A Case of Idiopathic Long QT Syndrome(LQTS)

Yun, Seong Min; Won, Jun Yeon; Kim, Young Dae; Jo, Yong Ho; Heu, Seong Ho; Lee, Du Ha; Lee, Sang Min; Jung, Toe Ho

Abstract

The idiopathic long-QT syndrome is an infrequent inherited disorder, characterized by prolonged QT interval and by the occurrence of life-threatening tachyarrhythmia, particularly in association with emotional or physical stree. In its characteristic presentation, with obvious QT prolongation and stree induced syncope with torsades de points, the diagnosis is straightforward for physicians aware of the disease. But sometimes in cases of borderline QT prolongation and vague symptom, a correct diagnosis is delayed and frequently misdiagnosed as a seizure disorder. The mortality of untreated symptomatic patients with LQTS exceeds 20% in the year after their first syncopal episode and aapproaches 50% within 10 years. But this high mortality rate has been significantly reduced by the use of pharmacological or surgical antiadrenergic therapy or both. So early detection of the disease and antiadrenergic treatment sympathetic innervation to the heart. Second, there was intrinsic gene abnormality in the mechanisms responsible for cardiac repolarization. We report one case of LQTS in a 24 years old female patient who had been suffering from stree related syncope. Her initial EKG had prologed QT interval(588msec) and increased QT intrval dispersion(200msec). In the Holter monitor, long and short cycle sequence induced (pause-dependent) torsades de pointes was detected. She has been treated by beta-blocker and has maintained her condition without recurrence of the symptoms.