Brugada syndrome 1

Common Name(s)

Brugada syndrome 1

Brugada syndrome is characterized by an ST segment elevation in the right precordial electrocardiogram leads (so-called type 1 ECG) and a high incidence of sudden death in patients with structurally normal hearts. The syndrome typically manifests during adulthood, with a mean age of sudden death of 41 +/- 15 years, but also occurs in infants and children (summary by {2:Antzelevitch et al., 2005}). Genetic Heterogeneity of Brugada Syndrome Brugada syndrome-2 ({611777}) is caused by mutation in the GPD1L gene ({611778}) on chromosome 3p22. Brugada syndrome-3 ({611875}) and Brugada syndrome-4 ({611876}), the phenotypes of which include a shortened QT interval on ECG, are caused by mutation in the CACNA1C gene ({114205}) on chromosome 12p13 and CACNB2 gene ({600003}) on chromosome 10p12, respectively. Brugada syndrome-5 ({612838}) is caused by mutation in the SCN1B gene ({600235}) on chromosome 19q13. Brugada syndrome-6 ({613119}) is caused by mutation in the KCNE3 gene ({604433}) on chromosome 11q13. Brugada syndrome-7 ({613120}) is caused by mutation in the SCN3B gene ({608214}) on chromosome 11q24. Brugada syndrome-8 ({613123}) is caused by mutation in the HCN4 gene ({605206}) on chromosome 15q24.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Brugada syndrome 1" for support, advocacy or research.

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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 1" returned 11 free, full-text research articles on human participants. First 3 results:

Brugada syndrome and abnormal splicing of SCN5A in myotonic dystrophy type 1.
 

Author(s): Karim Wahbi, Vincent Algalarrondo, Henri Marc Bécane, Véronique Fressart, Chérif Beldjord, Kamel Azibi, Arnaud Lazarus, Nawal Berber, Hélène Radvanyi-Hoffman, Tanya Stojkovic, Anthony Béhin, Pascal Laforêt, Bruno Eymard, Stéphane Hatem, Denis Duboc

Journal: Arch Cardiovasc Dis. 2013 Dec;106(12):635-43.

 

In patients with myotonic dystrophy type 1 (DM1), the mechanisms underlying sudden cardiac death, which occurs in up to 1/3 of patients, are unclear.

Last Updated: 2 Dec 2013

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Ventricular fibrillation with a 2:1 conduction block over the right ventricle in a Brugada syndrome patient.
 

Author(s): Marek Jastrzębski, Piotr Kukla

Journal: Kardiol Pol. 2013 ;71(9):991.

 

Last Updated: 25 Sep 2013

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Sinus node disease in subjects with type 1 ECG pattern of Brugada syndrome.
 

Author(s): Konstantinos P Letsas, Panagiotis Korantzopoulos, Michael Efremidis, Reinhold Weber, Louiza Lioni, George Bakosis, Vassilios P Vassilikos, Spyridon Deftereos, Antonios Sideris, Thomas Arentz

Journal: J Cardiol. 2013 Mar;61(3):227-31.

 

The spectrum of phenotypes related to mutations of the SCN5A gene include Brugada syndrome (BS), long QT syndrome, progressive cardiac conduction defect, and sinus node disease (SND). The present study investigated the incidence of SND in subjects with type 1 electrocardiogram (ECG) pattern of BS.

Last Updated: 13 Mar 2013

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Reviews from the PubMed Database

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The terms "Brugada syndrome 1" returned 0 free, full-text review articles on human participants.

 
 
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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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