J Korean Pediatr Soc.
1993 Sep;36(9):1236-1244.
A clinical survey of prolonged Q-T syndrome among Korean children with congenital deafness
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 symptom complex is accompanied by congenital deafness, it is called Jervell and Lange-Nieisen syndrome. This study was undertaken to investigate the 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> or =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 4.6+/-1.1 per 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 control group. A larger survey is needed to derive more statistically significant conclusions.
