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[Brugada syndrome].
- Source :
-
Presse medicale (Paris, France : 1983) [Presse Med] 2007 Jul-Aug; Vol. 36 (7-8), pp. 1109-16. Date of Electronic Publication: 2007 Apr 18. - Publication Year :
- 2007
-
Abstract
- Brugada syndrome is characterized clinically by the onset of syncopes or sudden death related to ventricular tachyarrhythmias in patients with a structurally normal heart. Its electrocardiographic features include right bundle branch bloc and ST-segment elevations in the precordial leads V1-V3. The estimated prevalence is 1 per 1000 in Asian countries and probably lower elsewhere: Asia is probably a birthplace of the syndrome. Its transmission is autosomal dominant with variable penetrance. Mutations have been identified in a gene coding for the alpha subunit of the sodium channel (SCN5A) in only 25% of cases. These genetic abnormalities cause a reduction of the density of the sodium current and explain the aggravation of electrocardiographic abnormalities caused by antiarrhythmic sodium channel blockers. Prognosis is very serious in symptomatic patients: prevention of sudden death requires implantation of an automatic defibrillator. The treatment decision is much more difficult for asymptomatic subjects with no family history.
- Subjects :
- Algorithms
Anti-Arrhythmia Agents
Asia epidemiology
Bundle-Branch Block etiology
Contraindications
Death, Sudden, Cardiac etiology
Decision Trees
Defibrillators, Implantable
Electrocardiography
France epidemiology
Genes, Dominant genetics
Genetic Counseling
Genetic Testing
Humans
Muscle Proteins genetics
Mutation genetics
NAV1.5 Voltage-Gated Sodium Channel
Penetrance
Prevalence
Prognosis
Psychotropic Drugs
Risk Assessment
Sodium Channels genetics
Syncope etiology
Tachycardia, Ventricular etiology
Brugada Syndrome diagnosis
Brugada Syndrome epidemiology
Brugada Syndrome genetics
Brugada Syndrome therapy
Subjects
Details
- Language :
- French
- ISSN :
- 0755-4982
- Volume :
- 36
- Issue :
- 7-8
- Database :
- MEDLINE
- Journal :
- Presse medicale (Paris, France : 1983)
- Publication Type :
- Academic Journal
- Accession number :
- 17446038
- Full Text :
- https://doi.org/10.1016/j.lpm.2007.03.019