C Stamm1, J Li, S Y Ho, A N Redington, R H Anderson. 1. Section of Pediatrics, National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom.
Abstract
OBJECTIVE: We aimed to elucidate the structural basis of supravalvular aortic stenosis in the context of its surgical repair. METHODS: We examined retrospectively the angiograms and echocardiograms of 37 patients and compared them with those of control groups. Additionally, we studied 8 pathologic specimens. RESULTS: Partial adhesion of the leaflets to the stenosing ridge was observed in 54% of the cases and the leaflets were thickened and less mobile in 30%. Forty-five percent of the angiograms showed evidence of coronary orificial stenosis. The sinuses of Valsalva were significantly enlarged in 75% of the cases. Changes in dimensions of the aortic root were demonstrated more clearly by angiography than by echocardiography. In all anatomic specimens, a marked redundancy of the leaflets was observed and quantified. CONCLUSIONS: Our data demonstrate that the entire valvular apparatus is always affected by the so-called supravalvular stenosis. Anatomic restoration of the aortic root should ideally take into account all of the deformed components by enlarging all three sinuses of Valsalva at the sinotubular junction.
OBJECTIVE: We aimed to elucidate the structural basis of supravalvular aortic stenosis in the context of its surgical repair. METHODS: We examined retrospectively the angiograms and echocardiograms of 37 patients and compared them with those of control groups. Additionally, we studied 8 pathologic specimens. RESULTS: Partial adhesion of the leaflets to the stenosing ridge was observed in 54% of the cases and the leaflets were thickened and less mobile in 30%. Forty-five percent of the angiograms showed evidence of coronary orificial stenosis. The sinuses of Valsalva were significantly enlarged in 75% of the cases. Changes in dimensions of the aortic root were demonstrated more clearly by angiography than by echocardiography. In all anatomic specimens, a marked redundancy of the leaflets was observed and quantified. CONCLUSIONS: Our data demonstrate that the entire valvular apparatus is always affected by the so-called supravalvular stenosis. Anatomic restoration of the aortic root should ideally take into account all of the deformed components by enlarging all three sinuses of Valsalva at the sinotubular junction.
Authors: Karuna M Das; Tarek S Momenah; Sven G Larsson; Shehla Jadoon; Abdullah S Aldosary; Edward Y Lee Journal: Pediatr Cardiol Date: 2014-08-20 Impact factor: 1.655
Authors: Constantinos A Contrafouris; Andrew C Chatzis; Meletios A Kanakis; Prodromos A Azariadis; Fotios A Mitropoulos Journal: Case Rep Surg Date: 2014-03-11