| A Case of Idiopathic Long QT Syndrome with 2:1 Atrioventricular Block |
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Kwang In Lee, Chul Ha Kim, In Seok Lim, Dong Keun Lee, Byoung Hoon Yoo |
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Department of Pediatrics, College of Medicine, Chung-Ang University, Seoul, Korea |
| 2:1 방실 차단을 동반한 특발성 QT 간격 연장 증후군 1례 |
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이광인, 김철하, 임인석, 이동근, 유병훈 |
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중앙대학교 의과대학 소아과학교실 |
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| Abstract |
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The idiopathic long QT syndrome is a very rare disorder in which affected individuals have an unusual electrocardiographic repolarization abnormality (QT prolongation) and a propensity to syncope and fatal ventricular arrhythmia.
Since Jervell and Lange-Nielsen first described this syndrome in 1957, progression has been made in the pathophysiology and in the treatment. Sinus bradycardia may be associated with this syndrome sometimes, but abnormalities in atrioventricular conduction are very rare.
We experienced a case of idiopathic long QT syndrome with 2:1 atrioventricular block in a 3 day old male neonate. He was transferred with chief complaints of central cyanosis and bradycardia. The electrocardiography showed prolonged QTc and 2:1 atriventricular block. Pacemaker was implanted, but he died of ventricular arrhythmia 2 days later.
We report a case of idiopathic long QT syndrome with a brief review of related literatures. |
| Key Words:
Idiopathic Long QTc, Atrioventricular block |
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