Patients undergoing the Fontan operation must be selected carefully. The best results are obtained in those who fulfill the criteria of Choussat et al. Other treatment options are now available. Patients known to be at high risk for the Fontan procedure should be considered for cavopulmonary anastomoses or cardiac transplantation. It is unknown whether performing the Fontan operation at an earlier age will help prevent the long-term problems with ventricular dysfunction. Long-term follow-up of all patients following the Fontan procedure is mandatory with noninvasive assessment of ventricular function and the anastomotic site. Arrhythmias should be managed aggressively with prompt restoration of sinus rhythm, when possible, ideally with antiarrhythmic agents with little or no negative inotropic action. Periodic assessment of serum proteins should be performed. Although the operative risk of the Fontan operation has continued to improve over the last few years, and in one series has been as low as 8%, long-term complications continue and reinforce the concept of the Fontan operation being a palliative rather than a curative procedure.