Brugada Syndrome

An autosomal dominant defect of cardiac conduction that is characterized by an abnormal ST-segment in leads V1-V3 on the ELECTROCARDIOGRAM resembling a right BUNDLE-BRANCH BLOCK; high risk of VENTRICULAR TACHYCARDIA; or VENTRICULAR FIBRILLATION; SYNCOPAL EPISODE; and possible sudden death. This syndrome is linked to mutations of gene encoding the cardiac SODIUM CHANNEL alpha subunit.

Also Known As:

Networked: 1270 relevant articles (39 outcomes, 96 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ventricular Fibrillation
2.	Paroxysmal ventricular fibrillation
3.	Sudden Death
4.	Ventricular Tachycardia
5.	Heart Arrest (Cardiac Arrest)

Experts

1.	Brugada, Pedro: 59 articles (05/2022 - 02/2002)
2.	Antzelevitch, Charles: 50 articles (05/2022 - 02/2002)
3.	Wilde, Arthur A M: 43 articles (12/2021 - 07/2002)
4.	Brugada, Josep: 42 articles (05/2022 - 02/2002)
5.	Brugada, Ramon: 38 articles (05/2022 - 02/2002)
6.	Probst, Vincent: 34 articles (10/2022 - 07/2002)
7.	Shimizu, Wataru: 30 articles (11/2020 - 02/2002)
8.	Tan, Hanno L: 25 articles (05/2022 - 03/2004)
9.	Behr, Elijah R: 24 articles (05/2022 - 05/2009)
10.	Borggrefe, Martin: 24 articles (05/2022 - 02/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Brugada Syndrome:

