Brugada Syndrome

Common Name(s)

Brugada Syndrome

Brugada syndrome is a condition that causes a disruption of the heart's normal rhythm (arrhythmia). Signs and symptoms usually develop in adulthood and include ventricular arrhythmia that can cause fainting, seizures, difficulty breathing, or sudden death. These complications often occur when an affected person is resting or asleep. Brugada syndrome is inherited in an autosomal dominant manner and may be caused by a mutation in any of several genes (most commonly the SCN5A gene). In many cases, the genetic cause is not identified. There have also been reports of acquired Brugada syndrome which have been associated with adverse reactions to certain drugs or other substances. Treatment may include use of an implantable cardioverter defibrillator (ICD) in people with a history of fainting or heart attack.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Brugada Syndrome" for support, advocacy or research.

SADS (Sudden Arrhythmia Death Syndromes) Foundation

Our mission is to save the lives of young people who are genetically predisposed to sudden death due to cardiac arrhythmia and to provide education and support to families and the medical community.

Last Updated: 29 Apr 2014

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Brugada Syndrome" for support, advocacy or research.

SADS (Sudden Arrhythmia Death Syndromes) Foundation

Our mission is to save the lives of young people who are genetically predisposed to sudden death due to cardiac arrhythmia and to provide education and support to families and the medical community.

http://www.StopSADS.org

Last Updated: 29 Apr 2014

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General Support Organizations

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General Resources

SADS Foundation Website

Information on living with SADS, diagnosis, treatment, prevention, and advocacy/education.

Updated 29 Apr 2014

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Brugada Syndrome" returned 339 free, full-text research articles on human participants. First 3 results:

Effect of the Brugada syndrome mutation A39V on calmodulin regulation of Cav1.2 channels.
 

Author(s): Brett A Simms, Ivana Assis Souza, Gerald W Zamponi

Journal:

 

The L-type calcium channel Cav1.2 is important for brain and heart function. The ubiquitous calcium sensing protein calmodulin (CaM) regulates calcium dependent gating of Cav1.2 channels by reducing calcium influx, a process known as calcium-dependent inactivation (CDI). Dissecting ...

Last Updated: 3 Jun 2014

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Event rates and risk factors in patients with Brugada syndrome and no prior cardiac arrest: a cumulative analysis of the largest available studies distinguishing ICD-recorded fast ventricular arrhythmias and sudden death.
 

Author(s): Pietro Delise, Giuseppe Allocca, Nadir Sitta, Paola DiStefano

Journal: Heart Rhythm. 2014 Feb;11(2):252-8.

 

All available studies that have addressed the issue of risk stratification in patients with type 1 Brugada electrocardiographic (ECG) pattern have considered a combined end point constituted by implantable cardioverter-defibrillator-recorded fast ventricular arrhythmias (ICD-FVA) ...

Last Updated: 10 Mar 2014

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Utilizing multiple in silico analyses to identify putative causal SCN5A variants in Brugada syndrome.
 

Author(s): Jyh-Ming Jimmy Juang, Tzu-Pin Lu, Liang-Chuan Lai, Chia-Hsiang Hsueh, Yen-Bin Liu, Chia-Ti Tsai, Lian-Yu Lin, Chih-Chieh Yu, Juey-Jen Hwang, Fu-Tien Chiang, Sherri Shih-Fan Yeh, Wen-Pin Chen, Eric Y Chuang, Ling-Ping Lai, Jiunn-Lee Lin

Journal:

 

Brugada syndrome (BrS) is an inheritable sudden cardiac death disease mainly caused by SCN5A mutations. Traditional approaches can be costly and time-consuming if all candidate variants need to be validated through in vitro studies. Therefore, we developed a new approach by combining ...

Last Updated: 27 Jan 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Brugada Syndrome" returned 38 free, full-text review articles on human participants. First 3 results:

Brugada syndrome.
 

Author(s): Jessica Jellins, Mitchell Milanovic, David-Joel Taitz, S H Wan, P W Yam

Journal: Hong Kong Med J. 2013 Apr;19(2):159-67.

 

As a clinical entity the Brugada syndrome has existed since 1992 and has been associated with a high risk of sudden cardiac death predominately in younger males. Patients can present with symptoms (ie syncope, palpitations, aborted sudden cardiac death) and asymptomatically. Its three ...

Last Updated: 28 Mar 2013

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Brugada syndrome: two decades of progress.
 

Author(s): Gumpanart Veerakul, Koonlawee Nademanee

Journal: Circ. J.. 2012 ;76(12):2713-22.

 

Two decades ago, a series of 8 idiopathic ventricular fibrillation patients who each had an abnormal ECG (right bundle branch block with coved-type ECG), but otherwise had normal hearts were described by Brugada and Brugada. Since then, the clinical entity has become known as Brugada ...

Last Updated: 22 Nov 2012

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Brugada syndrome 2012.
 

Author(s): Paola Berne, Josep Brugada

Journal: Circ. J.. 2012 ;76(7):1563-71.

 

Brugada syndrome (BS) is a cardiac disorder characterized by typical ECG alterations, and it is associated with a high risk for sudden cardiac death (SCD), affecting young subjects with structurally normal hearts. The prevalence of this disorder is still uncertain, presenting marked ...

Last Updated: 13 Jul 2012

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

DAPERB 3,4-DiAminoPyridine and Electrophysiological Response in Brugada Syndrome
 

Status: Not yet recruiting

Condition Summary: Brugada Syndrome

 

Last Updated: 1 Jul 2008

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Empiric Quinidine for Asymptomatic Brugada Syndrome
 

Status: Recruiting

Condition Summary: Arrhythmia

 

Last Updated: 20 May 2014

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Registry of Unexplained Cardiac Arrest
 

Status: Recruiting

Condition Summary: Cardiac Arrest; Long QT Syndrome; Brugada Syndrome; Catecholamine Sensitive Polymorphic Ventricular Tachycardia; Idiopathic Ventricular Fibrillation; Early Repolarization Syndrome

 

Last Updated: 7 Dec 2012

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