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.
Key words: Long QT syndrome, left cardiac sympathetic
denervation, permanent pacemaker, sudden cardiac death.