Neuropediatrics 1999; 30(3): 146-148
DOI: 10.1055/s-2007-973480
Short communications

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Reduced Heart Rate Variability in Patients Affected with Rett Syndrome. A Possible Explanation for Sudden Death

Francesca Guideri1 , M. Acampa1 , C. Hayek2 , M. Zappella2 , T. Di Perri1
  • 1Institute of Clinical Medicine, School of Medicine, University of Siena, Siena, Italy
  • 2Department of Child Neurology and Psychiatry, University of Siena, Siena, Italy
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Publication History

Publication Date:
12 March 2007 (online)


Incidence of sudden death in Rett syndrome is greater than that of the general population and cardiac electrical instability is a prime suspect cause. Our study shows that girls with Rett syndrome had significantly lower heart rate variability (marker of autonomic disarray) and longer corrected QT intervals compared with an age-matched group of healthy girls. These abnormalities increased with advancing stages of the syndrome. These findings suggest a possible role of cardiac dysfunction in the sudden death associated with Rett syndrome.