Hereditary Syndromes of Sudden Cardiac Death

Jeremy G Berberian

doi:10.1016/j.emc.2022.06.005

Hereditary Syndromes of Sudden Cardiac Death

Emerg Med Clin North Am. 2022 Nov;40(4):651-662. doi: 10.1016/j.emc.2022.06.005. Epub 2022 Oct 7.

Author

Jeremy G Berberian¹

Affiliation

¹ Department of Emergency Medicine, Christiana Care, 4755 Ogletown Stanton Road, Newark, DE 19718, USA. Electronic address: Jgberberian@gmail.com.

PMID: 36396213
DOI: 10.1016/j.emc.2022.06.005

Abstract

Sudden cardiac death (SCD) describes the unexpected natural death from a cardiac cause within a short time period, generally 1 hour or lesser from the onset of symptoms, often due to a cardiac dysrhythmia. Overall, the most common cause of SCD is coronary artery disease but for patients aged younger than 35 years, the most common cause of SCD is a dysrhythmia in the setting of a structurally normal heart. This article will review the background, diagnosis, and management of the common hereditary channelopathies and cardiomyopathies associated with an increased risk of SCD in patients without ischemic heart disease.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Arrhythmogenic right ventricular dysplasia; Brugada syndrome; Cardiogenic syncope; Epsilon waves; Hypertrophic cardiomyopathy; Hypertrophic obstructive cardiomyopathy; Long QT syndrome.

Publication types

Review

MeSH terms

Aged
Arrhythmias, Cardiac / diagnosis
Arrhythmias, Cardiac / genetics
Arrhythmias, Cardiac / therapy
Cardiomyopathies* / complications
Death, Sudden, Cardiac* / etiology
Death, Sudden, Cardiac* / prevention & control
Humans
Syndrome