Therefore, consideration of transplantation early in the course of PLE may be warranted. Evaluation of additional organs is necessary, particularly the liver, as these patients are susceptible to cirrhosis as a consequence of the Fontan circulation. Although symptoms may improve, there are no published data regarding impact on survival for transplanted Fontan patients with PLE compared with those who do not undergo transplantation. 10. Although catheterization plays an important role in management of single ventricle/Fontan patients, it is often driven by symptoms. The role of routine hemodynamic assessment is less certain. 11. Fontan circulation imparts risk of thrombosis, and anticoagulation with vitamin K antagonists should be offered as preventive therapy in clin- ical situations including prior arrhythmia.S4.4.2-60 Patients may also benefit from anticoagulation if they have significant residual intracardiac right- to-left shunt or veno-veno collaterals. 12. Catheter ablation has been associated with improved clinical status despite the frequent co- existence of multiple arrhythmia substrates.S4.4.2-61 Given the progressive nature of the atrial myop- athy, successful ablation is less frequent than in acquired heart disease or other congenital heart diseases, and recurrence is common. The devel- opment of new arrhythmias over time remains problematic, but multiple ablation procedures may be justified in selected patients.S4.4.2-13,S4.4.2-14 13. Conversion to a total cavopulmonary connec- tion Fontan combined with a modified right atrial Maze procedure may be considered in patients with symptomatic refractory recurrent intra-atrial In the pres- ence of documented AF, a left atrial Cox Maze procedure may also be indicated.S4.4.2-16,S4.4.2-62 Some patients may not be appropriate surgical candidates for reasons of elevated PA or Fontan pressures, elevated ventricular end-diastolic pres- sures, or renal or hepatic dysfunction, and the de- cision to perform Fontan revision surgery is rarely straightforward. 14. 