Heart Rate Variability in Patients with Congenital Long QT Syndrome

Background: The congenital long QT syndrome (LQTS) affecting myocardial repolarization is caused by mutations in different cardiac potassium or sodium channel genes. Adrenergic triggers are known to initiate life‐threatening torsade de pointes ventricular tachycardias in LQTS patients, and anti‐adrenergic therapy has been shown to be effective in many cases. Despite this well‐documented adrenergic component, the data about autonomic modulation of the heart rate in LQTS, as described by heart rate variability (HRV) analysis, are very limited.

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