Fontan-related hepatocellular carcinoma in a 15-year-old patient with hypoplastic left heart syndrome: A case report

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  • Fontan術後に肝細胞癌を発症した左心低形成症候群の15歳例

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Hepatocellular carcinoma (HCC) is known to arise from Fontan-associated liver diseases (FALD) after Fontan surgery. A 15-year-old male patient with hypoplastic left heart syndrome was diagnosed with HCC. He underwent fenestrated extracardiac total cavopulmonary connection at age 2 years and developed protein-losing enteropathy at age 3 years that temporarily remitted after stenting for left pulmonary artery stenosis. However, he developed plastic bronchitis at age 6 years and underwent surgical left pulmonary artery enlargement and re-fenestration for an extracardiac conduit at age 7 years. At age 15 years, liver ultrasonography revealed a heterogeneous mass of 21 mm in the S5/8 region, which was diagnosed as a well-differentiated HCC on liver biopsy. In cases of Fontan failure, it is especially important to perform regular liver ultrasound examinations to diagnose HCC at an earlier stage.

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