scholarly journals Patch aortoplasty for supravalvular aortic stenosis with bicuspid aortic valve associated with Williams syndrome

10.5580/33 ◽  
2009 ◽  
Vol 13 (2) ◽  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Williams Syndrome ◽  
Bicuspid Aortic Valve ◽  
Supravalvular Aortic Stenosis

An infant with suspected missed diagnosis of Williams syndrome failed weaning off CPB after surgical correction of pulmonary stenosis: a case report and literature review

Perfusion ◽  
2021 ◽  
pp. 026765912110468
Author(s):  
Yuan Yuan ◽  
Ronghua Zhou
Keyword(s):  
Aortic Stenosis ◽  
Williams Syndrome ◽  
Myocardial Protection ◽  
Developmental Disorder ◽  
Pulmonary Stenosis ◽  
Surgical Correction ◽  
Supravalvular Aortic Stenosis ◽  
Cardiac Insufficiency ◽  
Valvular Stenosis ◽  
Missed Diagnosis

Williams syndrome (WS) is a rare congenital developmental disorder caused by the deletion of between 26 and 28 genes on chromosome 7q11.23. For patients with WS, in view of the particularity of the supravalvular aortic stenosis, choosing appropriate arterial cannula, maintaining higher perfusion pressure as well as strengthening myocardial protection during cardiopulmonary bypass (CPB) is essential to the clinical outcome. Here, we report a child with pulmonary artery valvular stenosis who failed to wean off CPB because of malignant arrhythmias and cardiac insufficiency after surgical correction of pulmonary valvular stenosis. With the assistance of extracorporeal membrane oxygenation (ECMO), emergency cardiac catheterization revealed supravalvular aortic stenosis (SVAS), which suggests a suspected missed diagnosis of WS. Finally, under the support of ECMO, the cardiac function gradually returned to normal, and the child was discharged 23 days after surgery.

Left ventricular outflow tract obstruction (LVOTO)

2011 ◽  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Bicuspid Aortic Valve ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
Left Ventricular Outflow ◽  
Different Levels

Introduction 68Subvalvar aortic stenosis (AS) 70Bicuspid aortic valve 72Supravalvar AS 74LVOTO may occur at different levels: • Subvalvular.• Valvular—including bicuspid aortic valve.• Supravalvular.• Coarctation— see p.118.Effects of LVOTO, irrespective of site of lesion, are: • ↑ afterload on LV....

scholarly journals Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis

1998 ◽  
Vol 45 (12) ◽  
pp. 1203-1206 ◽  
Author(s):  
Shinji Kawahito ◽  
Hiroshi Kitahata ◽  
Hideyuki Kimura ◽  
Katsuya Tanaka ◽  
Yoko Sakai ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Williams Syndrome ◽  
Anaesthetic Management ◽  
Supravalvular Aortic Stenosis
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