[Brugada syndrome].
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.
Denjoy
Isabelle
I
Service de cardiologie, Hôpital Lariboisière, Paris. isabelle.denjoy@lrb.aphp.fr
Extramiana
Fabrice
F
Lupoglazoff
Jean-Marc
JM
Leenhardt
Antoine
A
D000465:Algorithms; D000889:Anti-Arrhythmia Agents; D001208:Asia / Q000453:epidemiology; D053840:Brugada Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D002037:Bundle-Branch Block / Q000209:etiology; D000075202:Contraindications; D016757:Death, Sudden, Cardiac / Q000209:etiology; D003663:Decision Trees; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005602:France / Q000453:epidemiology; D005799:Genes, Dominant / Q000235:genetics; D005817:Genetic Counseling; D005820:Genetic Testing; D006801:Humans; D009124:Muscle Proteins / Q000235:genetics; D009154:Mutation / Q000235:genetics; D062554:NAV1.5 Voltage-Gated Sodium Channel; D019683:Penetrance; D015995:Prevalence; D011379:Prognosis; D011619:Psychotropic Drugs; D018570:Risk Assessment; D015222:Sodium Channels / Q000235:genetics; D013575:Syncope / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000209:etiology
D000889:Anti-Arrhythmia Agents; D009124:Muscle Proteins; D062554:NAV1.5 Voltage-Gated Sodium Channel; D011619:Psychotropic Drugs; C568320:SCN5A protein, human; D015222:Sodium Channels