Cardiac Implantable Electronic Devices in the Fontan Patient

As a result of remarkable progress in operative techniques and cardiology care during childhood, Fontan patients continue to age and require team-based multidisciplinary expertise to manage complications encountered in adulthood. They face particular challenges in terms of altered hemodynamic stressors, cardiac and hepatic failure, and arrhythmias. Arrhythmias in Fontan patients are highly prevalent and associated with underlying anatomy, surgical technique, and postoperative sequelae. Diagnostic tools, treatments, and device strategies for arrhythmias in Fontan patients should be adapted to the specific anatomy, type of surgical repair, and clinical status. Great strides in our understanding of arrhythmia mechanisms, options and techniques to obtain access to relevant cardiac structures, and application of both old and new technologies have contributed to improving cardiac implantable electronic device (CIED) therapies for this unique population. In this state-of-the-art review, we discuss the various arrhythmias encountered in Fontan patients, their diagnosis, and options for treatment and prevention, with a focus on CIEDs. Throughout, access challenges particular to the Fontan circulation are considered. Recently developed technologies, such as the subcutaneous implantable cardioverter defibrillator, carry the potential to be transformative but require awareness of Fontan-specific issues. Moreover, new leadless pacing technology represents a promising strategy that may soon become applicable to Fontan patients with sinus-node dysfunction. CIEDs are essential tools in managing Fontan patients, but the complex clinical scenarios that arise in this patient population are among the most challenging for the electrophysiologist treating patients with congenital heart disease. En raison des progrès remarquables accomplis en matière de techniques opératoires et de soins cardiologiques pédiatriques, les patients qui ont subi l’intervention de Fontan continuent d’avancer en âge et ont besoin d’une expertise multidisciplinaire pour gérer les complications qu’ils rencontrent à l’âge adulte. Ces patients doivent composer avec des difficultés particulières ayant à voir avec la modification des facteurs de stress hémodynamique, l’insuffisance cardiaque et hépatique, et les arythmies. Ces dernières sont très fréquentes chez les patients ayant subi l’intervention de Fontan et se trouvent associées à l’anatomie sous-jacente, à la technique chirurgicale et aux séquelles p