07.12.03 – Doubly committed juxta-arterial ventricular septal defect without malalignment and with perimembranous extension

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IPCCC Term DOUBLY COMMITTED JUXTA-ARTERIAL VENTRICULAR SEPTAL DEFECT WITHOUT MALALIGNMENT AND WITH PERIMEMBRANOUS EXTENSION

IPCCC Code 07.12.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is an outlet ventricular septal defect with perimembranous extension, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, that is aligned with the trabecular muscular septum.

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07-12-03 In this anterior view, the free wall of the right ventricle has been folded rightward to demonstrate doubly committed and juxta-arterial ventricular septal defect, bordered postero-inferiorly by fibrous continuity between the leaflets of tricuspid, aortic, and mitral valves. The defect opens to the outlet of the right ventricle between the limbs of the septal band and extends cranially to be bordered by an area of fibrous continuity (black dots) between the aortic and pulmonary valves. There is alignment between the fibrous outlet septum and the apical part of the muscular ventricular septum.

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06.05.25 – Double orifice of left ventricular component of common atrioventricular valve

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IPCCC Term DOUBLE ORIFICE OF LEFT VENTRICULAR COMPONENT OF COMMON ATRIOVENTRICULAR VALVE

IPCCC Code 06.05.25

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which the left ventricular component of the common atrioventricular valve has two orifices.

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[su_carousel source=”media: 6728,6730″ limit=”27″ width=”600″ height=”460″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image #1 shows a short axis view of the base of the heart as viewed from the atrial aspect. There is an unbalanced atrioventricular septal defect with right ventricular dominance and separate orifices for the right and left ventricles. The smaller left ventricular component itself exhibits a dual orifice. The smaller orifice extends along the postero-inferior aspect of the ventricular septum. In Image #2, the left ventricle has been opened in clamshell fashion, demonstrating the characteristic scooped-out crest (red dots) of the ventricular septum and the adherent inferior and superior bridging leaflets which separate the common atrioventricular junction into separate right and left ventricular orifices. Within the left component, the smaller orifice is supported by its own papillary muscle.

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01.04.00 – Concordant atrioventricular connections

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IPCCC Term CONCORDANT ATRIOVENTRICULAR CONNECTIONS

IPCCC Code 01.04.00

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular finding in which the morphologically right atrium connects to the morphologically right ventricle and the morphologically left atrium connects to the morphologically left ventricle.

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01-04-00 This heart is sectioned to visualize all four chambers. The inferior half is shows and demonstrates concordant atrioventricular conncetions. The morphologically right atrium is connected to the morphologically right ventricle and the morphologically left atrium is connected to the morphologically left ventricle. The terminal crest within the right atrium is marked with a red arrow.

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

03.01.03 – Total mirror imagery

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IPCCC Term TOTAL MIRROR IMAGERY

IPCCC Code 03.01.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital malformation in which there is complete mirror-imaged arrangement of the internal organs along the left-right axis of the body.

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03-01-03 This is an anterior view of the organs as they lie in the body. There is complete mirror-imaged arrangement of the internal organs along the left-right axis of the body. The superior and inferior caval veins are left-sided and the brachiocephalic vein extends to the right. The apex of the heart points toward the right with its largest part contained within the right hemithorax. The spleen is on the right and the larger lobe of the liver is on the left. The appendix is within the left upper quadrant of the abdomen.

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

02.06.03 – Aortic root directly anterior to the pulmonary root

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IPCCC Term AORTIC ROOT DIRECTLY ANTERIOR TO THE PULMONARY ROOT

IPCCC Code 02.06.03

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 anterior to the pulmonary root or its remnant.

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02-06-03 This anterior view of the heart demonstrates the aorta positioned directly anterior to the pulmonary trunk in this heart with congenitally corrected transposition. The aortic arch extends to the left in the usual fashion and the brachiocephalic arteries branch normally.

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02.01.02 – Dextrocardia

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IPCCC Term DEXTROCARDIA

IPCCC Code 02.01.02

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the heart is predominantly to the right of the thoracic midline.

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02-01-02 The image shows an anterior view of the organs as they lie within the chest, demonstrating dextrocardia with the majority of the heart lying within the right chest and with its apex pointing to the right. Note that there is also mirror image arrangement of the caval veins, the brachiocephalic vein, and the arterial trunks, which spiral in mirror-imaged fashion.

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02.01.02 – Dextrocardia

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IPCCC Term DEXTROCARDIA

IPCCC Code 02.01.02

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the heart is predominantly to the right of the thoracic midline.

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02-01-02 The image shows an anterior view of the organs as they lie within the chest, demonstrating dextrocardia with the majority of the heart lying within the right chest and with its apex pointing to the right. Note that there is also mirror image arrangement of the caval veins, the brachiocephalic vein, and the arterial trunks, which spiral in mirror-imaged fashion.

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

01.01.07 – Pulmonary atresia with intact ventricular septum

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IPCCC Term PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

IPCCC Code 01.01.07

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication.

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[su_carousel source=”media: 6685,6687″ limit=”27″ width=”460″ height=”600″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image # 1: This specimen demonstrates pulmonary atresia-intact septum and is viewed in anatomic position with the free wall of the right atrium and ventricle removed. The cavity of the right ventricle is bipartite, being represented only by its inlet and outlet component due to mural overgrowth of the apical trabecular component. This produces a less favorable type of pulmonary atresia-intact septum within the spectrum of this malformation. The outflow tract is patent, but extremely stenotic. At the distal aspect of the outflow tract there is pulmonary atresia with a tiny remnant of an imperforate valve at the ventriculo-arterial junction. Image #2: This close-up view of the atretic pulmonary valve is viewed from above and demonstrates triradiating sinuses peripherally with a tiny, imperforate valve centrally. The dilated sinuses of Valsalva are easily appreciated.

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09.43.05 – Intramural proximal coronary arterial course

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IPCCC Term INTRAMURAL PROXIMAL CORONARY ARTERIAL COURSE

IPCCC Code 09.43.05

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the proximal coronary artery courses within and parallel to the wall of the aorta before it emerges to assume its epicardial course.

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[su_carousel source=”media: 6766,6768″ limit=”27″ width=”600″ height=”460″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image # 1 is the anterior superior view and image #2 is the superior view of the same specimen. In both images, the pulmonary trunk has been retracted to demonstrate a malformation in which the proximal left main coronary artery courses within and parallel to the wall of the aorta before it emerges to assume its usual epicardial course. The intramural course in this specimen is long and extremely stenotic. The left coronary orifice arises from the right coronary sinus and the coronary artery crosses the commissure (Image #1 – red star) between the right- and left-facing sinuses.

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09.46.21 – Left coronary artery from right aortic sinus with ventriculo-arterial concordance

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IPCCC Term LEFT CORONARY ARTERY FROM RIGHT AORTIC SINUS WITH VENTRICULO-ARTERIAL CONCORDANCE

IPCCC Code 09.46.21

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in the setting of ventriculo-arterial concordance in which the left coronary artery arises from, or immediately above, the right sinus of Valsalva.

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[su_carousel source=”media: 6766,6768″ limit=”27″ width=”600″ height=”460″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image # 1 is the anterior superior view and image #2 is the superior view of the same specimen. In both images, the pulmonary trunk has been retracted to demonstrate a malformation in which the proximal left main coronary artery courses within and parallel to the wall of the aorta before it emerges to assume its usual epicardial course. The intramural course in this specimen is long and extremely stenotic. The left coronary orifice arises from the right coronary sinus and the coronary artery crosses the commissure (Image #1 – red star) between the right- and left-facing sinuses.

[/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]