Cardiac channelopathies are caused by mutations in proteins related to the intra-cellular transport of sodium, potassium and calcium ions. These ion channel abnormalities predispose the patient to episodes of lethal… Click to show full abstract
Cardiac channelopathies are caused by mutations in proteins related to the intra-cellular transport of sodium, potassium and calcium ions. These ion channel abnormalities predispose the patient to episodes of lethal ventricular arrhythmias such as TdP and ventricular tachycardia (VT) or fibrillation (VF). While some may die during such arrhythmias, these lethal arrhythmias can be non-sustained with spontaneous termination. Cardiac output is significantly decreased during these non-sustained episodes and the resultant cerebral hypoperfusion may result in seizures and/or syncope [3]. The term ‘torsadogenic seizures’ has been used to describe this kind of paroxysmal activity in the past [4]. Misdiagnosis as epilepsy has been shown to be the most important reason for delay in diagnosis and could potentially result in sudden death [5].
               
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