Abstract 12566: Liver Health in Adults With Fontan Circulation: A Multicenter Cross-Sectional Study
Background: Liver disease is increasingly recognized in patients with Fontan circulation and may contribute to morbidity and mortality. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence of and predictors for liver disease in a multicenter cohort of adults with Fontan physiology.
Methods: Subjects were recruited from 6 adult congenital heart centers. Demographics, clinical history and laboratory, imaging and histopathology data, including tests of liver function, hepatitis serologies and biomarkers for hepatic fibrosis, were obtained where clinically appropriate. Each imaging study was reviewed by two radiologists and each biopsy by two pathologists.
Results: Among 238 subjects (53% male; median age 25.8y, range 11.8-59.4), median time since Fontan surgery was 20.3y (range 5.4-34.5). Primary cardiac defects included tricuspid atresia (34%), double inlet LV (24%) and hypoplastic left heart (7%). Imaging, histopathology and lab data are shown in Table 1. All CT and MR studies showed at least one abnormality. Liver biopsy (n=75) showed sinusoidal fibrosis, portal fibrosis and sinusoidal dilation in almost all cases. Lab data were available in 215 subjects; median number of abnormal values was 2/7 (IQR 1-2). Female sex was associated with fewer abnormal labs (p≤0.001) and lower Fibrosure score (p≤0.001). There was little correlation between degree of fibrosis, number of imaging abnormalities and number of abnormal labs (Fig. 1) but having no abnormal labs correlated with milder portal (p=0.002) or sinusoidal fibrosis (p=0.03).
Conclusion: In this multicenter, largest-to-date survey of liver disease in Fontan patients, abnormalities in imaging, histopathology or laboratory evaluation are common; findings appear to correlate poorly with each other. Further research continues so as to determine the best method for assessing liver health in adults after Fontan and which, if any, predict clinical outcomes.
- Adult congenital heart disease
- Congenital heart disease
- Fontan physiology
- Congenital heart surgery
- Pediatric cardiology
- © 2013 by American Heart Association, Inc.