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 335 free, full-text research articles on human participants. First 3 results:

Functional characterization of a novel frameshift mutation in the C-terminus of the Nav1.5 channel underlying a Brugada syndrome with variable expression in a Spanish family.
 

Author(s): Pablo Dolz-Gaitón, Mercedes Núñez, Lucía Núñez, Adriana Barana, Irene Amorós, Marcos Matamoros, Marta Pérez-Hernández, Marta González de la Fuente, Miguel Alvarez-López, Rosa Macías-Ruiz, Luis Tercedor-Sánchez, Juan Jiménez-Jáimez, Eva Delpón, Ricardo Caballero, Juan Tamargo

Journal:

 

We functionally analyzed a frameshift mutation in the SCN5A gene encoding cardiac Na(+) channels (Nav1.5) found in a proband with repeated episodes of ventricular fibrillation who presented bradycardia and paroxysmal atrial fibrillation. Seven relatives also carry the mutation and ...

Last Updated: 23 Dec 2013

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Prognosis after implantation of cardioverter-defibrillators in Korean patients with Brugada syndrome.
 

Author(s): Myoung Kyun Son, Kyeongmin Byeon, Seung-Jung Park, June Soo Kim, Gi-Byoung Nam, Kee-Joon Choi, You-Ho Kim, Sang Weon Park, Young-Hoon Kim, Hyung Wook Park, Jeong Gwan Cho, Young Keun On

Journal: Yonsei Med. J.. 2014 Jan;55(1):37-45.

 

Our study aims to analyze prognosis after implantable cardioverter-defibrillator (ICD) implantation in Korean patients with Brugada syndrome (BrS).

Last Updated: 16 Dec 2013

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Electrophysiological characteristics of a SCN5A voltage sensors mutation R1629Q associated with Brugada syndrome.
 

Author(s): Zhipeng Zeng, Jieqiong Zhou, Yuxi Hou, Xiaojing Liang, Ziguan Zhang, Xuejing Xu, Qiang Xie, Weihua Li, Zhengrong Huang

Journal:

 

Brugada syndrome (BrS) is an inherited arrhythmogenic syndrome leading to sudden cardiac death, partially associated with autosomal dominant mutations in SCN5A, which encodes the cardiac sodium channel alpha-subunit (Nav1.5). To date some SCN5A mutations related with BrS have been ...

Last Updated: 29 Oct 2013

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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 40 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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