Long QT syndrome with AV Wenckebaching & bundle branch block in a neonate

Abstract

We present a case of 21-day-old neonate brought with history of 3 episodes of syncope. Evaluation revealed congenital long QT syndrome associated with long cycle atypical AV Wenkebaching with a long short cycle sequence related left bundle branch aberrancy. Syncope was attributed to multiple episodes of Torsades de Pointes, necessitating emergency epicardial pacemaker implantation. In addition, child was started on oral propranolol therapy. On 2 months follow up, child was stable with no ventricular high rate episodes during pacemaker interrogation.

Keywords: Ashman phenomenon; Neonatal pacemaker; Neonatal syncope; Torsades de pointes.