Anesthesia for Adult Congenital Aortic Surgery

  • Barry D. Kussman
  • James A. DiNardo


Congenital supravalvular aortic stenosis (SVAS) is a rare form of left ventricular outflow tract (LVOT) obstruction that often is associated with peripheral pulmonary artery stenoses (approximately 40% of patients). Congenital SVAS is an elastin ­arteriopathy and is most commonly associated with Williams–Beuren syndrome. Williams–Beuren syndrome, commonly referred to as Williams syndrome (WS), is characterized by the presence of SVAS and peripheral pulmonary artery stenoses in association with mental retardation and distinctive elfin facies.


Aortic Arch Pulse Wave Velocity Subclavian Artery Patent Ductus Arteriosus Pulmonary Vascular Resistance 
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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Anesthesiology, Perioperative and Pain MedicineChildren’s Hospital Boston and Harvard Medical SchoolBostonUSA

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