Childhood Brugada Syndrome in Two Korean Families.
10.4070/kcj.2010.40.3.143
- Author:
Yun Sik LEE
1
;
Jae Suk BAEK
;
So Yeon KIM
;
Sang Won SEO
;
Bo Sang KWON
;
Gi Beom KIM
;
Eun Jung BAE
;
Sung Sup PARK
;
Chung Il NOH
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. eunjbaek@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Brugada syndrome;
Tachycardia, ventricular
- MeSH:
Adult;
Brugada Syndrome;
Death, Sudden, Cardiac;
Electrocardiography;
Humans;
Korea;
Sodium Channels;
Tachycardia, Ventricular
- From:Korean Circulation Journal
2010;40(3):143-147
- CountryRepublic of Korea
- Language:English
-
Abstract:
Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.
