Preprint Case Report Version 1 Preserved in Portico This version is not peer-reviewed

A Novel Presentation: Epsilon Waves in the Electrocardiogram of a Patient with Dravet Syndrome Without Structural Heart Disease

Version 1 : Received: 14 April 2024 / Approved: 15 April 2024 / Online: 15 April 2024 (14:22:05 CEST)

A peer-reviewed article of this Preprint also exists.

Bermejo Valdés, A. J., & Archer Jiménez, J. (2024). A Novel Presentation: Epsilon Waves in the Electrocardiogram of a Patient with Dravet Syndrome Without Structural Heart Disease. Journal of Clinical Cardiology and Cardiovascular Interventions, 7(4). doi: 10.31579/2641-0419/371. Bermejo Valdés, A. J., & Archer Jiménez, J. (2024). A Novel Presentation: Epsilon Waves in the Electrocardiogram of a Patient with Dravet Syndrome Without Structural Heart Disease. Journal of Clinical Cardiology and Cardiovascular Interventions, 7(4). doi: 10.31579/2641-0419/371.

Abstract

We document for the first time epsilon waves on the electrocardiogram of a patient with Dravet syndrome (DS) who does not exhibit structural cardiac pathology. DS, a severe form of pediatric epilepsy, involves mutations in the SCN1A gene, which disrupt sodium ion flow and increase the risk of sudden unexpected death in epilepsy. The epsilon waves, typically associated with arrhythmogenic right ventricular dysplasia, may also suggest a link between neuronal and cardiac dysfunctions. In our case, the epsilon waves appear to originate from abnormalities in sodium channels rather than structural changes in the myocardium. This finding underscores the importance of assessing both neurological and cardiac manifestations in patients with severe epilepsy, opening new avenues for the research and treatment of these interactions in DS.

Keywords

Dravet syndrome; epsilon waves; arrhythmogenic right ventricular dysplasia; sudden unexpected death in epilepsy; SCN1A gene

Subject

Medicine and Pharmacology, Cardiac and Cardiovascular Systems

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