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KMID : 0367419930360091236
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Journal of Korean Pediatric Society
1993 Volume.36 No. 9 p.1236 ~ p.1244
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A Clinical Survey of Prolonged Q-T Syndrome among Korean Children with Congenital Deafness
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이강우
김경범/손창성/이주원/독고영창
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Abstract
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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≥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.
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KEYWORD
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