Intrahepatic cholangiocarcinoma after the Fontan procedure

A long duration of Fontan circulation can cause fibrosis, cirrhosis of the liver and liver cancer. There have been some reports of hepatocellular carcinoma occurring after the Fontan procedure (FP), and we encountered a case of intrahepatic cholangiocarcinoma (ICC) after the FP. We herein report the case of 33-year-old man who developed ICC after receiving the FP. At five years of age, the patient underwent the original FP. At 16 years of age, he received total cavopulmonary connection conversion. Sixteen years later, he suffered right heart failure. Thereafter his symptoms showed repeated exacerbation and remission. One year later, he was admitted to the hospital due to the aggravation of congestive heart failure symptoms. He developed contrast nephropathy, and his renal function rapidly weakened. Computed tomography revealed multiple mass lesions in the liver. He ultimately died four months after his admission. An autopsy revealed the hepatic tumor to be ICC. Patients who have undergone the FP need to undergo a checkup with ultrasound on a regular basis.