Reduced heart rate variability in patients affected with Rett syndrome. A possible explanation for sudden death

Neuropediatrics. 1999 Jun;30(3):146-8. doi: 10.1055/s-2007-973480.


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*