Fontan Repair of Single Ventricle Physiology: Consequences of a Unique Physiology and Possible Treatment Options.

The Fontan operation is a unique approach to create a circulation in series without 2 distinct pumping chambers. Although the approach has enabled many patients to live well into adulthood, the unique physiology results in many complications, cardiac and extracardiac. This review focuses on the pathophysiology of the failing Fontan and suggests possible treatment strategies. By understanding the physiology and interpreting patient data based on this understanding, an individualized treatment plan can be designed for this unique patient population.