Commentary: Fontan circulation with lymphatic failure: Was the Holy Grail finally found?

Abstract

Fontan himself noted that “the Fontan operation creates an unusual state.it is the Fontan state itself and the transition (by surgery) from a state of congenital heart disease to the Fontan state that constitutes the risk and makes the operation palliative and not curative.” The Fontan state induces physiologic changes as a consequence of the missing subpulmonary ventricle. It was very clear for Fontan 30 years ago that “the Fontan state.imposes a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” Since then, important progress has been made in surgical and medical management, while at the same time the population of patients living with Fontan physiology also changed. Today, hypoplastic left heart syndrome is the most common underlying diagnosis, and it is known that these patients are more likely to develop Fontan failure. With the increasing number of patients who are palliated with Fontan operations worldwide, the number of patients with failing Fontan is also increasing. Pediatric and adult cardiologists, as well as congenital heart surgeons are facing a new challenge: management of the Fontan circulation with its different categories of failure, including ventricular dysfunction, Fontan circuit failure, lymphatic failure, and extracardiac organ failure. Although favorable results can be achieved after heart transplantation, the subsets of patients with preserved ventricular function have a much higher mortality.