How We Approach Peripheral Pulmonary Stenosis in Williams-Beuren Syndrome

Kim L. Gandy, James S. Tweddell, Andrew N Pelech

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

Williams-Beuren syndrome is associated with supravalvar aortic stenosis and peripheral pulmonary artery stenosis in the majority of affected individuals. Among patients in whom surgery for supravalvar aortic stenosis is contemplated, stenosis of the branch pulmonary arteries is common. For asymptomatic patients with subsystemic right ventricular pressure, the natural history is favorable and no intervention is necessary. For patients with important proximal branch pulmonary artery, stenoses patch arterioplasty can be accomplished at the time of surgery for supravalvar aortic stenosis. For patients with important peripheral pulmonary stenosis, preoperative catheter-based therapy is indicated. Surgical repair of peripheral pulmonary stenosis is indicated when preoperative intervention is unsuccessful and can include a combination of patch arterioplasty as well as intraoperative application of catheter-based techniques. This report will address the approach to this lesion with catheter-based approaches, surgical approaches, and their combination.

Original languageEnglish (US)
Pages (from-to)118-121
Number of pages4
JournalSeminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
Volume12
Issue number1
DOIs
StatePublished - 2009
Externally publishedYes

Keywords

  • Catheter intervention
  • Elastin arteriopathy
  • Pulmonary artery stenosis
  • Surgery for congenital heart disease

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery

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