Cardiac Channelopathies
Cardiac channelopathies are a group of heterogeneous disorders that affect the ion channels of the heart. These disorders cause cardiac arrhythmias in otherwise healthy and typically young individuals and can lead to syncope, cardiac arrest and sudden death. The ion channels mediate the flow of potassium, sodium and calcium across the myocytes. Inherited channelopathies can be due to mutations in  sodium, potassium, or calcium ion channel genes and are characterized by prolonged ventricular repolarization and risk for polymorphic ventricular tachycardia-and/or ventricular fibrillation.

Brugada Syndrome (BrS)
Loss of function variants in the sodium channel gene SCN5A account for 15-20% of the cases of BrS. BrS also causes ventricular arrhythmias, but the ECG is characterized by right precordial ST segment elevation and incomplete right bundle branch block rather than a prolonged QT interval. BrS is usually diagnosed at a later age than LQTS, often not until age 40. Implantation of an ICD is the only established effective treatment.

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