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Growth of the aorta in children with Williams syndrome: does surgery make a difference?

R F English1, S D Colan, P M Kanani

  • 1Division of Pediatric Cardiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA. robert.english@chp.edu

Pediatric Cardiology
|February 6, 2004
PubMed

Insights

Children with Williams syndrome often have aortic arch hypoplasia. Aortoplasty can reduce the aortic gradient but does not significantly improve aortic size in the short term for these patients.

Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Genetics

Background:

  • Williams syndrome is a genetic disorder characterized by supravalvular aortic stenosis (SAS) and aortic arch hypoplasia.
  • The impact of surgical intervention, specifically aortoplasty, on the growth of the hypoplastic aorta in Williams syndrome remains unclear.

Purpose of the Study:

  • To investigate the effect of aortoplasty on aortic growth in children with Williams syndrome and SAS.
  • To test the hypothesis that aortic growth remains deficient regardless of aortoplasty.

Main Methods:

  • Retrospective review of 14 pediatric patients with Williams syndrome and SAS.
  • Division into two groups: those who underwent aortoplasty and those who did not.
  • Echocardiographic measurements of aortic dimensions and velocity converted to zeta scores for comparison over time.

Main Results:

  • Patients undergoing aortoplasty showed a significant decrease in peak aortic velocity post-surgery.
  • No significant differences in aortic dimensions (annulus, ascending aorta, transverse aorta, isthmus) were observed between groups preoperatively.
  • Postoperatively, aortic dimensions showed no significant change in size over a mean follow-up of 43 months.

Conclusions:

  • Aortic arch hypoplasia in Williams syndrome appears to be a constant feature.
  • Aortoplasty effectively reduces the aortic gradient but does not promote short-term growth of the ascending aorta, transverse aorta, or aortic isthmus.

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