Document Detail

The Brugada syndrome: clinical, genetic, cellular, and molecular abnormalities.
MedLine Citation:
PMID:  11343671     Owner:  NLM     Status:  MEDLINE    
The Brugada syndrome is an arrhythmic syndrome characterized by a right bundle branch block pattern and ST segment elevation in the right precordial leads of the electrocardiogram in conjunction with a high incidence of sudden death secondary to ventricular tachyarrhythmias. No evidence of structural heart disease is noted during diagnostic evaluation of these patients. In 25% of families, there appears to be an autosomal dominant mode of transmission with variable expression of the abnormal gene. Mutations have been identified in the gene that encodes the alpha subunit of the sodium channel (SCN5A) on chromosome 3. This genetic defect causes a reduction in the density of the sodium current and explains the worsening of the above electrocardiographic abnormalities when patients are treated with sodium channel blocking antiarrhythmic agents, which further diminish the already reduced sodium current. The prognosis is poor with up to a 10% per year mortality. Antiarrhythmic drugs including beta-blockers and amiodarone have no benefit in prolonging survival. The treatment of choice is the insertion of an implantable cardioverter-defibrillator.
G V Naccarelli; C Antzelevitch
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  The American journal of medicine     Volume:  110     ISSN:  0002-9343     ISO Abbreviation:  Am. J. Med.     Publication Date:  2001 May 
Date Detail:
Created Date:  2001-05-09     Completed Date:  2001-05-17     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0267200     Medline TA:  Am J Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  573-81     Citation Subset:  AIM; IM    
Division of Cardiology, Cardiovascular Center, Pennsylvania State University College of Medicine, Hershey, Pennsylvania, USA.
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MeSH Terms
Action Potentials
Arrhythmias, Cardiac* / diagnosis,  genetics,  physiopathology,  therapy
Defibrillators, Implantable
Molecular Biology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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