Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology-pathology case series.

ABSTRACT

PURPOSE: Patients who have undergone Fontan palliation of single ventricle physiology congenital heart disease are prone to developing focal liver lesions. In our experience, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy-proven liver lesions in patients post-Fontan palliation of single ventricle physiology cared for at our institution. METHODS: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for lesion pathology, background liver findings, pertinent laboratory data, and demographic data. CT and MRI images were reviewed to describe imaging findings of the reported lesions. RESULTS: Twelve patients met inclusion criteria; 58% (7/12) of which were female. Fifteen lesions were biopsied including four macroregenerative/benign hepatocellular hyperplastic nodules, two hepatocellular adenomas, two hepatocellular carcinomas, two intrahepatic cholangiocarcinoma (in the same patient), one venous malformation, and one focus of vascularized scar tissue. Two additional lesions in patients postcardiac transplant were posttransplant lymphoproliferative disorder. CONCLUSION: Patients who have undergone Fontan palliation of single ventricle physiology are prone to develop a variety of liver lesions, both benign and malignant.