Abstract
Adults with complex congenital heart disease that resulted in a Fontan procedure frequently experience late cardiac failure. Increasingly, liver disease is recognized as an important complication of single-ventricle anatomy and Fontan physiology; however, there is no consensus regarding liver evaluation in this population. Here, we review what is known about liver disease in this unique group and propose screening and prevention measures. We also review controversial treatment areas including assist devices and transplantation, with a review of outcomes in isolated heart and combined heart-liver transplant.