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Archives of Disease in Childhood 1998;78:159-162; doi:10.1136/adc.78.2.159
Copyright © 1998 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.
Arch Dis Child 1998;78:159-162 ( February )

Tuberous sclerosis complex and Wolff-Parkinson-White syndrome

F J K O'Callaghan,a A C Clarke,a H Joffe,c B Keeton,d R Martin,c A Salmon,d R D Thomas,b J P Osbornea

a Bath Unit for Research into Paediatrics, Children's Centre, Royal United Hospital, Combe Park, Bath BA1 3NG, b Department of Cardiology, Royal United Hospital, Bath, c Bristol Royal Hospital for Sick Children, Bristol, d Wessex Regional Cardiothoracic Unit, Southampton General Hospital, Southampton

Correspondence to: Dr Osborne.


Accepted 4 October 1997

This report highlights the association between tuberous sclerosis and Wolff-Parkinson-White syndrome. Ten patients with concurrent diagnoses of Wolff-Parkinson-White syndrome and tuberous sclerosis were identified. Wolff-Parkinson-White syndrome presented early in life, nine cases being diagnosed in the first year. Eight of the 10 cases were male. In eight cases, the syndrome was associated with supraventricular tachycardias, and in nine with cardiac rhabdomyomata. One child died from cardiac failure secondary to obstruction of the left ventricular outflow tract by a rhabdomyoma. Five of nine survivors showed resolution of Wolff-Parkinson-White syndrome on follow up. The accessory pathway was localised in nine patients from surface electrocardiograms: six children had left sided pathways and three had right sided pathways.

Keywords: tuberous sclerosis; cardiac rhabdomyoma; Wolff-Parkinson-White syndrome; supraventricular tachycardia


© 1998 by Archives of Disease in Childhood

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