Atresia of the proximal left coronary artery associated with supravalvular aortic stenosis

AbstractSupravalvular aortic stenosis is associated with the Williams–Beuren syndrome, but it also occurs in a non-syndromatic congenital form. Anelastingene mutation of chromosome 7q11.23 is responsible in both cases. The vascular features are identical. These patients have a higher risk of sudden death, particularly when undergoing diagnostic or surgical procedures. We report the account of a family with a new mutation in theelastingene. Screening over three generations revealed eight affected individuals. The cardiac and vascular malformations ranged from mild asymptomatic supravalvular aortic stenosis and isolated dysplastic atrioventricular valves to diffuse arterial hypoplasia. Two infants presented arteries affected at multiple locations, including the left coronary artery. Both died of sudden cardiac death and myocardial ischaemia, one while under general anaesthesia for cardiac catheterisation, and the other perioperatively. We discuss the pathophysiological aspects in these patients that deserve consideration before any general anaesthesia is administered.

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Congenital Aneurysm of the Left Ventricle Associated With Supravalvular Aortic Stenosis and Aneurysm of the Left Main Coronary Artery: Case Report and Review of the Literature

Angiology ◽

10.1177/000331977502600304 ◽

1975 ◽

Vol 26 (3) ◽

pp. 269-275 ◽

Cited By ~ 10

Author(s):

Mohinder P. Gupta ◽

Samuel Zoneraich ◽

Agop Aintablain ◽

J. Mehta

Keyword(s):

Case Report ◽

Coronary Artery ◽

Left Ventricle ◽

Aortic Stenosis ◽

Left Main Coronary Artery ◽

Supravalvular Aortic Stenosis ◽

Review Of The Literature ◽

Left Main

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Surgical management of left coronary artery ostial atresia and supravalvar aortic stenosis

The Annals of Thoracic Surgery ◽

10.1016/0003-4975(92)91031-4 ◽

1992 ◽

Vol 54 (4) ◽

pp. 779-781 ◽

Cited By ~ 29

Author(s):

Eliot R. Rosenkranz ◽

Daniel J. Murphy ◽

Delos M. Cosgrove

Keyword(s):

Coronary Artery ◽

Aortic Stenosis ◽

Surgical Management ◽

Left Coronary Artery ◽

Supravalvar Aortic Stenosis

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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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