The “un-natural” history of the Fontan circulation portends a rather grim long-term outlook for patients with single ventricle congenital heart disease. Decades of experience with the Fontan circulation have shown the advantages of the total cavopulmonary connection that minimizes power loss to improve subpulmonary flow efficiency; yet, the altered hemodynamics impart a chronic attrition to the organ systems of the body leading to a range of complications and limited life expectancy for this patient cohort.
