title: Reduced heart rate variability in patients affected with Rett syndrome. A possible explanation for sudden death.
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.
authors.affiliation: Institute of Clinical Medicine, School of Medicine, Siena, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Guideri
authors.forename: M
authors.initials: M
authors.lastname: Acampa
authors.forename: G
authors.initials: G
authors.lastname: Hayek
authors.forename: M
authors.initials: M
authors.lastname: Zappella
authors.forename: T
authors.initials: T
authors.lastname: Di Perri
mesh_terms: D000293:Adolescent; D001341:Autonomic Nervous System / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000503:physiopathology; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D005260:Female; D006329:Heart Conduction System / Q000503:physiopathology; D006339:Heart Rate / Q000502:physiology*; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000503:physiopathology; D015518:Rett Syndrome / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000503:physiopathology*