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a Western Sydney
Genetics Program, The Royal Alexandra Hospital for Children, PO Box
3515 Parramatta, Westmead, Sydney, NSW 2124, Australia, b Adolph Basser Cardiac Institute, The
Royal Alexandra Hospital for Children, c Department of Paediatrics and Child Health,
University of Sydney, Sydney 2006, Australia, d The
TVW Telethon Institute for Child Health Research, West Perth 6827, Australia
Correspondence to: Correspondence to: Professor Christodoulou. email: Johnc{at}nch.edu.au
Accepted 18 December 1998
Rett syndrome is a severe neurodevelopmental disorder of
unknown aetiology. A prolonged QT interval has been described
previously in patients with Rett syndrome. To investigate QT
prolongation and the presence of cardiac tachyarrhythmias in Rett
syndrome electrocardiography and 24 hour Holter monitoring were
performed prospectively in a cohort of 34 girls with Rett syndrome. The corrected QT value was prolonged in nine patients. Compared with a
group of healthy controls of a similar age range, the patients with
Rett syndrome had significantly longer corrected QT values. Clinical
severity was not a predictor for prolonged QT intervals in the Rett
syndrome cohort. The prolonged QT syndrome is a serious and potentially
lethal cardiac disorder and should be considered in all girls with Rett syndrome.
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