Liver Disease After the Fontan Procedure

Abstract

Fifty years ago, only a quarter of infants born with congenital heart disease (CHD) survived beyond the first year of life. It has been estimated that 80% to 85% of patients can expect to reach adulthood. Today, there are close to 1 million adults living with CHD in the United States alone. Since its introduction, the Fontan operation has become established as the dominant surgical repair in patients with univentricular heart physiology. Long-term morbidity associated with this procedure is being more commonly appreciated and liver complications have become more frequently apparent. Hepatologist awareness and familiarity in managing this population alongside the CHD specialist is important for the care of these patients. This dual perspective can provide comprehensive integrated care.