title: Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients.
abstract: Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound, and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein, and gamma-glutamyl transpeptidase were significantly increased, and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of fibrosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan, but the incidence of ultrasound-detected liver abnormalities was variable. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Amyna
authors.initials: A
authors.lastname: Fidai
authors.affiliation: Division of Pediatric Cardiology, University of Sherbrooke, Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke , Sherbrooke, QC , Canada.
authors.forename: Frederic
authors.initials: F
authors.lastname: Dallaire
authors.affiliation: Libin Cardiovascular Institute of Alberta, University of Calgary , Calgary, AB , Canada.
authors.forename: Nanette
authors.initials: N
authors.lastname: Alvarez
authors.affiliation: Libin Cardiovascular Institute of Alberta, University of Calgary , Calgary, AB , Canada.
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Balon
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary, Calgary, AB, Canada; Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada.
authors.forename: Robin
authors.initials: R
authors.lastname: Clegg
authors.affiliation: Libin Cardiovascular Institute of Alberta, University of Calgary , Calgary, AB , Canada.
authors.forename: Michael
authors.initials: M
authors.lastname: Connelly
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Frank
authors.initials: F
authors.lastname: Dicke
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Deborah
authors.initials: D
authors.lastname: Fruitman
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Joyce
authors.initials: J
authors.lastname: Harder
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Kimberley
authors.initials: K
authors.lastname: Myers
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary, Calgary, AB, Canada; Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada.
authors.forename: David J
authors.initials: DJ
authors.lastname: Patton
authors.affiliation: Libin Cardiovascular Institute of Alberta, University of Calgary , Calgary, AB , Canada.
authors.forename: Tim
authors.initials: T
authors.lastname: Prieur
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Erika
authors.initials: E
authors.lastname: Vorhies
authors.affiliation: Liver Unit, Division of Gastroenterology, Cumming School of Medicine, University of Calgary , Calgary, AB , Canada.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Myers
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary , Calgary, AB , Canada.
authors.forename: Steven R
authors.initials: SR
authors.lastname: Martin
authors.affiliation: Department of Paediatrics, Cumming School of Medicine, Alberta Children's Hospital Research Institute, University of Calgary, Calgary, AB, Canada; Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada.
authors.forename: Steven C
authors.initials: SC
authors.lastname: Greenway
keywords: Fontan; Fontan-associated liver disease; congenital heart disease; fibrosis; liver