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: 827 relevant articles (21 outcomes, 56 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ventricular Fibrillation
2.	Sudden Death
3.	Ventricular Tachycardia
4.	Shock
5.	Pain (Aches)

Experts

1.	Antzelevitch, Charles: 41 articles (01/2015 - 02/2002)
2.	Brugada, Pedro: 34 articles (08/2015 - 02/2002)
3.	Wilde, Arthur A M: 30 articles (01/2015 - 07/2002)
4.	Shimizu, Wataru: 25 articles (08/2015 - 02/2002)
5.	Brugada, Ramon: 24 articles (01/2015 - 02/2002)
6.	Ackerman, Michael J: 20 articles (08/2015 - 08/2002)
7.	Brugada, Josep: 20 articles (01/2014 - 02/2002)
8.	Tan, Hanno L: 17 articles (02/2015 - 03/2004)
9.	Priori, Silvia G: 17 articles (01/2015 - 05/2002)
10.	Probst, Vincent: 16 articles (12/2015 - 07/2002)

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." 12/01/2015 - "Quinidine in Brugada Syndrome: Still a Long Way to Go…." 07/01/2013 - "To examine the effectiveness of WK, alone and in combination with quinidine, to suppress arrhythmogenesis in an experimental model of Brugada syndrome (BrS). "
2.	Paroxysmal ventricular fibrillationIBA 07/01/2013 - "Prognosis and risk factors for patients age 35years or younger with Brugada syndrome (BrS) were prospectively explored in a sub-analysis of the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS) registry. " 12/01/2007 - "A total of 188 consecutive individuals with Brugada syndrome (mean age 53 +/- 14 years, 178 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). " 01/01/2005 - "The recurrence of cardiac events in patients with idiopathic ventricular fibrillation (VF) excluding patients with the Brugada syndrome is unclear since this entity remains present in previous studies. " 11/15/2015 - "The predictive factors of LFs were the age, male sex, taller body length, ICD vs. pacemaker, lesser lead number, extra-thoracic puncture of the axillary vein vs. a cut-down of the cephalic vein, use of recalled leads and patients with idiopathic ventricular fibrillation (IVF) and Brugada syndrome (BrS). " 06/01/2011 - "KCNE5 (KCNE1L) variants are novel modulators of Brugada syndrome and idiopathic ventricular fibrillation."
3.	Anti-Arrhythmia AgentsIBA 10/01/1999 - "We sought to study the long-term value of electrophysiologic (EP)-guided therapy with Class IA antiarrhythmic drugs in patients with idiopathic VF with or without the 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. " 12/01/2015 - "Management of Brugada Syndrome: Thirty-Three-Year Experience Using Electrophysiologically Guided Therapy With Class 1A Antiarrhythmic Drugs." 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." 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.	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."
5.	Ajmaline (Wolfina)FDA Link 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. " 03/15/2014 - "The purpose of this study was to assess the response of intravenous administration of ajmaline in patients older than 75 years suspected to be affected by AV conduction disease with respect to unmask high-degree His-Purkinje block or the typical Brugada ECG pattern. " 02/23/2010 - "Local depolarization abnormalities are the dominant pathophysiologic mechanism for type 1 electrocardiogram in brugada syndrome a study of electrocardiograms, vectorcardiograms, and body surface potential maps during ajmaline provocation." 12/01/2007 - "On the basis of electrophysiological study, positive ajmaline test, persistent ST segment elevation in inferior leads and other clinical features (PQ interval of 240 ms, family history of SCD) a diagnosis of variant Brugada syndrome was made. " 01/01/2015 - "Brugada syndrome, which partly results from Nav 1.5 dysfunction and causes increased VF occurrence, can be unmasked by ajmaline. "
6.	Sodium Channels (Sodium Channel)IBA 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. " 04/01/2000 - "In this study we have identified and electrophysiologically characterized, in patients having either LQTS or Brugada syndrome, three mutations in SCN5A (a cardiac sodium channel gene). " 11/01/2015 - "Enhanced fast-inactivated state stability of cardiac sodium channels by a novel voltage sensor SCN5A mutation, R1632C, as a cause of atypical Brugada syndrome."
7.	Ion Channels (Ion Channel)IBA 01/01/2014 - "Disease-targeted sequencing of ion channel genes identifies de novo mutations in patients with non-familial Brugada syndrome." 11/01/2009 - "Brugada syndrome (BrS), caused by ion channel abnormalities, is characterized by ST segment elevation and negative T waves in the right precordial electrocardiographic (ECG) leads recorded over the right ventricular outflow tract (RVOT). " 02/01/2009 - "Transcriptional profiling of ion channel genes in Brugada syndrome and other right ventricular arrhythmogenic diseases." 01/30/2007 - "Eighty-two consecutive probands with Brugada syndrome were screened for ion channel gene mutations with direct sequencing. " 09/01/2002 - "The Brugada syndrome is therefore considered to be an ion channel disease. "
8.	Bepridil (Vascor)FDA Link 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." 10/12/2013 - "Efficacy and safety of bepridil for prevention of ICD shocks in patients with Brugada syndrome and idiopathic ventricular fibrillation."
9.	Sodium Channel Blockers (Potassium Sparing Diuretics)IBA 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. " 10/01/2014 - "ST-segment elevation in the right precordial electrocardiography (ECG) leads in Brugada syndrome (BS) can be unmasked by class I anti-arrhythmic drugs (sodium channel blockers) administration. " 05/10/2013 - "Sodium channel blockers are used to unmask the diagnostic ECG pattern of the Brugada syndrome (BrS) in case of a non-diagnostic baseline ECG. " 05/10/2013 - "Clinical and electrocardiographic predictors of positive response to the intravenous sodium channel blockers in patients suspected of the Brugada syndrome." 01/01/2008 - "Sodium channel blockers enhance the temporal QT interval variability in the right precordial leads in Brugada syndrome."
10.	SugammadexIBA 05/01/2013 - "Sugammadex and general anaesthesia in a patient with Brugada syndrome." 01/01/2013 - "Anesthetic management of a patient with Brugada syndrome--the use of sugammadex in major abdominal surgery." 01/01/2013 - "Anesthetic management of a patient with Brugada syndrome - the use of sugammadex in major abdominal surgery." 06/01/2012 - "Rocuronium and sugammadex used effectively for electroconvulsive therapy in a patient with Brugada syndrome."

