Long-term results after the modified Fontan operation

Since the introduction of palliative surgical therapy for single ventricle defect more and more patients have reached adulthood. Thus, nowadays the long-term results of large cohorts can be analyzed. The results of the modified Fontan operation performed with modern surgical technique are characterized by low morbidity and mortality rates. Therefore in the past 40 years a new patient population with very specific anatomical and physiological principles has arisen and continues to increase in size. The long-term follow-up reveals stable hemodynamics under non-cyanotic conditions. The preconditions for optimal long-term outcome are strict preoperative selection and subject-specific postoperative supervision. The long-term results can be significantly improved if therapy is started early enough. Nevertheless the Fontan circulation is limited through the non-physio-logical flow principle and decreased function of the single ventricle. Essential for the long-term stability of the hemodynamics are the reduction of pulmonary vascular resistance and improvement of ventricular filling. The conventional medication for heart failure is not applicable in treatment of the failing Fontan circulation. Severe Fontan complications, such as protein-losing enteropathy with chronic ascites and chronic Fontan failure are rare. Nevertheless the possibility for conventional treatment in such cases is limited and heart transplantation is the ultima ratio therapy.