Introduction:
Long QT syndrome is characterized
by life threatening ventricular arrhythmias, and even sudden cardiac
arrest (SCA) as the initial clinical presentation. High risk of SCA at
young age demands focused management of the children affected with this
genetic arrhythmia syndrome. Many treatment options are available for
the management of this condition, with a goal to prevent SCA. We present
a series of patients who were managed with left cardiac sympathetic
denervation (LCSD) combined with device implantation (permanent
pacemaker implantation in two children and implantable loop recorder in
one child).
Materials and Methods :
An institutional review board waiver was obtained to perform the study.
A formal informed oral consent was obtained from the parents of children
for the purpose of this study. An ethics approval statement was not
requiring, as IRB waiver was obtained. All the data was obtained from
the inpatient and outpatient records and recent follow-up data was
collected over the phone.
Case 1:
A 3 year 7-month-old girl, 2nd in birth order had
fetal bradycardia but no distress during antenatal period. Immediate
post-natal period was uneventful with low heart rate with varying T
wave morphology. Holter study reported as prolonged QT with T wave
alternans (TWA) throughout study and Brain Stem Evoked Response
Audiometry (BERA) revealed mild bilateral hearing loss. Child had poor
weight gain with large patent ductus arteriosus (PDA) on echo. There
was no family history of syncope or SCA and prolonged QTc in parents
or elder sibling. Attempted device closure of PDA was aborted due to
ventricular tachycardia (VT) and brief period of arrest – Torsade’s
de pointes (TDP) during the procedure and rhythm reverted by DC
version. She was started on high dose propranolol and planned for
further management. As the TWA persisted (Figure.1) and she had to
undergo PDA closure or ligation, an alternate strategy was planned.
She underwent PDA ligation, left cardiac sympathetic denervation
(T2-5) with epicardial lead placement through left posterolateral
thoracotomy. Post-operatively, specific T wave pattern with reduced
QTc interval was observed (Figure.2).