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).