The Fontan Circulation: Contemporary Review of Ongoing Challenges and Management Strategies

Abstract

Since its original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology and has resulted in an increasing number of these patients surviving to adulthood. The Fontan operation is a unique approach to create a circulation in series without two distinct pumping chambers. Although the Fontan operation increased the survival rates of patients with single ventricle physiology, it carries an inevitable risk of long-term morbidities that impacts the outcomes and quality of life in these patients. In this review, we discuss the challenges resulting from the unique pathophysiology of Fontan circulation and propose management strategies.