Atrial Arrhythmias in Adults with Fontan Palliation

Abstract

Single ventricle physiology is a rare form of congenital heart disease and was, historically, a uniformly lethal condition. However, the atriopulmonary Fontan operation, and its successive iterations, the lateral tunnel and extracardiac conduit Fontan, became the fundamental approach to treating single ventricle heart disease. Over time, dysrhythmias are some of the most common complications with Fontan physiology, compounding morbidity and mortality. Atrial arrhythmias are prevalent in the Fontan population and occur in about 15–60% of patients with Fontan palliation, increasingly with age. Diagnosing atrial arrhythmias in patients with Fontan palliation may be challenging because of low voltage amplitudes arising from myopathic atrial tissue making it difficult to clearly assess atrial depolarization on surface electrocardiograms (ECG), vague symptoms not suggestive of tachyarrhythmia, or atrial arrhythmia with ventricular rates below 100 beats per minute. Intra-atrial reentrant tachycardia (IART) is the most common type of supraventricular tachycardia in adults with Fontan palliation. Acute management of atrial arrhythmias in patients with Fontan palliation involves prompt assessment of a patient’s hemodynamic stability, anticoagulation and thrombosis risk, systemic ventricular function, and risk of sedation or anesthesia if needed. Long-term management of atrial arrhythmias is often multifactorial and may include long-term anti-arrhythmic therapy, permanent pacing, and ablation. The best approach for the management of atrial arrhythmias in adults with Fontan palliation is patient-specific and involves collaboration between congenital electrophysiologists, adult congenital cardiologists, and the patient.