Unmasking a Silent Killer and Understanding Sudden Cardiac Death in Brugada Syndrome: A Traditional Review.

Autor:	Moturu A; Department of Internal Medicine, Sri Ramaswamy Memorial Medical College Hospital and Research Centre, Chennai, IND., Bhuchakra HP; Department of Internal Medicine, Apollo Institute of Medical Sciences and Research, Hyderabad, IND., Bodar YP; Department of Internal Medicine, Orenburg State Medical University, Orenburg, RUS., Gandhi SK; Department of Internal Medicine, M.P. Shah Government Medical College, Jamnagar, IND., Patel P; Department of Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.; Department of Internal Medicine, Medical College Baroda, Vadodara, IND., Gutlapalli SD; Department of Internal Medicine, Richmond University Medical Center, New York City, USA.; Department of Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA., Arulthasan V; Department of Internal Medicine, Richmond University Medical Center, New York City, USA., Otterbeck P; Department of Internal Medicine, Richmond University Medical Center, New York City, USA.
Jazyk:	angličtina
Zdroj:	Cureus [Cureus] 2023 Jun 28; Vol. 15 (6), pp. e41076. Date of Electronic Publication: 2023 Jun 28 (Print Publication: 2023).
DOI:	10.7759/cureus.41076
Abstrakt:	Brugada syndrome (BrS) is an intricate and heterogeneous genetic disorder that engenders a formidable risk of life-threatening ventricular arrhythmias (VAs). While initially regarded as an electrophysiological aberration, emergent studies have illuminated the presence of underlying structural anomalies in select BrS cases. Although mutations in the SCN5A gene encoding the α-subunit of the cardiac sodium channel were originally identified as a primary causative factor; they account for only a fraction of the syndrome's multifaceted complexity pointing at genetic heterogeneity as a contributing factor. Remarkably, BrS has been linked to a higher incidence of fatal arrhythmic incidents and sudden cardiac death (SCD) with about 4% of SCD cases thought to be caused by BrS. Patients who spontaneously exhibit type one Brugada ECGs are more likely to experience cardiac events, emphasizing the importance of early risk stratification. To aid in risk stratification, the Shanghai score; a multifactorial risk stratification scoring system that incorporates ECG, clinical history, family history, and genetic test results; is utilized to identify those most susceptible to SCD. Beyond single ECGs, evaluation of arrhythmic findings from 24-hour Holter monitoring, ECG variables, electrophysiologic study (EPS) status in the temporal domain, and EPS data collected over time are all critical factors in risk classification. Among management options avoidance of triggers, early risk stratification, and implantation of an Implantable Cardioverter-Defibrillator (ICD) are recommended for asymptomatic patients. For symptomatic patients, pharmacotherapy and ICD implantation are available, with the latter being a highly effective choice for treating and preventing lethal arrhythmias in BrS. Competing Interests: The authors have declared that no competing interests exist. (Copyright © 2023, Moturu et al.)
Databáze:	MEDLINE
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