Although the Fontan operation may provide a durable circulation for some patients with functionally univentricular hearts for several decades, circulatory failure becomes more common over time. Medical and/or surgical interventions can improve the circulation for some period of time; however, many patients will experience end-stage circulatory failure. Heart transplantation may be considered in these patients. This review will cover the indications and evaluation for heart transplantation, management of patients while waiting for heart transplantation and the current outcomes of transplantation in this population.