Permanent Cardiac Pacing in the Fontan Population

Following the Fontan operation, electrophysiologic abnormalities requiring pacemaker implantation are common, consisting of sinus node dysfunction, complete atrioventricular block, and electromechanical dyssynchrony. Pacemaker implantation in this population can be challenging, as transvenous access to the cardiac chambers is often limited and may increase the risk of thromboembolism. Consequently, epicardial lead placement continues to be the default approach at most centers. Furthermore, permanent cardiac pacing has been associated with poor outcomes in this population (including an increased need for cardiac transplantation and death), even though it may be, depending on the approach, of great benefit for many individuals. Fortunately, improved understanding of the differential effects of cardiac pacing and novel approaches related to implantation have been developed and have increased their application to a growing number of patients. This review highlights the indications for pacing, methods to facilitate lead implantation, and associated outcomes in Fontan patients requiring permanent cardiac pacing.