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Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study
  1. Timothy Kendall (kendall.tim{at}gmail.com)
  1. Department of Cellular Pathology, University Hospital of Southampton, Southampton, United Kingdom
    1. Brian Stedman
    1. Department of Radiology, University Hospital of Southampton, Southampton, United Kingdom
      1. Nigel Hacking
      1. Department of Radiology, University Hospital of Southampton, Southampton, United Kingdom
        1. Marcus Haw
        1. Congenital Cardiac Centre, University Hospital of Southampton, Southampton, United Kingdom
          1. Joseph Vettukattill
          1. Congenital Cardiac Centre, University Hospital of Southampton, Southampton, United Kingdom
            1. Anthony Salmon
            1. Congenital Cardiac Centre, University Hospital of Southampton, Southampton, United Kingdom
              1. Richard Cope
              1. Congenital Cardiac Centre, University Hospital of Southampton, Southampton, United Kingdom
                1. Nick Sheron
                1. Liver Group, University of Southampton, Southampton, United Kingdom
                  1. Harry Millward-Sadler
                  1. Department of Cellular Pathology, University Hospital of Southampton, Southampton, United Kingdom
                    1. Gruschen Veldtman (gruschen.veldtman{at}suht.swest.nhs.uk)
                    1. Congenital Cardiac Centre, University Hospital of Southampton, Southampton, United Kingdom
                      1. John Iredale (jiredale{at}uun.ed.ac.uk)
                      1. Centre for Inflammation Research, University of Edinburgh, Edinburgh, United Kingdom

                        Abstract

                        Aims: To describe the histological features of the liver in patients with a Fontan circulation. Methods: 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, biopsies were also ranked by eye for severity to allow correlation with assigned scores. Results: Liver biopsies from 18 patients with a Fontan circulation were assessed. All biopsies showed sinusoidal fibrosis. In 17/18 cases there was at least fibrous spur formation, with 14/18 showing bridging fibrosis and 2/18 demonstrating frank cirrhosis. In 17/18 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 biopsies showed marked sinusoidal dilation, and 14/18 showed bile ductular proliferation. 1/18 biopsy showed minimal iron deposition, 1/18 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.

                        • Fibrosis
                        • Fontan Procedure
                        • Liver
                        • Liver Cirrhosis

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