Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation

Abstract

The majority of individuals who have undergone Fontan palliation are surviving into adulthood, yet complications are common. This review will focus on late complications—cardiac and extra-cardiac—which may present insidiously. Recent meta-analysis of patients after Fontan operation suggests that over 80% will survive 20 years beyond surgery. This group is at risk for structural complications, arrhythmias, vascular complications, and heart failure. Not all forms of Fontan failure are the same, and categorization into separate entities such as failure with preserved versus reduced function may help guide therapies. Pulmonary vasodilators in particular may be useful to improve hemodynamics. Novel therapies aimed at lymphatic complications are emerging and promising. Late hepatic complications including ascites and liver cancer may be seen, and scoring systems may identify patients at higher risk. Individuals who have undergone a Fontan surgery face challenges as they age which include both cardiac and non-cardiac systems.