title: Hepatic changes in the failing Fontan circulation.
abstract: BACKGROUND
  The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined.
  
  
  OBJECTIVE
  To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings.
  
  
  METHODS
  Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years.
  
  
  RESULTS
  Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038).
  
  
  CONCLUSIONS
  Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.
authors.affiliation: Southampton General Hospital, University Hospitals Southampton, Southampton, UK.
authors.forename: Christoph H
authors.initials: CH
authors.lastname: Kiesewetter
authors.forename: Nick
authors.initials: N
authors.lastname: Sheron
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Vettukattill
authors.forename: Nigel
authors.initials: N
authors.lastname: Hacking
authors.forename: Brian
authors.initials: B
authors.lastname: Stedman
authors.forename: Harry
authors.initials: H
authors.lastname: Millward-Sadler
authors.forename: Marcus
authors.initials: M
authors.lastname: Haw
authors.forename: Richard
authors.initials: R
authors.lastname: Cope
authors.forename: Anthony P
authors.initials: AP
authors.lastname: Salmon
authors.forename: Muthukumaran C
authors.initials: MC
authors.lastname: Sivaprakasam
authors.forename: Tim
authors.initials: T
authors.lastname: Kendall
authors.forename: Barry R
authors.initials: BR
authors.lastname: Keeton
authors.forename: John P
authors.initials: JP
authors.lastname: Iredale
authors.forename: Gruschen R
authors.initials: GR
authors.lastname: Veldtman
mesh_terms: D000293:Adolescent; D000328:Adult; D001794:Blood Pressure / Q000502:physiology; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D006801:Humans; D008102:Liver Circulation / Q000502:physiology*; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D008111:Liver Function Tests; D008297:Male; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed