S2701 Oncologic Sequelae Secondary to Fontan Procedure

Abstract

INTRODUCTION: Fontan associated liver disease (FALD) is a late complication for complex congenital heart disease that increases right-sided venous pressure leading to liver cirrhosis with increased risk of hepatocellular carcinoma (HCC). The Fontan procedure was developed as a treatment for tricuspid atresia leading to increased systemic venous pressure. The liver is exposed to an increased cardiac output and subsequently impaired venous drainage and sinusoidal dilation around the central veins. The latter insult continued over time leads to fibrosis and cirrhosis of the liver. It is well known that inflammation is key in the development of hepatocellular carcinoma and begets the question whether these patients should have HCC screening. CASE DESCRIPTION/METHODS: A 42-year-old male with a past medical history of hypoplastic left heart repaired surgically by Fontan technique at age two, atrial fibrillation and pulmonary embolism on warfarin, presented with a 2 month history of cough, jaundice and intermittent abdominal pain. Magnetic resonance imaging revealed a diffusely infiltrative HCC in the right lobe, with the largest segment of tumor measuring 11.6 × 6.5 cm with tumor invasion into the portal vein and a-Fetoprotein level of 12512 ng/mL. The patient was diagnosed with stage IV HCC. This case was presented at the institution’s multidisciplinary tumor board meeting, and given the size and infiltrative nature of the tumor, the HCC was deemed unresectable and outside transplant criteria. Ultimately, the patient underwent Yttrium-90 radioembolization and systemic treatment with Levatinib. Unfortunately, the tumor progressed despite treatment and the patient passed away two months after his initial diagnosis. DISCUSSION: The development of FALD is well described, however the characteristics and risk factors leading to HCC require further studies. Our patient presented with a large, infiltrating, unresectable HCC outside of liver transplant criteria. Given his history of a Fontan procedure, monitoring for the development of fibrosis and cirrhosis, the patient’s HCC may have been discovered earlier with more treatment options. Due to scarcity of data of risk factors but potential devastating outcomes, this case highlights the importance of recognizing FALD and the risk of HCC and raises the question whether all patients with Fontan procedure should have serial screening for HCC.Table 1.: Laboratory values at presentationFigure 1.: MR Abdomen with infiltrative HCC lesion.