Highlights the clinical challenges and potential failure of pacing in an infant with long QT syndrome and recurrent polymorphic VT storm.
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Pacing may fail in infants with LQTS and VT storm; leaves open optimal rescue strategies in this rare population.
4 month old female child diagnosed a case of long QT syndrome with recurrent polymorphic ventricular tachycardia (VT) storm with moderate left ventricular (LV) dysfunction with status epilepticus presented with recurrent seizures in an emergency. Patient was intubated and put on mechanical ventilation. Recurrent seizures subsided with intravenous midazolam and anticonvulsants. In view of unstable rhythm, recurrent polymorphic VT storm and unstable haemodynamic, patient required antiarrhythmic agents such as flecainide and multiple inotropic supports. There was frequent pause dependent VT hence it was decided to put on permanent pacemaker. A repeated pacing attempt failed and on table plan for cardiac sympathectomy was made. However, the parents refused for the later. The patient left back with her own rhythm and parents are prognosticated.
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Choudhury et al. (2019) studied this question.
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