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

Francesca Guideri; M. Acampa; C. Hayek; M. Zappella; T. Di Perri

doi:10.1055/s-2007-973480

Subscribe to RSS

Please copy the URL and add it into your RSS Feed Reader.

https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000041.xml

Share / Bookmark

Facebook X Linkedin Weibo

Download PDF

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

Short communications

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

Further Information

Publication History

Publication Date:
12 March 2007 (online)

PDF Download Permissions and Reprints

Abstract

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.

Key words

Rett syndrome - Heart rate variability - Sudden death - QTc

>