A Case of Congenital Long QT Syndrome Associated with Deafness and Syncope.
10.4070/kcj.1998.28.11.1882
- Author:
Seon Mee LEE
;
Chung Whee CHOE
;
Heung Sun KANG
;
Kown Sam KIM
;
Jung Sang SONG
;
Jong Hwa BAE
- Publication Type:Case Report
- Keywords:
Congenital long QT syndrome;
Jervell and Lange-Nielsen syndrome
- MeSH:
Adult;
Arrhythmias, Cardiac;
Child;
Deafness*;
Death, Sudden;
Death, Sudden, Cardiac;
Female;
Humans;
Incidence;
Jervell-Lange Nielsen Syndrome;
Life Expectancy;
Long QT Syndrome*;
Seizures;
Syncope*;
Tachycardia, Ventricular;
Unconsciousness;
Wills
- From:Korean Circulation Journal
1998;28(11):1882-1888
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital long QT syndrome (LQTS) is an inherited disease characterized by prolonged QT intervals and polymorphic ventricular tachycardia. The clinical manifestations vary from sudden cardiac death by ventricular arrhythmia to asymptom throughout life. In 1957, Jervell and Lange-Nielsen reported a syndrome of congen-ital sensory deafness associated with a prolonged QT interval in four children. The affected children had multiple syncopal episodes, and three died suddenly. The mode of inheritance is autosomal recessive. Affected persons are susceptible to recurrent syncope, and they have a high incidence of sudden death and short life expectancy. We report a case and review the literature on long QT syndrome diagnosed in a 30-year-old female with a history of convulsion and loss of consciousness during delivery.
