Electrophysiology issues in adult congenital heart disease.

Introduction Improved surgical outcomes in children have led to a growing population of adults with congenital heart disease. Rhythm disturbances in the adult congenital heart disease (ACHD) patient can be intrinsic to the anomaly or acquired after palliation. Tachyarrhythmias, either supraventricular or ventricular, and bradyarrhythmias, either sinus node dysfunction or atrioventricular block, may occur frequently. Technological advances in intervention and surgical approaches have led to prophylactic and therapeutic reduction in arrhythmias. In order of escalation, this article addresses medical management, catheter ablation, device therapy for antitachycardia pacing and defibrillation, and surgical intervention. There are now an estimated one million-plus ACHD patients living in the United States. An estimated 45% of those have simple defects (e.g., atrial septal defects), 40% have moderately complex defects (e.g., tetralogy of Fallot, or ToF), and 15% have severely complex defects (e.g., single ventri...