Table 1 Scoring criteria used to assess histological features in the livers of Fontan patients
Histological featureScoreComment
Gross architectural distortion (modified from METAVIR system10)0No definite fibrosis
1Minimal fibrosis (no septa or rare thin septum)
2Mild fibrosis (occasional thin septa)
3Moderate fibrosis (moderate thin septa; up to incomplete cirrhosis)
4Cirrhosis definite or probable
Sinusoidal fibrosis (modified from published system11)0No sinusoidal fibrosis
1Sinusoidal fibrosis present in <1/3 sinusoids
2Sinusoidal fibrosis present in 1/3–2/3 sinusoids
3Sinusoidal fibrosis affecting >2/3 sinusoids
Orcein positivity (gross architecture or sinusoidal fibrosis, possibly reflecting matrix maturity12)0None of the fibrotic matrix is orcein positive
1<1/3 fibrotic matrix is orcein positive
21/3–2/3 of fibrotic matrix is orcein positive
3>2/3 fibrotic matrix is orcein positive
Sinusoidal dilatation (previously published system13)0No sinusoidal dilatation
1<1/3 sinusoids dilated
21/3–2/3 sinusoids dilated
3>2/3 sinusoids dilated
Bile ductular reaction (previously published system14)0No ductular reaction
1Mild ductular reaction
2Moderate ductular reaction
3Severe ductular reaction
Cholestasis0No cholestasis
1Cholestasis present
Iron deposition0No iron deposition
1Stainable iron present
Hepatocellular damage0No evidence of hepatocyte damage
1Hepatocyte ballooning, necrosis or apoptosis
Inflammation0No lobular inflammation
1Lobular inflammation