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

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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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Reference

  1. Ashizawa T, Subramony SH (2001) What is Kearns-Sayre syndrome after all? Arch Neurol 58(7):1053–1054. doi:10.1001/archneur.58.7.1053

    Article  PubMed  CAS  Google Scholar 

  2. Berenberg RA, Pellock JM, DiMauro S, Schotland DL, Bonilla E, Eastwood A et al (1977) Lumping or splitting? “Ophthalmoplegia-plus” or Kearns-Sayre syndrome? Ann Neurol 1(1):37–54. doi:10.1002/ana.410010104

    Article  PubMed  CAS  Google Scholar 

  3. Charles R, Holt S, Kay JM, Epstein EJ, Rees JR (1981) Myocardial ultrastructure and the development of atrioventricular block in Kearns-Sayre syndrome. Circulation 63(1):214–219

    PubMed  CAS  Google Scholar 

  4. Gregoratos G, Abrams J, Epstein AE, Freedman RA, Hayes DL, Hlatky MA et al (2002) ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (ACC/AHA/NASPE committee to update the 1998 pacemaker guidelines). Circulation 106(16):2145–2161. doi:10.1161/01.CIR.0000035996.46455.09

    Article  PubMed  Google Scholar 

  5. Karanikis P, Korantzopoulos P, Kountouris E, Dimitroula V, Patsouras D, Pappa E et al (2005) Kearns-Sayre syndrome associated with trifascicular block and QT prolongation. Int J Cardiol 101(1):147–150. doi:10.1016/j.ijcard.2004.01.027

    Article  PubMed  Google Scholar 

  6. Katsanos KH, Pappas CJ, Patsouras D, Michalis LK, Kitsios G, Elisaf M et al (2002) Alarming atrioventricular block and mitral valve prolapse in the Kearns-Sayre syndrome. Int J Cardiol 83(2):179–181. doi:10.1016/S0167-5273(02)00040-2

    Article  PubMed  Google Scholar 

  7. Roberts NK, Perloff JK, Kark RA (1979) Cardiac conduction in the Kearns-Sayre syndrome (a neuromuscular disorder associated with progressive external ophthalmoplegia and pigmentary retinopathy). Report of 2 cases and review of 17 published cases. Am J Cardiol 44(7):1396–1400. doi:10.1016/0002-9149(79)90459-4

    Article  PubMed  CAS  Google Scholar 

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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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Authors and 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

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