Early Repolarization Syndrome Leading to Recurrent Cardiac Arrest in a Young Active Duty Patient

Abstract

A previously healthy, active duty 37-year-old male experienced recurrent cardiac arrests because of ventricular fibrillation and polymorphic ventricular tachycardia. Initial evaluation did not reveal a clear ischemic, structural, toxic, or metabolic cause. Close monitoring of telemetry before his third cardiac arrest revealed the cause to be early repolarization syndrome (ERS). In this case, we review the diagnosis, epidemiology, and prognostic significance of early repolarization pattern as it relates to ERS. We also discuss acute and long-term treatment strategies for patients with ERS.

KCNQ1 p.D446E Variant as a Risk Allele for Arrhythmogenic Phenotypes: Electrophysiological Characterization Reveals a Complex Phenotype Affecting the Slow Delayed Rectifier Potassium Current (IKs) Voltage Dependence by Causing a Hyperpolarizing Shift and a Lack of Response to Protein Kinase A Activation

“…Patients with ER show current imbalances between epi-and endocardial layers in a dispersion of de-and repolarization that might be the result of increased outward potassium currents, mainly the transient-outward K + (I to ) and adenosine triphosphate-sensitive current (IK ATP ) or decreased inward depolarizing currents (sodium I Na , and calcium I CaL ) [18]. Although KCNQ1 gain-of-function mutations have been described in short QT syndrome [19], to our knowledge there is only one report of a heterozygous KCNQ1 mutation also located within D5 (p.A399S) in a young man with ER and sudden cardiac death, without establishing the causal role of the mutation [20].…”