&lt;p&gt;Brugada syndrome: updated perspectives&lt;/p&gt;

Walia, Jagdeep S.; Steinberg, Christian; Laksman, Zachary

doi:10.2147/rrcc.s182162

Cited by 5 publications

(5 citation statements)

References 101 publications

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“…Nevertheless, it is considered that a type 1 ECG solely, whether spontaneous or documented during a provocation test, is positive for BrS. Given the relative difficulty and absence of supporting evidence in its prognostic value, the Shanghai Score System has not yet achieved universal recognition; therefore, a large percentage of medical centers continue to diagnose BrS merely on a type 1 ECG in the absence of particular adjustable variables [ 25 ].…”

Section: Clinical Characteristics and Diagnostic Criteria Of Brsmentioning

confidence: 99%

Brugada Syndrome: From Molecular Mechanisms and Genetics to Risk Stratification

Popa

Şerban

Mărănducă

et al. 2023

IJMS

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Brugada syndrome (BrS) is a rare hereditary arrhythmia disorder, with a distinctive ECG pattern, correlated with an increased risk of ventricular arrhythmias and sudden cardiac death (SCD) in young adults. BrS is a complex entity in terms of mechanisms, genetics, diagnosis, arrhythmia risk stratification, and management. The main electrophysiological mechanism of BrS requires further research, with prevailing theories centered on aberrant repolarization, depolarization, and current-load match. Computational modelling, pre-clinical, and clinical research show that BrS molecular anomalies result in excitation wavelength (k) modifications, which eventually increase the risk of arrhythmia. Although a mutation in the SCN5A (Sodium Voltage-Gated Channel Alpha Subunit 5) gene was first reported almost two decades ago, BrS is still currently regarded as a Mendelian condition inherited in an autosomal dominant manner with incomplete penetrance, despite the recent developments in the field of genetics and the latest hypothesis of additional inheritance pathways proposing a more complex mode of inheritance. In spite of the extensive use of the next-generation sequencing (NGS) technique with high coverage, genetics remains unexplained in a number of clinically confirmed cases. Except for the SCN5A which encodes the cardiac sodium channel NaV1.5, susceptibility genes remain mostly unidentified. The predominance of cardiac transcription factor loci suggests that transcriptional regulation is essential to the Brugada syndrome’s pathogenesis. It appears that BrS is a multifactorial disease, which is influenced by several loci, each of which is affected by the environment. The primary challenge in individuals with a BrS type 1 ECG is to identify those who are at risk for sudden death, researchers propose the use of a multiparametric clinical and instrumental strategy for risk stratification. The aim of this review is to summarize the latest findings addressing the genetic architecture of BrS and to provide novel perspectives into its molecular underpinnings and novel models of risk stratification.

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Section: Clinical Characteristics and Diagnostic Criteria Of Brsmentioning

confidence: 99%

Brugada Syndrome: From Molecular Mechanisms and Genetics to Risk Stratification

Popa

Şerban

Mărănducă

et al. 2023

IJMS

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“…Brugada syndrome is an arrhythmogenic cardiac manifestation first described in 1992, which is responsible for ventricular tachycardia and sudden death. The Shanghai Criteria has been proposed for the diagnosis of Brugada syndrome, usually in the presence of a type 1 ECG [20]. There is overlap with ACM considering the ventricular fibrotic changes, dilatation of RVOT, RV focal dyskinesia, and even the presence of common gene mutations (sodium channel 5A-SCN5A).…”

Section: Differential Diagnosis-pathologic Conditionsmentioning

confidence: 99%

“…There is overlap with ACM considering the ventricular fibrotic changes, dilatation of RVOT, RV focal dyskinesia, and even the presence of common gene mutations (sodium channel 5A-SCN5A). However, patients with Brugada syndrome do not exhibit global RV systolic dysfunction or global dilatation [20], and one study showed that only 8% presented LGE and none met rTFC 2010 [21].…”

Section: Differential Diagnosis-pathologic Conditionsmentioning

confidence: 99%

Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI

Manole

Pintican

Popa³

et al. 2022

JPM

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Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involvement, which includes, in various proportions, both the right (RV) and left ventricle (LV), currently referred to as ACM (arrhythmogenic cardiomyopathy). Its pathological substrate is defined by the replacement of the ventricular myocardium with fibrous adipose tissue that further leads to inadequate electrical impulses and translates into varies degrees of malignant ventricular arrythmias and dyskinetic myocardium movements. Particularly, the cardio-cutaneous syndromes of Carvajal/Naxos represent rare causes of ACM that might be suspected from early childhood. The diagnostic is sometimes challenging, even with well-established rTFC or Padua criteria, especially for pediatric patients or ACM with LV involvement. Cardiac MRI gain more and more importance in ACM diagnostic especially in non-classical forms. Furthermore, MRI is useful in highlighting myocardial fibrosis, fatty replacement or wall movement with high accuracy, thus guiding not only the depiction, but also the patient’s stratification and management.

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“…[78] The causes of Brugada syndrome include genetic, environmental, and hormonal components. [79] The most feasible mechanism for n-3 polyunsaturated fatty acids' anti-arrhythmic action is altering the cardiac ion channels. [80] The rapid sodium channel current is inhibited by n-3 polyunsaturated fatty acids, while the transient outward potassium current and the delayed outward potassium current are promptly activated.…”

Section: Role Of Polyunsaturated Fatty Acids In Brugada Syndromementioning

confidence: 99%

A Comprehensive Review on Brugada Syndrome: Etiology, Hormonal role, Current Treatment Regimen, and Role of Polyunsaturated Fatty Acids

Bhattacharjee

Das

Ikbal

et al. 2021

Int. J. Pharm. Sci. Drug Res.

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Brugada syndrome is a disorder caused by alteration or mutation in the ion channels, abnormal ECG resulting in elevated ST segment and blockage of right bundle brank. It has a genetic history and can be passed down from the parents to their offspring and may be further complicated if someone already had a cardiac arrest or other cardiac-related issues. At present, there is no permanent cure strategy available to treat this disorder. However, some surgical and pharmacological intervention approaches, such as the use of defibrillators, catheters can provide some relief, but due to their excessive cost and complications, their use has been limited. Polyunsaturated fatty acids (PUFA) components: Eicosapentaenoic acid and docosahexaenoic acid were found to play a significant role in reducing the cardiovascular events and mortality rate. The primary sources of these fatty acids are fish and fish oils. This review attempts to summarize the cardioprotective role of PUFA in preventing ventricular arrhythmias and Brugada syndrome through alteration of cardiac ion channels.

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<p>Brugada syndrome: updated perspectives</p>

Cited by 5 publications

References 101 publications

Brugada Syndrome: From Molecular Mechanisms and Genetics to Risk Stratification

Brugada Syndrome: From Molecular Mechanisms and Genetics to Risk Stratification

Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI

A Comprehensive Review on Brugada Syndrome: Etiology, Hormonal role, Current Treatment Regimen, and Role of Polyunsaturated Fatty Acids

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