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Congenital Long QT Syndrome and 2:1 Atrioventricular Block with a Mutation of the SCN5A Gene

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Infants with congenital long QT syndrome (LQTS) and 2:1 atrioventricular block (AVB) have been recognized as a clinical subset of children with LQTS. However, the genotype of this disorder is not well-known. We report an infant with LQTS and 2:1 AVB with a mutation of the SCN5A gene (LQT3). In some patients with LQTS and 2:1 AVB, the disorder may be due to mutation of the SCN5A gene (LQT3).

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Authors and Affiliations

  1. Department of Cardiology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono, Kiyose shi, Tokyo Japan, 204 8567, , , , , , JP

    M. Miura

  2. Department of Pediatrics, Keio University Hospital, Tokyo, Japan, , , , , , JP

    H. Yamagishi & Y. Morikawa

  3. Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan, , , , , , JP

    R. Matsuoka

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  1. M. Miura
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  2. H. Yamagishi
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  3. Y. Morikawa
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  4. R. Matsuoka
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Miura, M., Yamagishi, H., Morikawa, Y. et al. Congenital Long QT Syndrome and 2:1 Atrioventricular Block with a Mutation of the SCN5A Gene . Pediatr Cardiol 24, 70–72 (2003). https://doi.org/10.1007/s00246-002-0169-5

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  • DOI: https://doi.org/10.1007/s00246-002-0169-5

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