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Updated: Apr 15, 2026
Heterotopic Auxiliary Rat Liver Transplantation With Flow-regulated Portal Vein Arterialization in Acute Hepatic Failure
Published on: September 13, 2014
Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes
Fred M Wu1, Maureen M Jonas2, Alexander R Opotowsky1
1Department of Cardiology, Boston Children's Hospital, Harvard Medical School; Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School.
Insights
Individuals with Fontan physiology have a high prevalence of hepatic fibrosis, with nearly all showing centrilobular fibrosis and over 80% exhibiting portal fibrosis. Liver disease signs did not predict these findings, and advanced disease risk factors were few.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- The Fontan operation is a palliative surgical procedure for single ventricle congenital heart disease.
- Congestive hepatopathy and cirrhosis are known complications, but prevalence and predictors of liver disease are unclear.
Purpose of the Study:
- To investigate the prevalence and characteristics of liver disease in individuals with Fontan circulation.
- To identify predictors of liver histopathology and their impact on survival.
Main Methods:
- Retrospective study of liver histopathology from 68 Fontan subjects via biopsy or autopsy.
- Grading of liver specimens and chart review for clinical data.
- Analysis of histopathology findings in relation to clinical factors and survival.
Main Results:
- All specimens showed centrilobular fibrosis (41.2% Grade 4); 82.3% had portal fibrosis (14.7% cirrhosis).
- Megamitochondria observed in 58.8% of specimens.
- Higher fibrosis grades in dominant ventricle types; portal fibrosis correlated with alkaline phosphatase and biopsy mode. No correlation with survival.
Conclusions:
- Fontan physiology is associated with a high prevalence of hepatic fibrosis.
- Clinical signs of liver disease did not correlate with histopathology.
- Few risk factors for advanced liver disease were identified, and histopathology did not predict survival.
Background:
The Fontan operation redirects venous blood flow directly to the pulmonary circulation in subjects with single ventricle anatomy. Congestive hepatopathy and cirrhosis have been described in subjects with Fontan circulation, but the prevalence of and predictors for liver disease remain unknown.
Methods:
We performed a retrospective study of liver histopathology in Fontan subjects who had liver biopsy or autopsy. All specimens were graded using a pre-determined protocol. Additional data were collected through chart review. Among 68 subjects, specimens were obtained at a median age of 23.2 years (range 5.0 to 52.7 years). Median time since Fontan was 18.1 years (range 1.2 to 32.7 years).
Results:
Centrilobular fibrosis was seen in every specimen, with 41.2% showing Grade 4 centrilobular fibrosis. Portal fibrosis was seen in 82.3% of specimens, with 14.7% showing cirrhosis. Megamitochondria were seen in 58.8% of specimens. Centrilobular fibrosis grade was greater in those with a dominant left or right ventricle than in those with a combined right and left systemic ventricle (p = 0.008). Portal fibrosis grade correlated with alkaline phosphatase (p = 0.04) and mode of biopsy (p = 0.02). Neither centrilobular fibrosis nor portal fibrosis grade was predictive of transplant-free survival or overall survival.
Conclusions:
Individuals with Fontan physiology have a high prevalence of hepatic fibrosis. Signs and symptoms of liver disease did not predict histopathologic findings. Few risk factors for advanced disease were identified. Histopathology findings did not predict transplant-free survival. The role of liver biopsy in this population remains uncertain.
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