Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

Taylor E. Katt, Robert L. Spicer, Anji T. Yetman, Ali N. Ibrahimiye, James M. Hammel, Jeffrey A. Robinson

Research output: Contribution to journalArticlepeer-review


Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.)

Original languageEnglish (US)
Pages (from-to)1716-1719
Number of pages4
JournalJACC: Case Reports
Issue number11
StatePublished - Sep 2020


  • aortic arch hypoplasia
  • congenital heart disease
  • genetic syndrome
  • pulmonary artery stenosis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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