title: Fontan-associated liver disease: Implications for heart transplantation.
abstract: Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury. Pathology studies have generally shown that FALD worsens as time post-Fontan increases, but the prevalence of FALD is not well defined because the majority of Fontan patients, even those with significant hepatic fibrosis, appear to be asymptomatic and biochemical or functional hepatic abnormalities are usually subtle or absent. Alternate non-invasive investigations, derived from the study of other chronic liver diseases, have been tested in small series of pediatric and adult Fontan patients, but they have been confounded by congestion and do not correlate well with liver biopsy findings. Liver disease can complicate Fontan circulatory failure and may even be significant enough to be considered a contraindication to heart transplantation or require combined heart-liver transplantation. The search for the optimal management strategy continues in the setting of increasing numbers of Fontan patients surviving to adulthood and being referred for heart transplantation. Thus, in this review we attempt to define the scope and significance of FALD and address transplant-related assessment and management of this challenging disorder.
authors.affiliation: Department of Paediatrics and Alberta Children's Hospital Research Institute, University of Calgary, Calgary, Alberta, Canada; Department of Cardiac Sciences and the Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, Alberta, Canada. Electronic address: steven.greenway@albertahealthservices.ca.
authors.forename: Steven C
authors.initials: SC
authors.lastname: Greenway
authors.affiliation: Department of Paediatric Cardiology and Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: David S
authors.initials: DS
authors.lastname: Crossland
authors.affiliation: The Liver Unit, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: Mark
authors.initials: M
authors.lastname: Hudson
authors.affiliation: Department of Paediatrics and Alberta Children's Hospital Research Institute, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Steven R
authors.initials: SR
authors.lastname: Martin
authors.affiliation: Liver Unit, Division of Gastroenterology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Myers
authors.affiliation: Department of Cardiac Sciences and the Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Tim
authors.initials: T
authors.lastname: Prieur
authors.affiliation: Department of Paediatric Cardiology and Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: Asif
authors.initials: A
authors.lastname: Hasan
authors.affiliation: Department of Paediatric Cardiology and Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Kirk
mesh_terms: D018729:Fontan Procedure / Q000009:adverse effects*; D006333:Heart Failure / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D008107:Liver Diseases / Q000209:etiology*; D011183:Postoperative Complications / Q000209:etiology*
keywords: Fontan; combined heart-liver transplantation; heart transplantation; liver disease; liver fibrosis