IPCCC Term CONGENITAL GIANT RIGHT ATRIUM

IPCCC Code 05.01.12

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which the right atrium is severely dilated. This is an isolated finding not secondary to abnormalities of the tricuspid valve or right ventricle.[restabs alignment=”osc-tabs-right” pills=”nav-pills” responsive=”true” icon=”true” text=”More”][restab title=”Anatomic Specimen” active=”active”]

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Image # 1 demonstrates he anterior anatomic view of a grossly dilated right atrium. Image # 2 shows the heart an anterior view of the heart with an opened right atrium, demonstrating marked dilation of the appendage and atrial chamber. The oval foramen is probe patent without appreciable dilation of the flap valve. There are concordant atrioventricular and ventriculo-arterial connections without abnormalities of the atrioventricular or arterial valves.

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IPCCC Term ATRIOVENTRICULAR SEPTAL DEFECT WITH VENTRICULAR IMBALANCE: DOMINANT RIGHT VENTRICLE, HYPOPLASTIC LEFT VENTRICLE

IPCCC Code 06.07.05

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation that is a form of atrioventricular septal defect (atrioventricular canal defect) with the right ventricle significantly larger than the left.

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This heart has been sectioned to demonstrate the short axis of the ventricular mass. It is photographed to show the view from the apex looking towards the base. There is an atrioventricular septal defect with a common atrioventricular junction. The common atrioventricular valve is committed predominately to the right ventricle, producing right ventricular dominance. The left ventricle is hypoplastic, with excessive trabeculations and closely adjacent papillary muscles. The superior and inferior portions of the deficient muscular ventricular septum are marked with the black stars.

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IPCCC Term AORTIC ROOT DIRECTLY LEFTWARD TO PULMONARY ROOT (SIDE-BY-SIDE)

IPCCC Code 02.06.05

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly to the left of the pulmonary root or its remnant.

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This anterior view demonstrates the heart in anatomic position. The cardiac apex, not shown in this image, is directed to the right. The aortic root is positioned side by side and directly leftward of the pulmonary root.

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IPCCC Term PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION OF SCIMITAR TYPE

IPCCC Code 01.01.16

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation with partial anomalous pulmonary venous connection in which some of the pulmonary veins (usually the right pulmonary veins) connect anomalously to the inferior caval vein (inferior vena cava) or to the right atrium at the insertion of the inferior vena cava.

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This heart/lung block is obtained from a patient with Scimitar syndrome. Image # 1 shows the opened right atrium viewed from the front and slightly to the right. A probe has been placed in the solitary pulmonary vein draining the right lung, which opens anomalously into the inferior caval vein immediately above the diaphragm. Although not viewed in this image, the left pulmonary veins drained in the usual fashion to the left atrium.

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