirrhotic patients. However in Fontan patients, fibrosis was not closely correlated with global liver function, whereas viral cirrhotic patients exhibited a close correlation between function and fibrosis.

Original languageEnglish
Pages (from-to)77-81
Number of pages5
JournalInternational Journal of Cardiology
Issue number1
Publication statusPublished - 20 Mar 2013
Externally publishedYes


  • Cirrhosis
  • Congenital heart disease
  • Fontan
  • Indocyanine green
  • Liver function
  • Single ventricle


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