Exercising with a Single Ventricle: Limitations and Therapies

Treatment for Hypoplastic Left Heart Syndrome (HLHS) and other single ventricle conditions requires a series of surgical interventions for long-term survival, typically culminating in the Fontan procedure. The result is an abnormal circulatory physiology with an absence of a sub-pulmonary ventricle. Exercise capacity in the Fontan circulation is often limited and is due to multiple factors, both central and peripheral. Multiple interventions, both pharmacologic and nonpharmacologic, have been studied to attempt to overcome these inherent limitations. This review will focus on the physiology of the exercising Fontan patient and on the interventions aimed at the enhancement of exercise capacity studied thus far.