Key result
A 5-year-old boy with long QT syndrome and macroscopic T-wave alternans continued to experience loss of consciousness despite maximal beta-blocker therapy, necessitating left thoracoscopic cervical sympathectomy.
Case Report (n=1)
This case report highlights a rare presentation of macroscopic T-wave alternans in a pediatric patient with long QT syndrome who was refractory to beta-blocker therapy and required sympathectomy.
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May support sympathectomy in refractory pediatric LQTS with T-wave alternans; leaves open need for prospective validation.
Long QT syndrome (LQTS) is a congenital disorder characterized by prolongation of QT interval in the electrocardiogram (ECG) and a propensity to develop ventricular arrhythmias, which may lead to syncope, cardiac arrest or sudden death. T-wave alternans (TWA), a phenomenon of beat-to-beat variability in the repolarization phase of the ventricles, has been closely associated with an increased risk of ventricular tachyarrhythmic events (VTE) and sudden cardiac death (SCD).
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SiddharthNarayan Gadage (2018) conducted a case report in Long QT syndrome with macroscopic T wave alternans (n=1). Propranolol followed by left thoracoscopic cervical sympathectomy was evaluated on Episodes of loss of consciousness. A 5-year-old boy with long QT syndrome and macroscopic T-wave alternans continued to experience loss of consciousness despite maximal beta-blocker therapy, necessitating left thoracoscopic cervical sympathectomy.
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