Surgical Techniques in Management of Supravalvular Aortic Stenosis in Children

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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Avoidance of malignant arrhythmia caused by displacement of the right coronary artery ostium in surgical correction of supravalvular aortic stenosis

Journal of International Medical Research ◽

10.1177/0300060518818021 ◽

2019 ◽

Vol 47 (11) ◽

pp. 5702-5710

Author(s):

Bo Peng ◽

Qiang Wang

Keyword(s):

Coronary Artery ◽

Aortic Stenosis ◽

Right Coronary Artery ◽

Coronary Sinus ◽

Surgical Techniques ◽

Ecmo Support ◽

Supravalvular Aortic Stenosis ◽

Surgical Approaches ◽

Malignant Arrhythmia ◽

The Right

Objective This study was performed to identify the effects of different surgical approaches on the right coronary artery ostium in patients with congenital supravalvular aortic stenosis (SVAS) and to determine how to avoid surgically induced right coronary artery occlusion. Methods The surgical techniques and outcomes of 91 patients who underwent surgical treatment of SVAS from 2008 to 2015 in our institution were retrospectively reviewed to identify the causes of early death, reoperation, and extracorporeal membrane oxygenation (ECMO) support. Results Four perioperative deaths (Doty’s technique, n = 4), six cases of ECMO support (Doty’s technique, n = 4; Brom’s technique, n = 1; McGoon’s technique, n = 1), and eight reoperations (Doty’s technique, n = 5; Brom’s technique, n = 2; McGoon’s technique, n = 1) occurred. In cases of reoperation, adjustment of the shape and position of the right coronary sinus patch relieved the malignant arrhythmia. Conclusion Different surgical techniques have different effects on the right coronary artery. The shape of the patch inserted into the right coronary sinus should be carefully clipped to avoid distortion and ischemia of the right coronary artery.

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New Trends and Developments in Patients with Severe Aortic Stenosis – Towards Optimal Treatment?

European Cardiology Review ◽

10.15420/ecr.2009.5.2.81 ◽

2009 ◽

Vol 5 (2) ◽

pp. 81 ◽

Cited By ~ 2

Author(s):

Martijn WA van Geldorp ◽

Johanna JM Takkenberg ◽

Ad JJC Bogers ◽

A Pieter Kappetein ◽

◽

...

Keyword(s):

Aortic Stenosis ◽

Severe Aortic Stenosis ◽

Surgical Techniques ◽

Treatment Strategies ◽

Tissue Doppler ◽

Doppler Imaging ◽

Treatment Modalities ◽

Diagnostic Tools ◽

Number Of Patients ◽

Transcatheter Valve

Over the next few decades the number of patients diagnosed with aortic stenosis is expected to rise as the population ages and the use of several diagnostic tools expands. This will result in a growing need for both medical and surgical treatment and stimulate the development of new diagnostic and surgical techniques. This article briefly describes the prevalence, pathogenesis and clinical presentation of patients with aortic stenosis and focuses on developments in diagnostic tools, treatment strategies and treatment modalities: the use of echocardiography, tissue Doppler imaging, stress testing and biomarkers is discussed, as well as timing of surgery and the role microsimulation can play in prosthesis selection. Furthermore, newly developed transcatheter valve implantation techniques and their possible role in treating ‘inoperable’ or ‘elderly’ patients are discussed.

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Modeling Supravalvular Aortic Stenosis Syndrome With Human Induced Pluripotent Stem Cells

Circulation ◽

10.1161/circulationaha.112.116996 ◽

2012 ◽

Vol 126 (14) ◽

pp. 1695-1704 ◽

Cited By ~ 70

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

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Familial Supravalvular Aortic Stenosis

CHEST Journal ◽

10.1378/chest.70.4.494 ◽

1976 ◽

Vol 70 (4) ◽

pp. 494-500 ◽

Cited By ~ 10

Author(s):

Lewis W. Johnson ◽

Ronald A. Fishman ◽

Bernard Schneider ◽

Frederick B. Parker ◽

George Husson ◽

...

Keyword(s):

Aortic Stenosis ◽

Supravalvular Aortic Stenosis

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Supravalvular Aortic Stenosis and the Risk of Premature Death Among Patients With Homozygous Familial Hypercholesterolemia

The American Journal of Cardiology ◽

10.1016/j.amjcard.2020.12.080 ◽

2021 ◽

Author(s):

Ruiying Zhang ◽

Jinjie Xie ◽

Jie Zhou ◽

Liyuan Xu ◽

Yufan Pan ◽

...

Keyword(s):

Aortic Stenosis ◽

Familial Hypercholesterolemia ◽

Premature Death ◽

Supravalvular Aortic Stenosis ◽

Homozygous Familial Hypercholesterolemia

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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 ◽

10.1177/02676591211046876 ◽

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.

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