Abstract
IntroductionChannelopathies are associated with mutations of genes encoding proteins creating or interacting with the specialized ion channels in myocardial cell membranes, thus forming arrhythmogenic substrate predisposing the patient to sudden cardiac death. The study focuses the clinical and ECG presentation and management of children with channelopathies in Slovakia. Subject and methodsTwenty-two children with suspected channelopathy were admitted to Children's Cardiac Center Bratislava in the years 2007–2014. Genetic testing was made in 19 patients. ResultsFourteen patients were symptomatic. Long QT syndrome was genetically proven in eight and catecholaminergic polymorphic ventricular tachycardia in five patients. Twenty children are treated with beta-blockers, five in combination with mexiletine or flecainide. Nine patients received implantable cardiac defibrillator and one underwent left cardiac sympathetic denervation. ConclusionBoth clinical presentation and genetic testing must be considered in the diagnostic and therapeutic process of channelopathies. Early diagnosis allows for adequate treatment and lifestyle modification.
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