ORIGINAL ARTICLES

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

T J Kendall¹, B Stedman², N Hacking², M Haw³, J J Vettukattill³, A P Salmon³, R Cope³, N Sheron⁴, H Millward-Sadler¹, G R Veldtman³ and J P Iredale⁵

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.

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