Pediatric Cardiology and Cardiac Surgery
Vol.26 No.3 2010 (200-205)

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Martin J. LaPage and J. Philip Saul

Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, South Carolina, USA


Arrhythmias are a common cause of morbidity in patients with palliated single ventricle pathology. The relative risk of arrhythmia in Fontan patients strongly favors the development of intra-atrial reentry tachycardia (IART) or sinus node dysfunction (SND). More than 50% of Fontan patients will develop atrial tachycardias over long term follow-up. This review focuses on the characteristics and treatment of IART and SND. In general, IART is an arrhythmia specific to post-atriotomy atria; and is frequently a non-cavotricuspid isthmus dependent circuit, especially in the Fontan patient. Drug therapy alone is often inadequate for prevention of IART. Atrial antitachycardia pacing has not been reliable in patients with congenital heart disease. Radiofrequency (RF) ablation has shown increasing success rates as new technologies are implemented. Knowledge of both the anatomy and physiology are critical for successful ablation. Surgery is appropriate with significant hemodynamic abnormalities or failed arrhythmia management. Most importantly, recurrences are common and optimal therapy should include a combination of the discussed techniques.