01.01.33 Left heart obstruction at multiple sites (including Shone syndrome)

IPCCC Term Left heart obstruction at multiple sites (including Shone syndrome)

IPCCC Code 01.01.33


Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there are deep, endothelial lined, blind-ended intramyocardial tunnels communicating with the left ventricular chamber. These occur in the setting of aortic atresia or severe left ventricular outflow tract obstruction, intact ventricular septum and a patent mitral valve. [restabs alignment=”osc-tabs-right” pills=”nav-pills” responsive=”true” icon=”true” text=”More”][restab title=”Anatomic Specimen” active=”active”]

01-01-33 Left heart obstruction at multiple sites (including Shone syndrome) This heart has been sectioned along the long axis and is viewed from the left posterior lateral aspect. The left side of the heart demonstrates the features of Shone’s syndrome. There is marked endocardial fibroelastosis of the left atrium with a supravalvar mitral ring (red dots). The mitral valve is extremely stenotic and thickened with fused tendinous cords. There is a parachute mitral valve with a solitary papillary muscle. There is subvalvar aortic stenosis caused by a prominent fibrous ridge that was circumferential along with a prominent, subaortic, septal bulge. At the sinutubular junction there is a supravalvar ridge accounting for the supravalvar stenosis. The aortic valve is bicuspid.
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