A liver mass post-Fontan operation

A 19-year-old female was referred for urgent investigation of jaundice. She had a history of complex congenital heart disease with situs invertus, left atrial isomerism and juxtaposed atrial appendages, azygous continuation of the inferior vena cava and malposition of the great arteries with tricuspid atresia. In 1990, she had a successful atrio-pulmonary connection (‘Modified Fontan’ operation). She was followed up closely thereafter by the Cardiology team. Eight years later, she had an oesophageal variceal haemorrhage requiring variceal band ligation. At the time, it was felt that her portal hypertension was related to her cardiac history with other causes of liver disease excluded. She remained on endoscopic surveillance with 2-yearly upper gastrointestinal endoscopies thereafter. After 14 years, she was admitted to hospital with jaundice and exertional dyspnoea. Cardiac investigations were unchanged from before; however, a Magnetic Resonance Angiogram of pulmonary arteries coincidentally revealed a 20 mm × 14 mm × 15 mm thrombus of the left hepatic vein. Computerized tomography (CT) scan thereafter unfortunately showed an advanced hepatocellular carcinoma (HCC) (Figure 1) invading the portal vein. She was treated with Sorafenib, but deteriorated and died 3 months after diagnosis.