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Identifying predictors of hepatic disease in patients after the Fontan operation: A postmortem analysis.
MedLine Citation:
PMID:  23072704     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVE: We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease. METHODS: From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis. RESULTS: The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P < .003) and portal fibrosis (P < .002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P < .03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome. CONCLUSIONS: The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome.
Authors:
Jennifer A Johnson; Frank Cetta; Rondell P Graham; Thomas C Smyrk; David J Driscoll; Sabrina D Phillips; Anitha S John
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-13
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  -     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn.
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