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Arch Dis Child 70:512-514 doi:10.1136/adc.70.6.512
  • Research Article

Coarctation of the aorta in Kabuki syndrome.

  1. H E Hughes,
  2. S J Davies
  1. Institute of Medical Genetics, University Hospital of Wales, Heath Park, Cardiff.

      Abstract

      The incidence of congenital heart defects in patients with Kabuki syndrome is estimated to be about 30%. To date, no specific type of heart malformation is known to be associated with the syndrome. A further 20 unselected children with Kabuki syndrome are presented. The incidence of heart abnormalities in these children is almost twice that previously reported (55%) and juxta-ductal coarctation occurs with a frequency of 25%.