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Pediatric Cardiology

, Volume 34, Issue 1, pp 135–142 | Cite as

Portal and Sinusoidal Fibrosis are Common on Liver Biopsy After Fontan Surgery

  • Matthew C. SchwartzEmail author
  • Lisa M. Sullivan
  • Andrew C. Glatz
  • Elizabeth Rand
  • Pierre Russo
  • David J. Goldberg
  • Jonathan J. Rome
  • Meryl S. Cohen
Original Article

Abstract

Hepatic fibrosis is an important complication after Fontan surgery in patients with single-ventricle congenital heart disease. Few reports of hepatic histology in these patients exist, and sinusoidal fibrosis has been described. We aimed to characterize fibrosis at liver biopsy procedure in patients with previous Fontan surgery and to identify patient variables associated with the degree of fibrosis. All patients who had previous Fontan surgery and who subsequently underwent liver biopsy at our institution between January 1990 and July 2010 were identified. For each biopsy specimen, portal and sinusoidal fibrosis were graded and medical records reviewed. Biopsy specimens from 13 patients were examined; the median time from Fontan surgery to liver biopsy procedure was 16.9 years (range 6.9–25). At the most recent biopsy procedure, 12 patients (92 %) had evidence of portal fibrosis, including 1 patient with portal-based cirrhosis. Thirteen patients (100 %) had at least some degree of sinusoidal fibrosis, including 1 patient with centrilobular-based cirrhosis. Lower platelet count was associated with greater degree of portal fibrosis by ordinal regression (odds ratio 0.84, P = 0.04), and patients with no or mild portal fibrosis had significantly higher platelet counts compared with those with moderate or severe portal disease (278 ± 78 K vs. 160 ± 46 K, P = 0.005). Four patients underwent serial biopsy procedures; portal fibrosis was progressed in 3 patients, and sinusoidal fibrosis was progressed in 3 patients. After Fontan surgery, portal and sinusoidal fibrosis are common at liver biopsy and can progress over time. Lower platelet count may represent a marker of portal-based disease in these patients.

Keywords

Cirrhosis Fontan Liver fibrosis Platelets 

References

  1. 1.
    Adinolfi LE, Giordano MG, Andreana A et al (2001) Hepatic fibrosis plays a central role in the pathogenesis of thrombocytopenia in patients with chronic viral hepatitis. Br J Haematol 113:590–595PubMedCrossRefGoogle Scholar
  2. 2.
    Ghaferi AA, Hutchins GM (2005) Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 129:1348–1352PubMedCrossRefGoogle Scholar
  3. 3.
    Kendall TJ, Stedman B, Hacking N et al (2008) Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol 61:504–508PubMedCrossRefGoogle Scholar
  4. 4.
    Kiesewetter CH, Sheron N, Vettukattill JJ et al (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584PubMedCrossRefGoogle Scholar
  5. 5.
    Lackner C, Struber G, Liegl B et al (2005) Comparison and validation of simple noninvasive tests for prediction of fibrosis in chronic hepatitis C. Hepatology 41:1376–1382PubMedCrossRefGoogle Scholar
  6. 6.
    Myers RP, Cerini R, Sayegh R et al (2003) Cardiac hepatopathy: clinical, hemodynamic, and histologic characteristics and correlations. Hepatology 37:393–400PubMedCrossRefGoogle Scholar
  7. 7.
    Schwartz MC, Sullivan L, Cohen MS et al (2012) Hepatic pathology may develop before the Fontan surgery in children with functional single ventricle: An autopsy study. J Thorac Cardiovasc Surg 143(4):904–909Google Scholar
  8. 8.
    Sherlock S (1951) The liver in heart failure; relation of anatomical, functional, and circulatory changes. Br Heart J 13:273–293PubMedCrossRefGoogle Scholar
  9. 9.
    Wu FM, Ukomadu C, Odze RD, Valente AM, Mayer JE Jr., Earing MG (2011) Liver disease in the patient with Fontan circulation. Congenit Heart Dis 6:190–201PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Matthew C. Schwartz
    • 1
    Email author
  • Lisa M. Sullivan
    • 1
  • Andrew C. Glatz
    • 1
  • Elizabeth Rand
    • 1
  • Pierre Russo
    • 1
  • David J. Goldberg
    • 1
  • Jonathan J. Rome
    • 1
  • Meryl S. Cohen
    • 1
  1. 1.Division of CardiologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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