두 가계에서 진단된 소아 Brugada 증후군
Childhood Brugada Syndrome in Two Korean Families
Abstract
Since Brugada described patients with typical electrocardiogram (ECG) pattern and sudden cardiac death as a new clinical entity, Brugada syndrome has been diagnosed increasingly. This syndrome is known as an inherited disease via an autosomal dominant trait, and SCN5A mutation have been found in 20~25% of Brugada syndrome. Because Brugada syndrome primarily manifests in adulthood, only a little information is available about Brugada syndrome during childhood. Although there are several reports on adult Brugada syndrome in Korea, pediatric Brugada syndrome has not been reported. Herein, we report childhood Brugada syndrome in two families. One infantile Brugada syndrome patient and his family had SCN5A novel mutation (c.4035G>T, p.W1345C, heterozygote) in the domain III of the sodium channel.