Archive for category: IPCCC Structure

IPCCC Term CONGENITAL ANOMALY OF LEFT-SIDED ATRIOVENTRICULAR VALVE IN DOUBLE INLET VENTRICLE

IPCCC Code 06.04.11

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in association with double inlet ventricle (excluding common atrioventricular valve) in which the atrioventricular valve morphology cannot be determined. This term should be used for the left-sided atrioventricular valve in those hearts.

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This heart is viewed from posterior to anterior and has been cut in a four chamber echocardiographic plane. There is a double inlet left ventricle with an abnormality of the left-sided atrioventricular valve. It straddles the interventricular septum with the tension apparatus extending across the ventricular septal defect into the rudimentary, incomplete right ventricle. This heart was explanted following a Fontan procedure. Note the remnant of the right-sided atrial baffle (red star).

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IPCCC Term SINGLE CORONARY SUPPLYING ALL OF HEART

IPCCC Code 09.43.13

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a solitary coronary artery supplies the myocardium.

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In this left anterior lateral view the pulmonary trunk is folded forward and the subpulmonary muscular infundibulum has been dissected. The red dots mark the junction of the pulmonary arterial wall with the infundibular muscle. There is a single coronary artery with the left main coronary artery arising from the proximal right coronary artery and extending between the arterial trunks within the infundibular muscle. As it emerges from the muscle to the left of the pulmonary trunk, the artery divided in the usual fashion to the anterior interventricular and circumflex branches. The right coronary artery took its usual course within the right atrioventricular groove.

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IPCCC Term TRANSPOSITION OF THE GREAT ARTERIES WITH CONCORDANT ATRIOVENTRICULAR CONNECTIONS AND VENTRICULAR SEPTAL DEFECT AND LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION

IPCCC Code 01.05.01 + 01.04.00 + 07.10.00 + 07.09.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically right ventricle, the morphologically left atrium connects to the morphologically left ventricle, the morphologically right ventricle connects to the aorta, the morphologically left ventricle connects to the pulmonary trunk, one or more ventricular septal defects are present, and left ventricular outflow tract obstruction is present.

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In Image #1, the anterior free wall of the right ventricle and the aorta have been removed. This heart has concordant atrioventricular connections with discordant ventriculo-arterial connections along with a ventricular septal defect and posterior deviation of the outlet septum. The ventricular septal defect is within the outlet component of the right ventricle and extends to become perimembranous with malalignment of the outlet septum. The tendinous cords that support the tricuspid valve extend across the inferior aspect of the defect. Image #2 shows the left ventricular outflow tract from the same heart as in the left panel. The malalignment of the outlet septum narrows the left ventricular outflow tract and causes significant pulmonary stenosis. The pulmonary valve is bicuspid.

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IPCCC Term CONGENITAL TRUNCAL VALVAR STENOSIS

IPCCC Code 09.02.18

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is obstruction to flow through the truncal valve due to narrowing or stricture.

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In this anterior view the right ventricular outflow tract is opened to show the arterial valve in this heart with a common arterial trunk. The valve is quadricuspid, thickened and dysplastic. There is valvar stenosis with post-stenotic dilation of the common trunk. The pulmonary component branches very low from the common trunk, at the sinutubular junction and extending into the adjacent sinus of Valsalva. There is a restrictive, subarterial, muscular ventricular septal defect.

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IPCCC Term ANEURYSM OF AORTIC SINUS OF VALSALVA

IPCCC Code 09.18.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is dilation of a single aortic sinus of Valsalva.

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Image #1 is a close up, superior view of an opened aortic valve. The right coronary aortic valvar leaflet is retracted with a suture to demonstrate the dilated sinus of Valsalva. The aortic root in this case was poorly supported with a large membranous septum and no muscular support beneath the right coronary leaflet. Only the left coronary leaflet was supported by muscle (not shown). Note the left coronary orifice with its origin near the attachment of the left coronary leaflet at the sinutubular junction. In Image #2, the right atrioventricular junction is viewed anteriorly and in anatomic position. The aneurysm at the atrioventricular junction has been transected. The sectioned atrial myocardium and tricuspid valve demonstrate the close proximity of the aorta, atrioventricular and interventricular components of the membranous septum. The sinus of Valsalva aneurysm caused an ovoid, saccular dilation of the tricuspid valve hinge point that eventually perforated.

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