title: Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study.
abstract: OBJECTIVE
  To describe the histological features of the liver in patients with a Fontan circulation.
  
  
  METHODS
  Specimens from liver biopsies carried out as part of preoperative assessment prior to extracardiac cavopulmonary conversion of an older style Fontan were examined and scored semi-quantitatively for pertinent histological features. To support the use of the scoring, biopsy specimens were also ranked by eye for severity to allow correlation with assigned scores.
  
  
  RESULTS
  Liver biopsy specimens from 18 patients with a Fontan circulation were assessed. All specimens showed sinusoidal fibrosis. In 17 cases there was at least fibrous spur formation, with 14 showing bridging fibrosis and 2 showing frank cirrhosis. In 17 cases at least some of the dense or sinusoidal fibrosis was orcein positive, although a larger proportion of the dense fibrous bands were orcein positive compared with the sinusoidal component. All specimens showed marked sinusoidal dilatation, and 14 showed bile ductular proliferation; 1 showed minimal iron deposition, and 1 showed mild lobular lymphocytic inflammation. There was no cholestasis or evidence of hepatocellular damage. Similar appearances were observed in 2 patients with severe tricuspid regurgitation.
  
  
  CONCLUSIONS
  The histological features of the liver in patients with a Fontan circulation are similar to those described in cardiac sclerosis. Sinusoidal dilatation and sinusoidal fibrosis are marked in the Fontan series. The presence of a significant amount of orcein negative sinusoidal fibrosis suggests there may be a remediable component, although the dense fibrous bands are predominantly orcein positive, suggesting chronicity and permanence. No inflammation or hepatocellular damage is evident, suggesting that fibrosis may be mediated by a non-inflammatory mechanism.
authors.affiliation: Department of Cellular Pathology, University Hospital of Southampton, Southampton, UK. kendall.tim@gmail.com
authors.forename: T J
authors.initials: TJ
authors.lastname: Kendall
authors.forename: B
authors.initials: B
authors.lastname: Stedman
authors.forename: N
authors.initials: N
authors.lastname: Hacking
authors.forename: M
authors.initials: M
authors.lastname: Haw
authors.forename: J J
authors.initials: JJ
authors.lastname: Vettukattill
authors.forename: A P
authors.initials: AP
authors.lastname: Salmon
authors.forename: R
authors.initials: R
authors.lastname: Cope
authors.forename: N
authors.initials: N
authors.lastname: Sheron
authors.forename: H
authors.initials: H
authors.lastname: Millward-Sadler
authors.forename: G R
authors.initials: GR
authors.lastname: Veldtman
authors.forename: J P
authors.initials: JP
authors.lastname: Iredale
mesh_terms: D001706:Biopsy; D004108:Dilatation, Pathologic / Q000209:etiology / Q000473:pathology; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D010078:Oxazines / Q000378:metabolism; D012086:Reoperation; D014262:Tricuspid Valve Insufficiency / Q000473:pathology
chemical_list: D010078:Oxazines; C002313:PAcein