Early development of hepatic fibrosis after Fontan procedure: A non-invasive study of a subclinical liver disease.

Background: To evaluate subclinical liver fibrosis after Fontan procedure using a comprehensive method that reflects the overall liver status rather than the focal biopsy lesion.

Method: This cross-sectional study included 66 patients without symptoms of liver disease who had at least 5 years of elapsed time since the Fontan procedure (time since Fontan procedure 17.5 ± 7.3 years). Serum enhanced liver fibrosis (ELF) scores and 2D shear wave elastography (SWE), which are expected to detect liver fibrosis earlier than liver enzyme tests and ultrasonography, were evaluated along with general examinations.

Results: Liver cirrhosis diagnosed by ultrasonography showed a marked increase 16 years after surgery (elapsed time: 6-15 years since Fontan, 43.8%; 16-25 years, 70.8%; 26-35 years, 90.0%). The age-adjusted ELF score and liver stiffness according to SWE were elevated during the early postoperative period (6-15 years since Fontan). In adulthood (elapsed time > 16 years), the adjusted ELF score was correlated with liver stiffness (r = 0.514, p = .009) and liver enzyme levels. There was no correlation between liver fibrosis and the Fontan palliation type. AST to platelet count ratio and FIB-4, which are non-invasive fibrosis markers, presumed absence of fibrosis in approximately 90% of patients.

Conclusions: In the evaluation of early liver fibrosis after the Fontan procedure, abnormal findings of 2D SWE and ELF scores were observed before abnormal ultrasound and liver enzyme results. Therefore, these indicators may be helpful for the diagnosis of early liver fibrosis, and further longitudinal study might be needed.