Strategies for the Surgical Management of Highly Aggressive Williams Syndrome Aortopathy: A Three Case Report

Pediatr Cardiol. 2024 Jan;45(1):208-212. doi: 10.1007/s00246-023-03383-8. Epub 2023 Dec 17.


Severe aortopathy in Williams syndrome can sometimes present with an initial ascending aortic pathology, followed in short order by more distal multilevel obstruction and recurrence requiring reintervention. In this series, an early, comprehensive surgical approach using a combination of various access and perfusion strategies yielded excellent long-term results.

Keywords: Aortopathy; Supravalvar aortic stenosis; Thoracic aortic stenosis; Williams syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aorta / surgery
  • Humans
  • Williams Syndrome* / complications
  • Williams Syndrome* / surgery