Tuberous sclerosis complex and WolV-Parkinson-White syndrome

This report highlights the association between tuberous sclerosis and WolVParkinson-White syndrome. Ten patients with concurrent diagnoses of WolVParkinson-White syndrome and tuberous sclerosis were identified. WolV-ParkinsonWhite 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 WolVParkinson-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. (Arch Dis Child 1998;78:159–162)