Therapies and Procedures

1.	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). " 09/28/2004 - "Automatic implantable cardioverter-defibrillator therapy is considered the only effective treatment for high-risk patients with Brugada syndrome. " 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. " 01/01/2014 - "Our study aims to analyze prognosis after implantable cardioverter-defibrillator (ICD) implantation in Korean patients with Brugada syndrome (BrS). " 06/01/2013 - "The aim of this study was to investigate the impact of HM in a population of children with Brugada syndrome (BS) who received an implantable cardioverter defibrillator (ICD). "
2.	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)." 08/01/2015 - "Evaluation of the necessity for cardioverter-defibrillator implantation in elderly patients with Brugada syndrome." 06/01/2015 - "Long-term prognosis of patients with Brugada syndrome and an implanted cardioverter-defibrillator." 04/01/2015 - "After other causes of Brugada-like pattern were excluded, Brugada syndrome was diagnosed and a cardioverter-defibrillator was implanted. "
3.	Drug Therapy (Chemotherapy) 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 - "We present a case report which illustrates two important principles regarding Brugada syndrome, the dynamic ECG manifestations and the elimination of confounding variables (in this case significant coronary artery disease and cholinergic pharmacotherapy) prior to definitive diagnosis." 08/01/2007 - "Brugada syndrome in the presence of coronary artery disease and parasympathomimetic drug therapy."
4.	Electric Countershock (Cardioversion) 03/01/2012 - "Electrocardiogram (ECG) after cardioversion showed profound QRS prolongation and ST-segment elevation suggestive of either hyperkalaemia, acute myocardial ischaemia, or Brugada syndrome. " 08/01/2008 - "Following electrical cardioversion, the electrocardiogram showed sinus rhythm with type 1 pattern of Brugada syndrome. " 06/05/2002 - "Thirty-four symptomatic patients with Brugada syndrome (33 men and 1 woman; 44 +/- 12 years old) were classified into two groups according to the inducibility of VF with PVS: 22 patients with induced VF requiring direct cardioversion for termination (Induced VF group) and 12 patients without induced VF (Noninduced VF group). " 11/01/2014 - "Here, we describe electrocardiographic findings of widespread ST-segment elevation lasting at least 1 hour after DC cardioversion for ventricular defibrillation due to Brugada syndrome and mimicking acute myocardial infarction (AMI). " 01/01/2012 - "We present a case of Brugada syndrome in a young patient whose typical ECG pattern was 'masked' after ibutilide was administered for atrial flutter cardioversion. "
5.	Denervation 03/01/2002 - "Iodine-123-metaiodobenzylguanidine scintigraphy of total cardiac adrenergic denervation in Brugada syndrome." 07/01/1998 - "Iodine-123-metaiodobenzylguanidine SPECT of regional cardiac adrenergic denervation in Brugada syndrome." 06/01/2015 - "New trial data are also described for acute coronary syndromes (clopidogrel, prasugrel, ticagrelor), stable coronary artery disease (ivabradine), percutaneous coronary intervention (the role of thrombectomy or treatment of non-culprit lesions during primary intervention, pressure wire studies and outcomes of new stent designs), transcatheter aortic valve intervention data, atrial fibrillation (anticoagulation and direct current cardioversion), electrophysiology (leadless pacemaker devices, use of quinidine in Brugada syndrome) and coronary prevention (landmark Ezetimibe outcome data, PCSK9 clinical trials, childhood prevalence of hypertension, renal denervation for resistant hypertension and the role of cardiac computerized tomography in cardiovascular screening). "