Current Treatment Options of Fontan Arrhythmias: Etiology, Incidence, and Diagnosis

Purpose of the review: Patients who undergo single ventricle palliation with a Fontan are at a high risk for developing arrhythmias. This review will address our understanding of pathophysiology, incidence, presentation, and type of arrhythmia, focusing on recent developments. Recent findings: Patients with the initially described atriopulmonary connection type of Fontan have a high incidence of arrhythmias, with severe consequences. Arrhythmias are less of a problem with the two currently used surgical techniques, the lateral tunnel and the extracardiac conduit Fontan, with the extracardiac Fontan being potentially less arrhythmogenic in the long term. Summary: There is no role for the atriopulmonary Fontan any longer. As the lateral tunnel and extracardiac conduit patients age, there will be a greater understanding of the unique complications specific to their unique anatomy.