| A Clinical Survey of Prolonged Q-T Syndrome among Korean Children with Congenital Deafness |
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Kang Woo Lee, Kyung Bum Kim, Chang Sung Son, Joo Won Lee, Young Chang Tockgo |
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Department of Pediatrics, Korea University, College of Medicine, Seoul, Korea |
| 농아에서의 Long Q-T Syndrome에 관한 임상적 고찰 |
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이강우, 김경범, 손창성, 이주원, 독고영창 |
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고려대학교 의과대학 소아과학교실 |
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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 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. |
| Key Words:
Long Q-Tc, Deafness |
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