How we approach peripheral pulmonary stenosis in Williams-Beuren syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:118-21
Date
04/08/2009Pubmed ID
19349025DOI
10.1053/j.pcsu.2009.01.012Scopus ID
2-s2.0-63649100285 (requires institutional sign-in at Scopus site) 15 CitationsAbstract
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.
Author List
Gandy KL, Tweddell JS, Pelech ANMESH terms used to index this publication - Major topics in bold
Cardiac Surgical ProceduresHumans
Pulmonary Valve Stenosis
Williams Syndrome
