A rare indication of permanent pacemaker implantation in children: congenital long QT syndrome

Cardiol Young. 2020 Sep 30;1-2. doi: 10.1017/S1047951120003108. Online ahead of print.

Abstract

Congenital Long QT Syndrome (LQTS) is a dangerous arrhythmic disorder that can be diagnosed in children with bradycardia. It is characterised by a prolonged QT interval and torsades de pointes that may cause sudden death. Long QT syndrome is an ion channelopathy with complex molecular and physiological infrastructure. Unlike the acquired type, congenital LQTS has a genetic inheritance and it may be diagnosed by syncope, stress in activity, cardiac dysfunction, sudden death or sometimes incidentally. Permanent pacemaker implantation is required for LQTS with resistant bradycardia even in children to resolve symptoms and avoid sudden death.

Keywords: Long QT syndrome; permanent pacemaker; sudden death.