title: Clinical aspects and prognosis of Brugada syndrome in children.
abstract: BACKGROUND
  Brugada syndrome is an arrhythmogenic disease characterized by an ECG pattern of ST-segment elevation in the right precordial leads and augmented risk of sudden cardiac death. Little is known about the clinical presentation and prognosis of this disease in children.
  
  
  RESULTS
  Thirty children affected by Brugada syndrome who were <16 years of age (mean, 8+/-4 years) were included. All patients displayed a type I ECG pattern before or after drug provocation challenge. Diagnosis of Brugada syndrome was made under the following circumstances: aborted sudden death (n=1), syncope of unexplained origin (n=10), symptomatic supraventricular tachycardia (n=1), suspicious ECG (n=1), and family screening for Brugada syndrome (n=17). Syncope was precipitated by fever in 5 cases. Ten of 11 symptomatic patients displayed a spontaneous type I ECG. An implantable cardioverter-defibrillator was implanted in 5 children; 4 children were treated with hydroquinidine; and 1 child received a pacemaker because of symptomatic sick sinus syndrome. During a mean follow-up of 37+/-23 months, 1 child experienced sudden cardiac death, and 2 children received an appropriate implantable cardioverter-defibrillator shock; all of them were symptomatic and had manifested a type I ECG spontaneously. One child had a cardioverter-defibrillator infection that required explantation of the defibrillator.
  
  
  CONCLUSIONS
  In the largest population of children affected by Brugada syndrome described to date, fever represented the most important precipitating factor for arrhythmic events, and as in the adult population, the risk of arrhythmic events was higher in previously symptomatic patients and in those displaying a spontaneous type I ECG.
authors.affiliation: L'Institut du Thorax, CHU de Nantes, and INSERM U533, Nantes Cedex, France. vincent.probst@chu-nantes.fr
authors.forename: Vincent
authors.initials: V
authors.lastname: Probst
authors.forename: Isabelle
authors.initials: I
authors.lastname: Denjoy
authors.forename: Paola G
authors.initials: PG
authors.lastname: Meregalli
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Amirault
authors.forename: Fréderic
authors.initials: F
authors.lastname: Sacher
authors.forename: Jacques
authors.initials: J
authors.lastname: Mansourati
authors.forename: Dominique
authors.initials: D
authors.lastname: Babuty
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Villain
authors.forename: Jacques
authors.initials: J
authors.lastname: Victor
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Schott
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Lupoglazoff
authors.forename: Philippe
authors.initials: P
authors.lastname: Mabo
authors.forename: Christian
authors.initials: C
authors.lastname: Veltmann
authors.forename: Laurence
authors.initials: L
authors.lastname: Jesel
authors.forename: Philippe
authors.initials: P
authors.lastname: Chevalier
authors.forename: Sally-Ann B
authors.initials: SA
authors.lastname: Clur
authors.forename: Michel
authors.initials: M
authors.lastname: Haissaguerre
authors.forename: Christian
authors.initials: C
authors.lastname: Wolpert
authors.forename: Hervé
authors.initials: H
authors.lastname: Le Marec
authors.forename: Arthur A M
authors.initials: AA
authors.lastname: Wilde
mesh_terms: D000293:Adolescent; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D053840:Brugada Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D005799:Genes, Dominant; D006801:Humans; D007223:Infant; D008297:Male; D009124:Muscle Proteins / Q000235:genetics; D062554:NAV1.5 Voltage-Gated Sodium Channel; D011379:Prognosis; D011802:Quinidine / Q000627:therapeutic use; D026941:Sodium Channel Blockers; D015222:Sodium Channels / Q000235:genetics; D013575:Syncope / Q000209:etiology; D013617:Tachycardia, Supraventricular / Q000175:diagnosis / Q000209:etiology
chemical_list: D000889:Anti-Arrhythmia Agents; D009124:Muscle Proteins; D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human; D026941:Sodium Channel Blockers; D015222:Sodium Channels; D011802:Quinidine