The efficacy of pacemaker implantation for extracardiac total cavopulmonary connection in a pediatric patient with bradycardia-tachycardia syndrome.

A 9-year-old boy, diagnosed with double outlet right ventricle after birth, suffered sinus node dysfunction and non-sustained junctional tachycardia after an extracardiac total cavopulmonary connection (TCPC). Spontaneous atrial tachycardia appeared 3 years after an extracardiac TCPC. Sotalol was administered but the bradycardia was obvious. It was difficult to increase sotalol and atrial tachycardia was uncontrollable. Atrial tachycardia continued with symptoms; direct current (DC) cardioversion was frequently required. Five years after extracardiac TCPC, we implanted a pacemaker with atrial antitachycardia pacing (ATP) using epicardial leads. On day 2 post operation, wide QRS tachycardia appeared. Due to decreased blood pressure, DC cardioversion was immediately performed, but it recurred from atrial premature contraction. We judged this was atrial tachycardia with 1:1 atrioventricular conduction based on an intracardiac electrogram and it was terminated by burst atrial pacing from the pacemaker. After changing atrial pacing rate to 150 ppm, atrial tachycardia could be suppressed. Due to atrial pacing and increasing sotalol gradually, junctional tachycardia terminated spontaneously, and atrial tachycardia was not induced after pacemaker implantation. In conclusion, implantation of a pacemaker with ATP and intensification of antiarrhythmic drugs is an effective treatment strategy for pediatric patients with bradycardia-tachycardia syndrome after extracardiac TCPC. <Learning objective: The treatment for bradycardia-tachycardia syndrome in children after extracardiac total cavopulmonary connection (TCPC) is challenging. The appropriate antiarrhythmic drugs for atrial tachycardia cannot be administered due to bradycardia, and it is often difficult to perform radiofrequency catheter ablation on small children. Surgical pacemaker implantation, although invasive, is the most effective treatment for bradycardia-tachycardia syndrome in small children after extracardiac TCPC.>.