Journal of cardiovascular electrophysiology
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J. Cardiovasc. Electrophysiol. · Jul 1997
High-rate atrial pacing as an innovative bridging therapy in a neonate with congenital long QT syndrome.
The neonatal presentation of the long QT syndrome is rare, although it is frequently accompanied by life-threatening arrhythmias. Infants may not survive childhood despite traditional management with beta-adrenergic blockade and pacing. ⋯ High-rate atrial pacing with 2:1 AV block is presented as a novel and "bridging" therapy for neonatal long QT syndrome and 2:1 AV block with ventricular arrhythmias. Definitive therapy with ICD implantation was then possible when patient size was substantially increased. The electrophysiologic response to intentional elevation of the serum potassium suggests a genetic defect in an inward potassium channel and demonstrates a possible therapy of long QT syndrome in some future patients.