Predictive value of biomarkers of hepatic fibrosis in adult Fontan patients
Section snippets
Study design
A total of 241 subjects consented to participate in the Alliance for Adult Research in Congenital Cardiology Fontan liver health study from all eligible individuals who presented to 1 of 6 United States adult congenital heart disease centers for evaluation from September 2009 through April 2012. Subjects were eligible for enrollment if they were aged ≥16 years and had previously undergone Fontan surgery. Exclusion criteria included a history of cardiac transplantation, known inherited forms of
Baseline characteristics
We identified 27 patients who had a liver biopsy and FibroSure or HA testing (25 with FibroSure and 17 with HA). The liver biopsy in 23 of 27 patients was performed through a transjugular approach, and 3 were performed percutaneously. One patient died during the data collection period and underwent liver evaluation at autopsy. The median age was 26.8 years (range, 17.4–59.8 years), and the median time since initial Fontan surgery was 20.4 years (range, 12.0–31.3). The most common congenital
Discussion
This study investigated the correlation between direct and indirect biomarkers of liver fibrosis and histologic fibrosis in patients with Fontan physiology. In our cohort of patients with a high prevalence of advanced-stage hepatic fibrosis, FibroSure and HA are inaccurate predictors for such.
The histologic features of liver disease typically seen in patients with Fontan circulation are dilated sinusoids; congestion, atrophy, and necrosis of the centrilobular hepatocytes; sinusoidal fibrosis;
Disclosure statement
None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript. C.U. currently employed by Novartis.
The authors acknowledge the clinical and administrative staff of the Boston Adult Congenital Heart (BACH) and Pulmonary Hypertension Program for their support in the preparation of this manuscript.
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