With the prolonged survival of children with serious congenital heart disease (CHD), we have encountered new problems and new challenges within the focus on long-term morbidity and quality of life. Recently, hepatic complications have been increasingly reported in patients who underwent Fontan surgery for serious CHD with single ventricles. We recognized many hepatic complications ranged from liver fibrosis, cirrhosis, and even liver cellular carcinoma (HCC). We had a chance to experience children with liver complications after Fontan operations in Shizuoka Children's Hospital. Initially, it was difficult to identify the liver complications, because in the majority of these children, routine liver function tests such as transaminase (AST, ALT) levels were usually within the reference range. However, these serum markers for developing liver fibrosis, hyaluronic acid, type IV collagen, pro-collagen-III-peptide, were usually increased in children with liver complications. It was also difficult to differentiate liver cirrhosis from serious liver fibrosis. Both US and CT imaging findings on the liver are useful in judging developing cirrhosis and various types of liver nodules such as focal nodular hyperplasia, adenoma, HCC, etc.In this article, we reviewed the important reports on liver complications after Fontan operations. And we especially discussed the mechanism of liver complications, diagnosis, and tentative management for liver complications after Fontan operations.
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