A liver mass post-Fontan operation.
### Learning Point for Clinicians

This case report raises awareness of a rare devastating complication of congenital heart diseases that require correction via the “Fontan” operation. In these patients, liver cirrhosis and its complications are now being recognised including hepatocellular carcinoma. With recognition, strategies for early treatment /interventions in a complicated population can be devised.

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 …