Brugada syndrome: Clinical features, risk stratification, and management

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.

Ventricular Tachycardia in an Elderly Male With Brugada Syndrome

“…5 Careful risk stratification and consideration of quinidine trial in select group of patients are warranted because excessive ICD shocks can occur with NSVT. 2 Quinidine works by blocking ion channels in the heart, including the sodium and potassium channels, which can help to mitigate atrial and ventricular arrhythmias. However, quinidine can also cause a variety of side effects, including nausea, diarrhea, headache, dizziness, and even potentially life-threatening arrhythmias.…”

“…Brugada syndrome can cause ventricular tachycardia (VT), ventricular fibrillation (VF), and SCD. 2 Acute stressors or drugs can unmask the typical ECG features and precipitate arrhythmia.…”

“… 5 Careful risk stratification and consideration of quinidine trial in select group of patients are warranted because excessive ICD shocks can occur with NSVT. 2 …”

“…A defibrillator was also not indicated due to the absence of any malignant symptoms such as syncope or a family 1201005H ICXXX10.1177/23247096231201005Journal of Investigative Medicine High Impact Case ReportsJaber et al case-report20232023 1 Riverside University Health System Medical Center, Moreno Valley, CA, USA 2 Loma Linda University Medical Center, CA, USA history of SCD. Instead, an implantable loop recorder (ILR) was implanted prior to discharge to monitor VT burden.…”

Novel SCN5A and GPD1L Variants Identified in Two Unrelated Han-Chinese Patients With Clinically Suspected Brugada Syndrome

“…BrS, which often manifests as syncope, has a comparatively higher prevalence in Southeast Asia than those in Europe or the United States ( 18 ). It is eight to ten times more common in adult men than women ( 19 ). The mean age of the first episode can be 40 years, while BrS may also occur in infancy or early childhood, even leading to sudden infant death syndrome ( 15 ).…”