Liver Diseases in the Fontan Circulation

Abstract

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. Recentlyhepatic 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 fibrosiscirrhosisand even liver cellular carcinoma HCC. We had a chance to experience children with liver complications after Fontan operations in Shizuoka Childrens Hospital. Initially it was difficult to identify the liver complicationsbecause in the majority of these childrenroutine liver function tests such as transaminase ASTALT levels were usually within the reference range. Howeverthese serum markers for developing liver fibrosis hyaluronic acidtype IV collagenpro-collagen-III-peptidewere 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 articlewe 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.