Numerical Analysis of Stenoses Severity and Aortic Wall Mechanics in Patients with Supravalvular Aortic Stenosis

2021 ◽  
pp. 104573
Author(s):  
Talha Lone ◽  
Angelica Alday ◽  
Rana Zakerzadeh
Keyword(s):  
Numerical Analysis ◽  
Aortic Stenosis ◽  
Aortic Wall ◽  
Supravalvular Aortic Stenosis

scholarly journals Modeling Supravalvular Aortic Stenosis Syndrome With Human Induced Pluripotent Stem Cells

Circulation ◽  
2012 ◽  
Vol 126 (14) ◽  
pp. 1695-1704 ◽  
Author(s):  
Xin Ge ◽  
Yongming Ren ◽  
Oscar Bartulos ◽  
Min Young Lee ◽  
Zhichao Yue ◽  
...  
Keyword(s):  
Stem Cells ◽  
Aortic Stenosis ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Supravalvular Aortic Stenosis ◽  
Induced Pluripotent

Familial Supravalvular Aortic Stenosis

CHEST Journal ◽  
1976 ◽  
Vol 70 (4) ◽  
pp. 494-500 ◽  
Author(s):  
Lewis W. Johnson ◽  
Ronald A. Fishman ◽  
Bernard Schneider ◽  
Frederick B. Parker ◽  
George Husson ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
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.

scholarly journals Iatrogenic Supravalvular Aortic Stenosis

Aorta ◽  
2016 ◽  
Vol 04 (05) ◽  
pp. 172-174 ◽  
Author(s):  
Paolo Bosco ◽  
Antonella Ferrara ◽  
Samer Nashef
Keyword(s):  
Aortic Stenosis ◽  
Aortic Wall ◽  
Rare Complication ◽  
Suture Line ◽  
Anastomotic Site ◽  
Resonance Imaging ◽  
Sandwich Technique ◽  
Type A Aortic Dissection ◽  
Preoperative Magnetic Resonance ◽  
Aortic Anastomosis

AbstractWe describe a case of hemolytic anemia and proximal anastomotic site stenosis following emergency repair of a Type A aortic dissection. This rare complication led to a reoperation to correct the iatrogenic aortic stenosis and cure the consequent hemolysis. A “sandwich” technique (with two Teflon strips on the outside and inside of the aortic wall) was used in the initial repair to reinforce the suture line and prevent bleeding from the aortic anastomoses. At the time of reoperation, the inner Teflon strip at the proximal aortic anastomosis was found to have inverted into the aortic lumen, as suggested by the preoperative magnetic resonance imaging. Surgical treatment consisted of resecting the portion of inner Teflon that had turned in and tacking the remaining part back onto the aortic wall. The observed hemolysis was likely due to the turbulent flow associated with the supra-aortic stenosis and the collision of red cells with the internal Teflon strip. The patient made an uncomplicated recovery with no further hemolysis and was discharged on postoperative day 8.

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis

2021 ◽  
pp. 1-8
Author(s):  
Mehmet Biçer ◽  
Mehmet Dedemoğlu ◽  
Oktay Korun ◽  
Hüsnü F. Altın ◽  
Okan Yurdakök ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Surgical Repair ◽  
Pulmonary Stenosis ◽  
Surgical Techniques ◽  
Supravalvular Aortic Stenosis ◽  
Surgical Reconstruction ◽  
Residual Stenosis ◽  
Post Operative Period ◽  
Surgical Methods

Abstract Background: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. Methods: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. Results: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0–9.9) years. Ten (34.5%) patients had Williams–Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7–7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). Conclusion: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

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