All issues > Volume 43(5); 2000

Case Report

J Korean Pediatr Soc. 2000;43(5):725-729. Published online May 15, 2000.

A Case of Congenital Long QT Syndrome with Reccurent Syncope

Jong Hwa JH Hwang¹, Hong Bae HB Kim¹

¹Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea

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.

Keywords :Congenital Long QT Syndrome, Syncope