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Rapid development of life-threatening complete atrioventricular block in Kearns-Sayre syndrome

European Journal of Pediatrics volume 168, pages 757–759 (2009)Cite this article

Abstract

Kearns-Sayre syndrome is a rare mitochondrial disorder with defined diagnostic criteria. Knowledge of these diagnostic criteria and early diagnosis are important to ensure periodic electrocardiograms for identification of cardiac conduction disorders, which are the most important prognostic factor of the disease. We report on a 9-year-old girl with rapid development of a life-threatening complete atrioventricular block within 10 months and discuss the importance and time interval of regular electrocardiograms. Our patient survived by placing a temporary transvenous pacemaker lead followed by permanent pacemaker implantation a few days later.

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Acknowledgement

This case report was developed without any funding or grants. No author has any conflict of interest.

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Affiliations

  1. Department of Neonatology and Paediatric Intensive Care, University Hospital of Cologne, Children’s Hospital, Kerpener Str. 62, 50937, Cologne, Germany

    L. Welzing, A. Kribs, F. Eifinger & C. Huenseler

  2. Department of Paediatric Neurology, University Hospital of Cologne, Children’s Hospital, Cologne, Germany

    J. C. von Kleist-Retzow

  3. Department of Paediatric Cardiology, University Hospital of Cologne, Cologne, Germany

    N. Sreeram

Authors
  1. L. Welzing
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  2. J. C. von Kleist-Retzow
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  3. A. Kribs
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  4. F. Eifinger
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  5. C. Huenseler
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  6. N. Sreeram
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Correspondence to L. Welzing.

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Welzing, L., von Kleist-Retzow, J.C., Kribs, A. et al. Rapid development of life-threatening complete atrioventricular block in Kearns-Sayre syndrome. Eur J Pediatr 168, 757–759 (2009). https://doi.org/10.1007/s00431-008-0831-x

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  • DOI: https://doi.org/10.1007/s00431-008-0831-x

Keywords

  • Kearns-Sayre syndrome
  • Periodic electrocardiogram
  • Complete atrioventricular block
  • Pacemaker implantation