title: Predictive value of biomarkers of hepatic fibrosis in adult Fontan patients.
abstract: BACKGROUND
  Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis.
  
  
  METHODS
  Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study. Biopsy specimens were scored for degree of sinusoidal and portal fibrosis on a 3- and 5-point scale, respectively. Histologic findings were correlated with FibroSure and HA results.
  
  
  RESULTS
  The study included 27 subjects. Median age was 26.8 years (range, 17.4-59.8 years), and the median time since the Fontan surgery was 20.4 years (range, 12.0-31.3 years). FibroSure scores were elevated (>0.21) in 21 of 23 subjects (91%), and the scores for 3 (13%) suggested cirrhosis (>0.74). HA suggested cirrhosis (>46 ng/mL) in 3 of 17 subjects (18%). One subject died during the collection period. Eleven of 26 subjects (42%) had 4/5 or 5/5 portal fibrosis, consistent with cirrhosis; 17 (63%) had 3/3 sinusoidal fibrosis involving >66% of sinusoids. The FibroSure score and HA levels did not correlate with the degree of hepatic fibrosis and did not predict cirrhosis.
  
  
  CONCLUSIONS
  Abnormal biomarkers of hepatic fibrosis and specimen-proven hepatic fibrosis are common in adults with Fontan circulation. However, FibroSure and HA do not accurately predict the degree of histologic hepatic fibrosis. Further studies are needed to guide strategies for surveillance of liver disease in this population.
authors: Department of Cardiology, Boston Children's Hospital, Harvard Medical School; Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address: fred.wu@cardio.chboston.org.
authors: Fred M
authors: FM
authors: Wu
authors: Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin.
authors: Michael G
authors: MG
authors: Earing
authors: Ahmanson/University of California Los Angeles Adult Congenital Heart Disease Center, Los Angeles, California.
authors: Jamil A
authors: JA
authors: Aboulhosn
authors: Department of Pathology.
authors: Melanie E
authors: ME
authors: Johncilla
authors: Department of Cardiology, Boston Children's Hospital, Harvard Medical School; Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors: Michael N
authors: MN
authors: Singh
authors: Department of Pathology.
authors: Robert D
authors: RD
authors: Odze
authors: Division of Gastroenterology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors: Chinweike
authors: C
authors: Ukomadu
authors: Department of Cardiology, Boston Children's Hospital, Harvard Medical School.
authors: Kimberlee
authors: K
authors: Gauvreau
authors: Department of Cardiology, Boston Children's Hospital, Harvard Medical School; Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors: Michael J
authors: MJ
authors: Landzberg
authors: Department of Cardiology, Boston Children's Hospital, Harvard Medical School; Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors: Anne Marie
authors: AM
authors: Valente
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001707:Biopsy, Needle; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D006820:Hyaluronic Acid / Q000097:blood*; D007150:Immunohistochemistry; D008103:Liver Cirrhosis / Q000097:blood / Q000209:etiology* / Q000473:pathology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D018570:Risk Assessment; D055815:Young Adult
chemical_list: D015415:Biomarkers; D006820:Hyaluronic Acid
keywords: FibroSure; Fontan; cirrhosis; congenital heart disease; congestive hepatopathy; hyaluronic acid