Clinical aspects and prognosis of Brugada syndrome in children

Probst, Vincent
Denjoy, Isabelle
Meregalli, Paola G.
Amirault, Jean-Christophe
Sacher, Frederic
Mansourati, Jacques
Babuty, Dominique
Villain, Elisabeth
Victor, Jacques
Schott, Jean-Jacques
Lupoglazoff, Jean-Marc
Mabo, Philippe
Veltmann, Christian
Jesel, Laurence
Chevalier, Philippe
Clur, Sally-Ann B.
Haissaguerre, Michel
Wolpert, Christian
Le Marec, Herve
Wilde, Arthur A. M.

Open link

Publication date

January 2007

DOI

10.1161/circulationaha.106.664219

Publisher

Ovid Technologies (Wolters Kluwer Health)

ISSN

0009-7322

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. METHODS AND 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=1...