Pacing and clinical electrophysiology : PACE
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Pacing Clin Electrophysiol · May 2016
Case ReportsCompound Heterozygous Triadin Mutation Causing Cardiac Arrest in Two Siblings.
We present the case of two siblings who both presented with an out-of-hospital cardiac arrest at 2 years of age. Both siblings underwent internal cardiac defibrillator implantation and both had recurrent episodes of ventricular fibrillation (VF). ⋯ The combination of these mutations has resulted in a particularly arrhythmogenic phenotype, with cardiac arrest occurring at a very young age and recurrent episodes of VF despite β-blockade. Flecainide seems to have been very effective in preventing clinical arrhythmias for this particular mutation.