A Case of Congenital Long QT Syndrome with Reccurent Syncope.
- Author:
Jong Hwa HWANG
1
;
Hong Bae KIM
Author Information
1. Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea.
- Publication Type:Original Article
- Keywords:
Congenital Long QT Syndrome;
Syncope
- MeSH:
Adolescent;
Arrhythmias, Cardiac;
Atenolol;
Deafness;
Death, Sudden;
Electrocardiography;
Female;
Humans;
Jervell-Lange Nielsen Syndrome;
Long QT Syndrome*;
Mothers;
Reference Values;
Romano-Ward Syndrome;
Syncope*;
Wills
- From:Journal of the Korean Pediatric Society
2000;43(5):725-729
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital long QT syndrome (LQTS) is a disease characterized by prolongation of ventricle repolarization and by the occurrence, usually during emotional or physical stress, of life-threatening arrhythmias that lead to sudden death in most symptomatic and untreated patients. Two variants have been initially identified:the original Jervell and Lange-Nielsen syndrome of congenital deafness and autosomal recessive inheritance, and the more frequent Romano-Ward syndrome of autosomal dominant inheritance. Evidence also shows that approximately 25 to 30% of the cases are sporadic with syncope and a prolonged QT interval but without showing evidence for familial involvement. Familial and sporadic cases have been grouped under the definition of congenital long QT syndrome. We experienced a case of congenital long QT syndrome in a 13-year-old female girl. She had episodes of recurrent syncope and QT interval prolongation(QTc=0.46sec) in electrocardiogram(ECG). The ECG of her mother showed QT interval prologation(QTc=0.46sec). After applying atenolol, the QT interval returned to normal range and syncope has not occurred. We report a case of congenital long QT syndrome with a brief review of related literatures.
