IPCCC Term Muscular outlet ventricular septal defect with posteriorly malaligned outlet septumIPCCC Code 07.11.16ICD-11 Code PENDINGSynonyms PENDINGAbbreviations PENDINGIPCCC Definition A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle, with exclusively muscular borders when viewed from the right ventricle, and in which the muscular outlet septum is malaligned in postero-caudal fashion relative to the apical part of the muscular septum such that there is obstruction to the left ventricular outflow tract.

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This heart has been sectioned along the long axis and is viewed from the left posterior lateral aspect. A ventricular septal defect (interventricular communication) opens to the outlet of the right ventricle, with exclusively muscular borders. The muscular outlet septum is malaligned in postero-caudal fashion relative to the apical part of the muscular septum causing obstruction to the left ventricular outflow tract.

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IPCCC Term Outlet ventricular septal defect

IPCCC Code 07.12.00

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle. [restabs alignment=”osc-tabs-right” pills=”nav-pills” responsive=”true” icon=”true” text=”More”][restab title=”Anatomic Specimen” active=”active”]

In this anterior view, the free wall of the right ventricle has been lifted away from the septal surface demonstrating a perimembranous ventricular septal defect (interventricular communication). The defect extends into the outlet component of the right ventricle without malalignment of the outlet septum. The yellow dots mark the fibrous continuity between the tricuspid and aortic valves, rendering this defect perimembranous.

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IPCCC Term Muscular trabecular ventricular septal defect

IPCCC Code 07.11.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is a ventricular septal defect within the apical component of the muscular ventricular septum.

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The free wall of this hypertrophied right ventricle has been lifted rightward to expose the outlet component and is viewed anteriorly. There is a muscular ventricular septal defect (interventricular communication) that opens to the anterior part of the right ventricle, and is antero-superior (or cephalad) to the body of the septomarginal trabeculation, or septal band.

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

IPCCC Code 01.01.33

ICD-11 Code PENDING

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”]

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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IPCCC Term Congenital left ventricular outflow tract obstruction

IPCCC Code 07.09.28

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac condition in which the flow through the left ventricular outflow tract (proximal to the valve[s] guarding the outflow from the left ventricle) is blocked or impeded.

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This heart was sectioned along the long axis of the heart and is viewed from left posterior lateral aspect. The left side of the heart demonstrates subaortic stenosis secondary to asymmetric septal hypertrophy in this patient with hypertrophic cardiomyopathy.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram.[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography.[/restab][restab title=”Angiography”]Content for Angiography.[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos.[/restab][/restabs]