Management of channelopathies in children.

Abstract

Cardiac channelopathies are a group of hereditary diseases that expose affected children and adolescents to an increased risk of syncope and sudden cardiac death (SCD) due to malignant ventricular tachyarrhythmias. Although cardiac channelopathies are rare, with an estimated prevalence of 1:2000-1:10,000, early recognition in order to start treatment and prevent SCD is warranted. The following article provides an overview of current recommendations and facts on the diagnosis and treatment of cardiac channelopathies in children and adolescents. The most commonly encountered cardiac channelopathies during childhood and adolescence include long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada syndrome (BrS) and short QT syndrome (SQTS). While subjects with LQTS and CPVT commonly respond well to β‑blocker medication, and flecainide is an additional option in patients with CPVT, no such highly effective drug therapy exists for the treatment of patients with BrS or SQTS. Left cardiac sympathetic denervation is an additional treatment option in subjects with LQTS or CPVT. Implantable cardioverter-defibrillator implantation is indicated in patients with channelopathies and life-threatening ventricular tachyarrhythmias despite adequate antiarrhythmic medication.