Abstract 15604: Histopathologic Features of the Liver in Patients After Fontan Palliation
BACKGROUND The Fontan procedure, a palliative operation for complex congenital heart disease, directs systemic venous blood into the pulmonary arteries without an intervening ventricle, resulting in elevated systemic venous pressure. Congestive hepatopathy and cirrhosis have been documented in these patients, but the prevalence of these findings remains poorly defined.
METHODS This is a retrospective study of liver histopathology in Fontan patients who underwent biopsy or autopsy in a single center. All specimens were evaluated and scored by a single hepatopathologist blinded to clinical data.
RESULTS 73 patients (21 male) with 81 specimens were identified. Preliminary data are available for 41 specimens from 39 patients. At biopsy or autopsy, median age was 20.8 years (range 5.0-46.9 years) and median time since Fontan operation was 16.0 years (range 1.5-31.9 years). 14 (35.9%) had previous ascites at the time of biopsy. Two (5.1%) had esophageal varices, 20 (51.3%) had previous tachyarrhythmia, 2 (5.1%) had previous thromboembolic events, 6 (15.4%) had protein-losing enteropathy, 2 (5.1%) had plastic bronchitis, and 23 (59.0%) had a history of congestive heart failure. Major histopathologic findings are shown in Table 1. In addition, central-central or central-portal bridging was seen in 27 cases (65.9%) and portal-portal bridging was seen in 15 cases (36.6%). Fibrosis typically presented in a patchy distribution. Megamitochondria were seen in 25 cases (61.0%). Correlations between clinical features and histology will be presented in the final analysis.
CONCLUSION Abnormal liver histology was present in all Fontan patients studied, even those who were asymptomatic. Megamitochondria are a common finding that is not previously described in this population. Patients who undergo Fontan palliation early in life are at high risk for progressive and severe chronic liver disease.
- © 2011 by American Heart Association, Inc.