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Noninvasive assessment of liver fibrosis in adult patients following the Fontan procedure. Congenit Heart Dis 2012 May-Jun;7(3):235-42



Pubmed ID




Scopus ID

2-s2.0-84861547650   50 Citations


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.

DESIGN:   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.

Author List

Ginde S, Hohenwalter MD, Foley WD, Sowinski J, Bartz PJ, Venkatapuram S, Weinberg C, Tweddell JS, Earing MG


Peter J. Bartz MD Professor in the Pediatrics department at Medical College of Wisconsin
Salil Ginde MD, MPH Associate Professor in the Pediatrics department at Medical College of Wisconsin
Mark D. Hohenwalter MD Vice Chair, Professor in the Radiology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Fontan Procedure
Heart Defects, Congenital
Liver Cirrhosis
Pilot Projects
Predictive Value of Tests
Severity of Illness Index
Time Factors
Tomography, Spiral Computed
Young Adult