Liver Involvement after a Fontan Procedure for Congenital Heart Disease

Abstract

Patients with congenital heart disease are living longer into adulthood. As these patients age, we see extracardiac complications of Fontan circulation. Liver dysfunction is almost universal in patients who have had a Fontan surgery. This paper seeks to describe the current understanding of the pathophysiology, diagnosis, and management of Fontan-associated liver dysfunction (FALD). In this era of noninvasive markers for liver diseases, unfortunately, these markers have been found to be less sensitive in patients with FALD. There have been small, mostly single center, reports about the need for combined heart and liver transplant in these patients. Clinical expertise is needed to determine optimal treatment for patients with FALD and it requires a multidisciplinary team of liver and congenital heart disease experts to manage this population.