The Failing Fontan

Fontan palliation is the final palliative pathway for children with a variety of single ventricle congenital heart disease. While the outcomes of the Fontan procedure have improved over time, the unique physiology related to the Fontan, as well as the prior palliations, places children and young adult of developing a multitude of complications. Collectively, this is referred to as failing Fontan physiology and can impact the heart as well as other organs. Unfortunately, despite this improvement in the Fontan procedure, Fontan failure is essentially universal although it is quite heterogenous in regard to the timing of onset and manifestation. In this chapter, we will discuss the various types of Fontan failure, as well as their etiology, assessment, and management.