Outcomes after the Fontan procedure

Over the past two decades, advances in congenital heart surgery, pediatric cardiology, and intensive care medicine have dramatically increased the survival of infants with critical congenital heart disease. The group of patients that has perhaps benefited the most from this progress has been the single-ventricle population. Staged palliation culminating in the Fontan procedure has resulted in a decreasing mortality rate and an increase in the number of single-ventricle survivors. Over the past 18 months, many studies have focused on outcomes after the Fontan procedure. These reports demonstrate progressive improvement in early postoperative survival and intermediate and late postoperative outcomes due to surgical innovations, such as the lateral tunnel and extracardiac Fontan modifications, and fenestration, as well as technological improvements, such as modified ultrafiltration. Despite these improvements, significant morbidity remains after the Fontan completion, including myocardial systolic and diastolic dysfunction, systemic arterial and venous hemodynamic abnormalities, diminished exercise capacity, arrhythmias, protein-losing enteropathy, somatic growth retardation, neo-aortic valve root dilation and insufficiency, thromboembolic complications, and below-average cognitive development.