HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS REGISTER		Author Keyword(s) Vol Page [Advanced]

This Article Full Text Full Text (PDF) All Versions of this Article: jcp.2007.052365v1 61/4/504    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Add article to my folders Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Kendall, T J Articles by Iredale, J P Search for Related Content PubMed PubMed Citation Articles by Kendall, T J Articles by Iredale, J P

Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study

¹ Department of Cellular Pathology, University Hospital of Southampton, Southampton, UK
² Department of Radiology, University Hospital of Southampton, Southampton, UK
³ Liver Research Unit, University Hospital of Southampton, Southampton, UK
⁴ Congenital Cardiac Centre, University Hospital of Southampton, Southampton, UK
⁵ MRC Centre for Inflammation Research, Queen’s Medical Research Institute, University of Edinburgh, Edinburgh, UK

Correspondence to:
Dr Timothy J Kendall, Department of Histopathology, Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh EH16 4TJ, UK; kendall.tim{at}gmail.com

Aims: 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.

Discussion: 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.