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Review
. 2020 Apr-Jun;21(2):88-96.
doi: 10.4103/HEARTVIEWS.HEARTVIEWS_44_20. Epub 2020 Jun 29.

Brugada Syndrome: Clinical Features, Risk Stratification, and Management

Balal Rasheed Malik  1 , Ahmed Mohamed Ali Rudwan  2 , Mohamed Salah Abdelghani  3 , Mohammed Mohsen  3 , Shahul Hameed A Khan  3 , Nora Aljefairi  3 , Elsayed Mahmoud  3 , Nidal Asaad  3 , Sajad Ahmed Hayat  3
Affiliations
Review

Brugada Syndrome: Clinical Features, Risk Stratification, and Management

Balal Rasheed Malik et al. Heart Views. 2020 Apr-Jun.

Abstract

In 1992, the Brugada brothers published a patient series of aborted sudden death, who were successfully resuscitated from ventricular fibrillation (VF). These patients had a characteristic coved ST-segment elevation in the right precordial leads on their 12-lead electrocardiogram with no apparent structural heart abnormality. This disease was referred to as "right bundle branch block, persistent ST-segment elevation, and sudden death syndrome." The term Brugada syndrome (BrS) was first coined for this new arrhythmogenic entity in 1996. BrS is more prevalent in Southeast Asian ethnic groups and was considered a familial disease due to the presence of syncope and/or sudden deaths in several members of the same family, however, the genetic alteration was only noted in 1998. The genetic characterization of BrS has proven to be challenging. The most common and well-established BrS genotype involves loss-of-function mutations in the SCN5A gene, but only represents between 15% and 30% of the diagnosed patients. Patients with BrS can present with a range of symptoms which can include syncope, seizures, and nocturnal agonal breathing due to polymorphic ventricular tachycardia or VF. If these arrhythmias are sustained, sudden cardiac death may result. Despite the significant progress on the understanding of BrS over the last two decades, there remain a number of uncertainties and challenges; we present an update review on the subject.

Keywords: Brugada syndrome; electrical storm; epicardial ablation; polymorphic ventricular tachycardia; right ventricular outflow tract ablation; sudden cardiac death; ventricular fibrillation.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Type 1 Brugada electrocardiogram pattern showing a concave ST-segment elevation ≥2 mm in ≥1 right precordial lead, followed by a negative T-wave. (b) Type 2 Brugada electrocardiogram pattern showing a convex ST-segment elevation ≥0.5 mm (generally >2 mm) in ≥1 right precordial lead followed by a positive T-wave[31]
Figure 2
Figure 2
Pathophysiology of Brugada syndrome[27]
Figure 3
Figure 3
High-risk electrocardiogram features seen in some Brugada syndrome patients. (a) Early repolarization pattern in inferior and/or lateral leads.[45] (b) Fragmented QRS – arrows.[46] (c) Example of significant S wave in three patients with Brugada syndrome[49]
Figure 4
Figure 4
Summary flowchart for the management of Brugada syndrome patients[11]
See this image and copyright information in PMC

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