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A Case of Congenital Long QT Syndrome with Reccurent Syncope

Journal of the Korean Pediatric Society 2000;43(5):725-729.
Published online May 15, 2000.
A Case of Congenital Long QT Syndrome with Reccurent Syncope
Jong Hwa Hwang, Hong Bae Kim
Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea
반복적 실신을 동반한 선천성 QT 간격 연장 증후군 1례
황종화, 김홍배
왈레스기념 침례병원 소아과
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.
Key Words: Congenital Long QT Syndrome, Syncope

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