Preventing sudden death after repair of tetralogy of Fallot: complex therapy for complex patients.

Sudden arrhythmic death in patients with repaired tetralogy of Fallot or its variants has a variety of causes. Consequently, it can serve as a paradigm for management of potentially malignant arrhythmias in all pediatric patients, particularly with regard to the use of nonpharmacologic therapy for management. Five cases are presented as touchpoints for discussion and demonstrate a number of important issues concerning the assessment and reduction of sudden cardiac death risk in these patients. First, there are no clinical parameters that can be used to accurately assess risk. Second, pharmacologic agents alone rarely are adequate therapy. Third, catheter ablation and antitachycardia devices continue to play an ever increasing role in management of these patients, and, finally, additional data are necessary to establish clear management guidelines in patients with congenital heart disease at risk for arrhythmic death.