Long-term results and consequences of single ventricle palliation

Severe hypoplasia of the right ventricle leads to the need to offer an alternative strategy beyond two-ventricle repair for the infant with pulmonary atresia and intact ventricular septum. Although the relative short-term results for the Fontan operation have improved considerably over the past two decades, long-term results are worrisome. The Fontan circulation with resultant elevation in systemic venous pressure and low cardiac output leads to end-organ dysfunction and a series of new diseases. In this review, we discuss the physiological consequences of the Fontan circulation, its effect on the liver and gastrointestinal tract, and propose a new clinical care model for the management of these unique patients.