Clinical aspects and prognosis of Brugada syndrome in children.
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.
Probst
Vincent
V
L'Institut du Thorax, CHU de Nantes, and INSERM U533, Nantes Cedex, France. vincent.probst@chu-nantes.fr
Denjoy
Isabelle
I
Meregalli
Paola G
PG
Amirault
Jean-Christophe
JC
Sacher
Fréderic
F
Mansourati
Jacques
J
Babuty
Dominique
D
Villain
Elisabeth
E
Victor
Jacques
J
Schott
Jean-Jacques
JJ
Lupoglazoff
Jean-Marc
JM
Mabo
Philippe
P
Veltmann
Christian
C
Jesel
Laurence
L
Chevalier
Philippe
P
Clur
Sally-Ann B
SA
Haissaguerre
Michel
M
Wolpert
Christian
C
Le Marec
Hervé
H
Wilde
Arthur A M
AA
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
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