All issues > Volume 38(12); 1995

Case Report

J Korean Pediatr Soc. 1995;38(12):1701-1705. Published online December 15, 1995.

A Case of Idiopathic Long QT Syndrome with 2:1 Atrioventricular Block

Kwang In KI Lee¹, Chul Ha CH Kim¹, In Seok IS Lim¹, Dong Keun DK Lee¹, Byoung Hoon BH Yoo¹

¹Department of Pediatrics, College of Medicine, Chung-Ang University, Seoul, Korea

Abstract

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.

Keywords :Idiopathic Long QTc, Atrioventricular block