1.	Quinidine (Chinidin)FDA LinkGeneric 03/01/2009 - "The long-term reproducibility of the EP efficacy of quinidine in patients with idiopathic VF and Brugada syndrome is excellent. " 01/01/2013 - "We report two cases of Brugada syndrome that presented with electrical storm and were treated successfully with oral quinidine, an "endangered species" drug. " 09/28/2004 - "Efficacy of quinidine in high-risk patients with Brugada syndrome." 07/01/2018 - "Quinidine effective for the management of ventricular and atrial arrhythmias associated with Brugada syndrome." 07/14/2017 - "She was further evaluated by cardiology services and was diagnosed with type 2 Brugada syndrome, for which she was started on quinidine. "
2.	Ajmaline (Wolfina)FDA Link 11/01/2017 - "We describe a unique case in which a patient developed inferior J waves with an early repolarization-type electrocardiogram following successful epicardial infundibular substrate ablation (which eliminated the Brugada syndrome electrocardiogram on ajmaline challenge). " 01/01/2004 - "Our results support the hypothesis that (1) sudden unexpected death syndrome and Brugada syndrome are the same disease; (2) male predominance of the phenotype observed in sudden unexpected death syndrome does not apply to this family, suggesting that factors other than the specific mutation determine the gender distinction; and (3) ajmaline may provide false-positive results. " 12/19/2022 - "This study aimed to investigate the predictive value of T-peak to T-end interval and corrected T-peak to T-end interval for predicting the positive response of the ajmaline challenge test in suspected Brugada syndrome patients. " 03/02/2021 - "Evidence-based studies clearly showed that a positive ajmaline test does not provide any additional information on the risk stratification for major ventricular arrhythmic events on asymptomatic individuals with a non-diagnostic Brugada ECG pattern." 01/01/2018 - "This study aimed to assess the impact of systematic ajmaline provocation testing using high right precordial leads (RPLs) on the diagnostic yield of Brugada syndrome (BrS) in a large cohort of SADS families. "
3.	Anti-Arrhythmia AgentsIBA 11/01/2021 - "We aimed: 1) to validate the digitization process for paper ECGs in Brugada patients; and 2) to quantify the acute class I antiarrhythmic drug effect on relevant ECG parameters in Brugada syndrome. " 12/01/2015 - "Information on long-term clinical outcome of patients with Brugada syndrome treated with electrophysiologically guided class 1A antiarrhythmic drugs (AAD) is limited. " 10/01/2015 - "The ability to distinguish distinct molecular mechanisms of proximal Brugada syndrome mutations demonstrates the potential of these methods to reveal how inherited mutations, post-translational modifications, and antiarrhythmic drugs alter NaV1.5 at the molecular level." 01/01/2011 - "The type 1 (coved) ECG pattern is diagnostic for Brugada syndrome; types 2 and 3 require antiarrhythmic drug challenge to confirm its presence. " 02/01/2008 - "Because of the concerns of proarrhythmias with antiarrhythmic drugs, PVI may be an effective strategy for highly symptomatic patients with AF who have a Brugada ECG pattern."
4.	Procainamide (Procan)FDA LinkGeneric 01/01/2022 - "This novel DL model achieved cardiologist-level accuracy in classifying Brugada type 1. Applying DL to 24-hour 12-lead Holters significantly improved the detection of Brugada type 1 in patients with procainamide-induced and suspected Brugada syndrome. " 12/01/2001 - "Our data suggest that the new higher intercostal space lead ECG, with or without the procainamide test is helpful in detecting the Brugada sign in sudden unexplained death syndrome survivors and their relatives." 09/01/2014 - "This study analyzes a pediatric case series of procainamide challenges done to evaluate for possible Brugada syndrome. " 02/01/2022 - "Suspicion for Brugada syndrome leads to performing a procainamide challenge, which was significant for ST changes in the anterolateral leads. " 02/01/2022 - "Upon completion of a procainamide challenge, it was determined that he did not have Brugada syndrome and was shortly discharged. "
5.	Bepridil (Vascor)FDA Link 10/01/2022 - "Bepridil Might be Potent Against the Coronavirus Disease 2019-related Electrical Storm in Brugada Syndrome." 10/01/2010 - "The purpose of this study was to assess the efficacy of low-dose bepridil (100 mg/day) for VF prevention in patients with Brugada syndrome with and without SCN5A mutation. " 10/01/2010 - "Treatment with bepridil prevented recurrence of VF along with improvement of ST elevation and LAS40 in patients with Brugada syndrome with the SCN5A mutation." 10/01/2010 - "It has been reported that bepridil prevents ventricular fibrillation (VF) in patients with Brugada syndrome, but the comparative efficacy with and without mutation in the SCN5A gene has not been elucidated. " 10/01/2010 - "Efficacy of low-dose bepridil for prevention of ventricular fibrillation in patients with Brugada syndrome with and without SCN5A mutation."
6.	Disopyramide (Norpace)FDA LinkGeneric 06/01/2010 - "Findings observed in our case suggest that disopyramide could be added in our arsenal of medications for treating arrhythmic storms in patient with Brugada syndrome." 04/01/1997 - "However, ST-segment elevation and the rSr' pattern in leads V(1-3) characteristic of Brugada syndrome became exaggerated by disopyramide. " 04/01/1997 - "Discrepant drug action of disopyramide on ECG abnormalities and induction of ventricular arrhythmias in a patient with Brugada syndrome." 06/01/2010 - "High efficacy of disopyramide in the management of ventricular fibrillation storms in a patient with Brugada syndrome." 04/01/1997 - "Disopyramide exerted discrepant action on the electrocardiographic (ECG) abnormalities and induction of VF in this patient, suggesting the efficacy of antiarrhythmic drugs assessed by an electrophysiologic study may be unrelated to ECG abnormalities in cases of Brugada syndrome."
7.	Sodium Channels (Sodium Channel)IBA 07/01/2016 - "In addition, in vitro studies demonstrated the interaction between plakophilin-2 and sodium channel, the most prevalent gene associated with Brugada Syndrome. " 06/01/2016 - "The reported Brugada syndrome causing mutation R1512W in cardiac sodium channel α subunit encoded gene SCN5A, without obvious loss of function of cardiac sodium channel in previous in vitro study, was identified as the first genetic cause of Chinese SUNDS by us. " 09/10/2013 - "Previous studies have implicated that SUNDS is probably allelic to cardiac sodium channel diseases such as Brugada syndrome. " 09/01/2002 - "Systematic studies in patients with Brugada syndrome have demonstrated that a significant proportion of patients suffering from this SCN5A-gene mutation react with Brugada-like ECG changes on the administration of sodium channel blocking agents. " 11/01/2000 - "Molecular study did not reveal the same mutations in the cardiac sodium channel gene (SCN5A) as previously reported in Brugada syndrome. "
8.	Proteins (Proteins, Gene)FDA Link 01/01/2019 - "In this work, we present a computational study designed to obtain a fingerprint of Andersen-Tawil mutated proteins and differentiate them from mutated proteins associated with Brugada syndrome and from functional groups of proteins belonging to APD3, UniProt, and CPPsite databases. " 01/01/2020 - "Identification of transmembrane protein 168 mutation in familial Brugada syndrome." 01/01/2018 - "Loss-of-function mutations in SCN5A lead to lower expression levels of SCN5A or production of defective Nav1.5 proteins and cause Brugada syndrome, an electrical disease with minor structural changes in the heart. " 01/01/2017 - "This finding offers a new target for the treatment of the Brugada syndrome, based on protein maturation management. " 01/01/2016 - "Molecular Autopsy of Desmosomal Protein Plakophilin-2 in Sudden Unexplained Nocturnal Death Syndrome."
9.	Biomarkers (Surrogate Marker)IBA 01/01/2023 - "The aim of this study was to assess the role of micro-RNAs from peripheral blood as candidate biomarkers in Brugada syndrome. " 10/21/1997 - "We carried out a study to identify the clinical markers for patients at high risk of developing sudden unexplained death syndrome (SUDS) and long-term outcomes. " 01/01/2023 - "Leucocyte-derived micro-RNAs as candidate biomarkers in Brugada syndrome." 01/01/2020 - "A highly specific biomarker for Brugada syndrome. " 11/01/2018 - "Integration of "Omics" Strategies for Biomarkers Discovery and for the Elucidation of Molecular Mechanisms Underlying Brugada Syndrome."
10.	Sodium Channel BlockersIBA 12/01/2000 - "A mutation in a cardiac Na+ channel gene, SCN5A, has been linked to Brugada syndrome, and sodium channel blockers have been shown to be effective in unmasking the syndrome when concealed. " 03/16/2023 - "A diagnosis of Brugada syndrome (BrS) is based on the presence of a type 1 electrocardiogram (ECG) pattern, either spontaneously or after a Sodium Channel Blocker Provocation Test (SCBPT). " 03/16/2023 - "The r'-Wave Algorithm: A New Diagnostic Tool to Predict the Diagnosis of Brugada Syndrome after a Sodium Channel Blocker Provocation Test." 12/01/2022 - "The implications of a drug-induced type 1 Brugada ECG pattern following sodium channel blocker provocation (SCBP) are not fully understood. " 10/01/2022 - "Sodium channel blocker (SCB) infusion is used to unmask the electrocardiographic pattern of Brugada syndrome. "

