Normalization of Ventricular Repolarization with Flecainide in Long QT Syndrome Patients with SCN5A:ΔKPQ Mutation

摘要

The Long QT Syndrome (LQTS) is a genetic channelopathy with life‐threatening implications. The LQT3 form of this disease is caused by mutations of the SCN5A sodium‐channel gene. A specific mutation, SCN5A:ΔKPQ, is associated with repetitive reopenings of the sodium channel and prolonged inward current. This dominant inward current is manifest on the electrocardiogram as QT prolongation. Flecainide is a potent blocker of the open sodium channel.