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