09.16.18 Congenital supravalvar aortic stenosis

IPCCC Term Congenital supravalvar aortic stenosis

IPCCC Code 09.16.18


Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation with narrowing of the aorta at the level of the sinotubular junction which may extend into the ascending aorta.

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[su_carousel source=”media: 4986,4987″ limit=”27″ width=”460″ height=”510″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no”] Image # 1 is an anterior superior view of the aortic root as it exits the ventricular mass there is marked dilation of the sinuses of Valsalva secondary to a fibrous membrane present internally at the level of the sinotubular junction (red dots).In image # 2 the left ventricular outflow tract and the aortic root shows subvalvar and supravalvar aortic stenosis in this patient noted to have Shone’s syndrome. The subvalvar stenosis is caused by a prominent fibrous ridge that is circumferential and extends over the aorta to mitral fibrous continuity. At the sinotubular junction (red dots) there is a fibrous shelf accounting for the distal valvar stenosis. Although this is the lesion that is commonly defined as being “supravalvar”, it is in fact a valvar lesion since the “commissures”, or the peripheral attachments of the leaflets, are part of the valvar mechanism. The sinuses of Valsalva are markedly dilated (not seen in this image) with the right and non-adjacent leaflets tethered to the fibrous shelf.

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