Arrhythmia management of patients with Fontan physiology

Abstract

Arrhythmia has emerged as a principal long-term complication requiring management in patients who have undergone the Fontan procedure. Surgical and hemodynamic injury to the right atrium result in a notably high prevalence of sinus node dysfunction and reentrant atrial tachycardias. In addition to causing clinical symptoms, these rhythm disorders are associated with elevated risks of thrombosis and death. Management of these arrhythmias is challenging, and has prompted innovation in catheter ablation, cardiac surgical techniques and implantable device therapy. Additional progress in preventing and treating arrhythmia in Fontan patients will require a more complete understanding of the pathogenesis of its myocardial substrate.