Electrocardiographic findings in Rett syndrome: an explanation for sudden death?

J Pediatr. 1994 Jul;125(1):80-2. doi: 10.1016/s0022-3476(94)70128-8.


Girls with Rett syndrome had significantly longer corrected QT intervals (p < 0.001) and more T-wave abnormalities (p < 0.001) than were found in age-matched healthy girls. With advancing stages of the syndrome, the proportion of corrected QT interval prolongations and T-wave changes increased. The findings suggest a possible cardiac basis for sudden, unexpected death in Rett syndrome.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Death, Sudden / etiology*
  • Electrocardiography*
  • Female
  • Heart Diseases / complications
  • Heart Diseases / diagnosis
  • Humans
  • Rett Syndrome / complications
  • Rett Syndrome / physiopathology*