Left cardiac sympathetic denervation and device implantation in congenital long QT syndrome -- A case series and review.

Abstract

Introduction and aim of the study: Long QT syndrome is a life threatening genetic arrhythmia syndrome which is characterized by ventricular arrhythmias with few children often having their initial clinical presentation as sudden cardiac arrest. This condition poses a very high risk of sudden death demanding proper management of such children. Many treatment modalities are available in this era, with each one of them carrying advantages and disadvantages. Materials and methods: We present a series of four children suffering from Long QT syndrome, who were successfully managed with left cardiac sympathetic denervation (LCSD) combined with device implantation (permanent pacemaker implantation in two children and implantable loop recorder in one child). Results: All four children are asymptomatic since hospital discharge with no episodes of syncope or presyncope, or device-detected ventricular tachyarrhythmia till to-date. Conclusion: Left cardiac sympathetic denervation is an underutilized simple surgical procedure, for Long QT syndrome. When combined with other treatment strategies (like device implantation in our case series), outcome is far better than a single technique. Beta-blockers play a very important role both in pre-operative and immediate post-operative period and have to be continued for the rest of life.