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.
MeSH terms
-
Adolescent
-
Autonomic Nervous System / physiopathology
-
Autonomic Nervous System Diseases / diagnosis
-
Autonomic Nervous System Diseases / genetics
-
Autonomic Nervous System Diseases / mortality
-
Autonomic Nervous System Diseases / physiopathology
-
Cause of Death
-
Child
-
Child, Preschool
-
Death, Sudden, Cardiac / etiology*
-
Electrocardiography
-
Female
-
Heart Conduction System / physiopathology
-
Heart Rate / physiology*
-
Humans
-
Long QT Syndrome / diagnosis
-
Long QT Syndrome / genetics
-
Long QT Syndrome / mortality
-
Long QT Syndrome / physiopathology
-
Rett Syndrome / diagnosis
-
Rett Syndrome / genetics
-
Rett Syndrome / mortality
-
Rett Syndrome / physiopathology*