title: Noninvasive assessment of liver fibrosis in adult patients following the Fontan procedure.
abstract: OBJECTIVE
  Recent data indicate that patients after the Fontan procedure are at risk for significant liver dysfunction; however, the prevalence and extent of liver disease in the Fontan population remains unknown. Furthermore, limited data exist in regard to screening for liver disease in adult Fontan patients. We sought to determine the prevalence of liver disease in adult patients following the Fontan procedure using computed tomography (CT) and serum biomarkers of liver fibrosis.
  
  
  METHODS
  Adult Fontan patients underwent screening for liver disease as part of their annual evaluation. Screening consisted of laboratory evaluation and dual-phase liver CT scan. Laboratory evaluation included analysis of liver function, viral hepatitis serologies, and FibroSURE panel (LabCorp), a test that analyzes the results of serum biomarkers to provide a quantitative surrogate marker for liver fibrosis.
  
  
  RESULTS
  Sixteen patients, mean age 30.3 (range 20-41) years, were enrolled in the study. Mean length of follow-up from time of Fontan palliation was 20.5 (range 11-33) years. No patients had serologic evidence of viral hepatitis or synthetic liver dysfunction. Twelve patients (75%) had abnormal FibroSURE scores, seven (44%) had elevated FibroSURE scores predictive of Metavir fibrosis stage F2 or greater on liver biopsy, and one (6%) had a FibroSURE score predictive of cirrhosis on biopsy. All 16 patients had abnormal radiologic liver findings identified on CT, including heterogeneous enhancement in 11 (69%), varices in six (38%), and liver nodules in five patients (31%). Length of time since Fontan surgery correlated significantly with an elevated FibroSURE score (P = .05) and having more CT scan abnormalities (P = .04).
  
  
  CONCLUSIONS
  Liver fibrosis detected by serum biomarkers and dual phase CT scan is common in adult patients following the Fontan procedure. Further studies are needed to determine the long-term clinical significance of these findings.
authors.affiliation: Departments of Pediatrics, Division of Pediatric Cardiology Radiology Internal Medicine, Division of Cardiovascular Medicine Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA. sginde@chw.org
authors.forename: Salil
authors.initials: S
authors.lastname: Ginde
authors.forename: Mark D
authors.initials: MD
authors.lastname: Hohenwalter
authors.forename: W Dennis
authors.initials: WD
authors.lastname: Foley
authors.forename: Jane
authors.initials: J
authors.lastname: Sowinski
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Bartz
authors.forename: Suneetha
authors.initials: S
authors.lastname: Venkatapuram
authors.forename: Catherine
authors.initials: C
authors.lastname: Weinberg
authors.forename: James S
authors.initials: JS
authors.lastname: Tweddell
authors.forename: Michael G
authors.initials: MG
authors.lastname: Earing
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D008103:Liver Cirrhosis / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology; D008297:Male; D010865:Pilot Projects; D011237:Predictive Value of Tests; D015995:Prevalence; D012720:Severity of Illness Index; D013997:Time Factors; D036542:Tomography, Spiral Computed; D014922:Wisconsin / Q000453:epidemiology; D055815:Young Adult
chemical_list: D015415:Biomarkers