title: Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function.
abstract: BACKGROUND
  Liver cirrhosis is recognized with long-term follow-up of patients after the Fontan procedure. The effect of liver cirrhosis on the use of heart transplant (HT) and on post-HT outcomes is unknown.
  
  
  METHODS
  We reviewed Fontan patients evaluated for HT from 2004 to 2012 with hepatic computed tomography (CT) imaging, classified as normal, non-cirrhotic changes, or cirrhosis. The primary outcome was 1-year all-cause mortality, and the secondary outcome was differences in serial post-HT liver evaluation.
  
  
  RESULTS
  CT imaging in 32 Fontan patients evaluated for HT revealed 20 (63%) with evidence of liver disease, including 13 (41%) with cirrhosis. Twenty underwent HT, including 5 non-cirrhotic and 7 cirrhosis patients. Characteristics at listing between normal or non-cirrhotic (n = 13) and cirrhosis (n = 7) groups were similar, except cirrhosis patients were older (median 17.6 vs 9.6 years, p = 0.002) and further from Fontan (median 180 vs 50 months, p < 0.05). Serial liver evaluation was similar, including aspartate aminotransferase, alanine aminotransferase, bilirubin, albumin, and tacrolimus dose at 1, 3, 6, 9, and 12 months. Overall patient survival was 80% at 1 year, with no difference between cirrhosis and non-cirrhosis patients (86% vs 77%, p = 0.681). Liver biopsies were performed in 7 patients before HT, and all specimens showed architectural changes with bridging fibrosis.
  
  
  CONCLUSIONS
  Most patients evaluated for HT had abnormal liver findings by CT, with cirrhosis in 41%. One-year mortality and serial liver evaluation were similar between groups after HT. Liver cirrhosis identified by CT imaging may not be an absolute contraindication to HT alone in this population.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri. Electronic address: simpson_k@kids.wustl.edu.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Simpson
authors.affiliation: School of Medicine, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Amir
authors.initials: A
authors.lastname: Esmaeeli
authors.affiliation: Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Geetika
authors.initials: G
authors.lastname: Khanna
authors.affiliation: Department of Pathology, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Francis
authors.initials: F
authors.lastname: White
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Yumirle
authors.initials: Y
authors.lastname: Turnmelle
authors.affiliation: Department of Surgery, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Pirooz
authors.initials: P
authors.lastname: Eghtesady
authors.affiliation: Department of Surgery, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Umar
authors.initials: U
authors.lastname: Boston
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002648:Child; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000601:surgery*; D016027:Heart Transplantation / Q000401:mortality*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D008103:Liver Cirrhosis / Q000150:complications*; D008111:Liver Function Tests; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed
keywords: Fontan procedure; heart transplant; liver cirrhosis; liver transplant; pediatric cardiology