All issues > Volume 36(9); 1993

Original Article

J Korean Pediatr Soc. 1993;36(9):1236-1244. Published online September 15, 1993.

A Clinical Survey of Prolonged Q-T Syndrome among Korean Children with Congenital Deafness

Kang Woo KW Lee¹, Kyung Bum KB Kim¹, Chang Sung CS Son¹, Joo Won JW Lee¹, Young Chang YC Tockgo¹

¹Department of Pediatrics, Korea University, College of Medicine, Seoul, Korea

Abstract

The clinical symptom complex characterized by syncopal attacks and sudden death in patients with electrocardiographic anomalies, especially a prolonged Q-t interval is known as the Romano-Ward syndrome. When a similar syptom complex is accompanied by congenital deafness, it is called Jervell and Lange-Nieisen syndrome. This study was underataken to investigate the incidence of the latter syndrome in the Seoul area. A total of 1,013 children with congenital deafness who attend schools for the deaf were studied by analyzing their electrocardiograms. A corrected Q-T interval of ≥0.44 seconds was defined as a prolonged Q-T interval. The overall incidence of long Q-T syndrome observed in the deaf in the Seoul area was 0.49% as compared to 0.25% reported from elsewhere in the world. The mean age of patients with long Q-T syndrome was 11.3±5.9 years and the first syncopal attacks was 4.±1.1per patient. A valsalva maneuver and exercise tests in the test group were associated with significant changes in T wave configuration and prolonged Q-T intervals while similar changes were not observed in the contol group. A larger survey is needed to derive more statistically significant conclusions.

Keywords :Long Q-Tc, Deafness