The fate of the Fontan circulation.

Since the first operation performed in 1968, the worldwide population of patients living with Fontan circulation is dramatically growing, with 40% reaching adulthood in the current era. Despite this remarkable improvement in survival, some patients struggle with Fontan-related complications including heart failure, arrhythmias, end-organ dysfunction, and premature death. This review aims at describing common complications of the Fontan circulation, proposing a clinical and mortality risk score to better stratify this complex population as well as exploring the current state of art in catheter interventions and transplant.