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: 23 Apr 2015

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How do you compare to others with this condition?

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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: 23 Apr 2015

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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 347 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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Clinical characteristics and treatment outcomes of patients with Brugada syndrome in northeastern Thailand.
 

Author(s): Pattarapong Makarawate, Narumol Chaosuwannakit, Suda Vannaprasaht, Wichittra Tassaneeyakul, Kittisak Sawanyawisuth

Journal: Singapore Med J. 2014 Apr;55(4):217-20.

 

Brugada syndrome (BrS) is a common genetic cause of sudden cardiac arrest (SCA) due to polymorphic ventricular tachycardia and ventricular fibrillation. The current recommended therapy for high-risk BrS patients is the use of an implantable cardioverter defibrillator (ICD). The present ...

Last Updated: 25 Apr 2014

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Unexpected α-α interactions with NaV1.5 genetic variants in Brugada syndrome.
 

Author(s): Hugues Abriel, Valentin Sottas

Journal: Circ Cardiovasc Genet. 2014 Apr;7(2):97-9.

 

Last Updated: 16 Apr 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 41 free, full-text review articles on human participants. First 3 results:

Brugada syndrome.
 

Author(s): Ramon Brugada, Oscar Campuzano, Georgia Sarquella-Brugada, Josep Brugada, Pedro Brugada

Journal: Methodist Debakey Cardiovasc J. ;10(1):25-8.

 

Brugada syndrome is a rare cardiac arrhythmia characterized by electrocardiographic right bundle branch block and persistent ST-segment elevation in the right precordial leads. It is associated with ventricular fibrillation and a high risk for sudden cardiac death, predominantly in ...

Last Updated: 16 Jun 2014

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Arrhythmogenic cardiomyopathy and Brugada syndrome: diseases of the connexome.
 

Author(s): Esperanza Agullo-Pascual, Marina Cerrone, Mario Delmar

Journal: FEBS Lett.. 2014 Apr;588(8):1322-30.

 

This review summarizes data in support of the notion that the cardiac intercalated disc is the host of a protein interacting network, called "the connexome", where molecules classically defined as belonging to one particular structure (e.g., desmosomes, gap junctions, sodium channel ...

Last Updated: 15 Apr 2014

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Brugada syndrome 1992-2012: 20 years of scientific excitement, and more.
 

Author(s): Pedro Brugada, Josep Brugada, Denis Roy

Journal: Eur. Heart J.. 2013 Dec;34(47):3610-5.

 

This article describes recent progress on a novel disease that started as a scientific curiosity and has not yet found its end as a scientific revolution.

Last Updated: 16 Dec 2013

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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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Evaluation of Subcutaneous Implantable Cardiac Defibrillator in Brugada Patients
 

Status: Not yet recruiting

Condition Summary: Brugada Syndrome; S-ICD System (Implantable Defibrillator)

 

Last Updated: 22 Jan 2015

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Empiric Quinidine for Asymptomatic Brugada Syndrome
 

Status: Recruiting

Condition Summary: Arrhythmia

 

Last Updated: 23 Mar 2015

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