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

Next Generation Sequencing and Linkage Analysis for the Molecular Diagnosis of a Novel Overlapping Syndrome Characterized by Hypertrophic Cardiomyopathy and Typical Electrical Instability of Brugada Syndrome.
 

Author(s): Ruggiero Mango, Andrea Luchetti, Raffaele Sangiuolo, Valentina Ferradini, Nicola Briglia, Emiliano Giardina, Fabrizio Ferrè, Manuela Helmer Citterich, Francesco Romeo, Giuseppe Novelli, Federica Sangiuolo

Journal: Circ. J.. 2016 ;80(4):938-49.

 

Familial hypertrophic cardiomyopathy (HCM) is an autosomal dominant inherited disorder; mutations in at least 20 genes have been associated. Brugada syndrome (BrS) is an autosomal dominant inherited disorder caused by mutations mainly in theSCN5Agene. A new clinical entity that consists ...

Last Updated: 29 Mar 2016

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Common Variant Near HEY2 Has a Protective Effect on Ventricular Fibrillation Occurrence in Brugada Syndrome by Regulating the Repolarization Current.
 

Author(s): Yukiko Nakano, Hidenori Ochi, Yuko Onohara, Masaaki Toshishige, Takehito Tokuyama, Hiroya Matsumura, Hiroshi Kawazoe, Shunsuke Tomomori, Akinori Sairaku, Yoshikazu Watanabe, Hiroki Ikenaga, Chikaaki Motoda, Kazuyoshi Suenari, Yasufumi Hayashida, Daiki Miki, Nozomu Oda, Shinji Kishimoto, Noboru Oda, Yukihiko Yoshida, Satoshi Tashiro, Kazuaki Chayama, Yasuki Kihara

Journal: Circ Arrhythm Electrophysiol. 2016 Jan;9(1):e003436.

 

Risk stratification of Brugada syndrome (BrS) remains controversial and the majority of patients with BrS have no genetic explanation. We investigated relationships between genotypes of 3 single-nucleotide polymorphisms reported in a recent genome-wide association study and BrS phenotypes.

Last Updated: 5 Jan 2016

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Quinidine in Brugada Syndrome: Still a Long Way to Go….
 

Author(s): Vincent Probst, Jean-Baptiste Gourraud

Journal: Circ Arrhythm Electrophysiol. 2015 Dec;8(6):1309-10.

 

Last Updated: 16 Dec 2015

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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 54 free, full-text review articles on human participants. First 3 results:

A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome.
 

Author(s): Ofer Havakuk, Sami Viskin

Journal: J. Am. Coll. Cardiol.. 2016 Jan;67(1):100-8.

 

The Brugada syndrome (BrS) and long-QT syndrome (LQTS) present as congenital or acquired disorders with diagnostic electrocardiograms (ST-segment elevation and prolonged QT interval, respectively) and increased risk for malignant arrhythmias. Our understanding of the 2 disease forms ...

Last Updated: 14 Jan 2016

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Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects.
 

Author(s): Charles Antzelevitch, Bence Patocskai

Journal: Curr Probl Cardiol. 2016 Jan;41(1):7-57.

 

Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome first described as a new clinical entity in 1992. Electrocardiographically characterized by distinct coved type ST segment elevation in the right-precordial leads, the syndrome is associated with a high risk for sudden ...

Last Updated: 16 Dec 2015

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Update on the Diagnosis and Management of Brugada Syndrome.
 

Author(s): Jitendra Vohra, Sulekha Rajagopalan,

Journal: Heart Lung Circ. 2015 Dec;24(12):1141-8.

 

Brugada Syndrome (BrS) is an autosomal dominant channelopathy with variable penetrance affecting the sodium channel. It reduces the transport of sodium ions essential for proper generation of the cardiac action potential. The resulting inhomogeneous repolarisation in areas of the ...

Last Updated: 21 Nov 2015

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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

Ablation in Brugada Syndrome for the Prevention of VF
 

Status: Not yet recruiting

Condition Summary: Brugada Syndrome

 

Last Updated: 26 Sep 2016

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Diagnostic Value and Safety of Flecainide Infusion Test in Brugada Syndrome
 

Status: Recruiting

Condition Summary: Brugada Syndrome; Arrhythmogenic Right Ventricular Cardiomyopathy

 

Last Updated: 21 Sep 2016

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Empiric Quinidine for Asymptomatic Brugada Syndrome
 

Status: Recruiting

Condition Summary: Arrhythmia

 

Last Updated: 8 Jan 2017

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