Therapies and Procedures

1.	Therapeutics 09/01/2002 - "Despite the knowledge on Brugada syndrome has dramatically improved in the recent years, the clinical management is still often empirical and limited by the lack of pharmacological therapies. " 01/01/2020 - "Future research should focus on the identification of genes associated with the diseases and other risk factors, improved risk stratification and, in particular for Brugada syndrome, effective therapies." 01/01/2019 - "The online MEDLINE database was searched for published reports and yielded 828 studies on outcome and complications after ICD therapy in patients with Brugada syndrome. " 01/01/2018 - "The aim of this study was to identify possible triggers for appropriate ICD therapies in patients with Brugada syndrome, focusing on their past and current therapeutic profiles. " 03/10/2015 - "The objective of this study was to investigate clinical features, management, and long-term follow-up of ICD therapy in patients with Brugada syndrome. "
2.	Implantable Defibrillators (Implantable Cardioverter-Defibrillator) 01/01/2015 - "An implantable cardioverter-defibrillator (ICD) is the only proven effective therapeutic strategy for patients with Brugada syndrome (BS). " 11/01/2010 - "Implantable cardioverter-defibrillator placement remains the only effective treatment for patients with symptomatic Brugada syndrome; however, risk stratification of asymptomatic patients continues to remain a challenge. " 05/01/2023 - "Correction to: Subcutaneous versus transvenous implantable cardioverter-defibrillator among drug-induced type-1 ECG pattern Brugada syndrome: a propensity score matching analysis from IBRYD study." 05/01/2023 - "Subcutaneous versus transvenous implantable cardioverter-defibrillator among drug-induced type-1 ECG pattern Brugada syndrome: a propensity score matching analysis from IBRYD study." 01/01/2014 - "Our study aims to analyze prognosis after implantable cardioverter-defibrillator (ICD) implantation in Korean patients with Brugada syndrome (BrS). "
3.	Catheter Ablation 01/01/2021 - "Subsequently, several studies have shown that catheter ablation of these substrates normalizes the Brugada ECG pattern and is very effective in eliminating these substrates and preventing recurrent VF episodes. " 01/01/2018 - "We suggest that this novel ablation strategy is effective in Brugada syndrome patients with ICD, and early response after catheter ablation can be predicted by high precordial leads." 03/29/2011 - "Catheter ablation over this abnormal area results in normalization of the Brugada ECG pattern and prevents Vt/VF, both during electrophysiological studies as well as spontaneous recurrent Vt/VF episodes in patients with BrS." 10/27/2023 - "Outcomes of catheter ablation in high-risk patients with Brugada syndrome refusing an ICD implantation." 06/01/2023 - "Correction to: Efficacy and safety of catheter ablation for Brugada syndrome: an updated systematic review."
4.	Defibrillators (Defibrillator) 03/21/2006 - "Our data suggest that dmLSB is effective in eliminating the arrhythmogenic substrate responsible for the Brugada syndrome and that it deserves further study as a pharmacological adjunct to implanted cardioverter/defibrillator usage." 06/01/2008 - "Outcome after implantation of cardioverter defibrillator [corrected] in patients with Brugada syndrome: a multicenter Israeli study (ISRABRU)." 08/01/2019 - "He was diagnosed with Brugada syndrome due to which a subcutaneous implantable cardiac defibrillator (S-ICD) was inserted using the standard technique. " 06/01/2018 - "Concomitant Brugada syndrome substrate ablation and epicardial abdominal cardioverter-defibrillator implantation in a child." 10/17/2017 - "Patients With Brugada Syndrome and Implanted Cardioverter-Defibrillators: Long-Term Follow-Up."
5.	Drug Therapy (Chemotherapy) 01/01/2018 - "Complex interactions in a novel SCN5A compound mutation associated with long QT and Brugada syndrome: Implications for Na+ channel blocking pharmacotherapy for de novo conduction disease." 10/01/2005 - "Drug therapy in Brugada syndrome." 01/01/2004 - "Role of pharmacotherapy in Brugada syndrome." 01/01/2013 - "Drug Therapy For Preventing Ventricular Arrhythmia In Brugada syndrome: Do We Have The Answers Yet?" 08/01/2007 - "Brugada syndrome in the presence of coronary artery disease and parasympathomimetic drug therapy."