Five decades of Fontan palliation: What have we learned? What should we expect?

Abstract

The Fontan procedure is the final palliative surgery in a series of staged surgeries to reroute the systemic venous blood flow directly to the lungs, with the ventricle(s) pumping oxygenated blood to the body. Advances in medical and surgical techniques have improved patients' overall survival after the Fontan procedure. However, Fontan-associated chronic comorbidities are common. In addition to chronic cardiac dysfunction and arrhythmias, complications involving other organs such as the liver, lungs, intestine, lymphatic system, brain, and blood frequently occur. This narrative review focuses on the immediate and late consequences in children, pregnant women, and other adults with Fontan circulation. In addition, we describe the technical advancements that might change the way single-ventricle patients are managed in future.

Keywords: Fontan; Fontan-associated liver disease; Single-ventricle physiology; adult congenital heart disease; arrhythmias; cardiac transplantation; congenital heart disease; cyanosis; heart failure; neurodevelopmental outcomes.