01.01.10 – Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect

IPCCC Term TRANSPOSITION OF THE GREAT ARTERIES WITH CONCORDANT ATRIOVENTRICULAR CONNECTIONS AND VENTRICULAR SEPTAL DEFECT

IPCCC Code 01.01.10

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, and one or more ventricular septal defects are present.

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01-01-10 The anterior free wall of the right atrium, right ventricle and aorta have been removed to view the septal surfaces of this heart with transposition. There are concordant atrioventricular connections and discordant ventriculo-arterial connections with the tricuspid valve guarding the inlet of the right ventricle and the aorta arising from a complete muscular infundibulum. The defect extends into the outlet component of the right ventricle and has a muscular postero-inferior border. (Red star – pulmonary trunk) There is an atrial septal defect.
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04.03.10 – Interrupted inferior caval vein with absent suprarenal segment and azygos continuation

IPCCC Term INTERRUPTED INFERIOR CAVAL VEIN WITH ABSENT SUPRARENAL SEGMENT AND AZYGOS CONTINUATION

IPCCC Code 04.03.10

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is an absence of the renal-to-hepatic segment of the inferior caval vein (inferior vena cava) with connection to a superior caval vein (superior vena cava) through the azygos venous system.

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04-03-10 In this posterior view of an organ block prior to dissection shows absence of the renal-to-hepatic segment of the inferior caval vein (inferior vena cava). The inferior caval vein continues posteriorly through the right paravertebral gutter, draining through the azygos vein into the superior caval vein (superior vena cava). In the usual situation, the left renal veins passes anterior to the aorta, but when the caval vein is interrupted, it runs posteriorly. In this specimen, there are two left renal veins.
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02.07.00 – Spiralling course of great arteries

IPCCC Term SPIRALLING COURSE OF GREAT ARTERIES

IPCCC Code 02.07.00

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular finding in which the course of the great arteries results in a nearly orthogonal relationship of the axis of the ascending aorta to the axis of the pulmonary trunk.

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02-07-00 alt This anterior view of the normal heart shows the spiraling course of the intrapericardial arterial trunks. The ascending aorta takes its origin from the rightward and posterior aortic root. The pulmonary trunk, taking a superior and leftward origin, extends to the right and inferiorly as it passes beneath the aortic arch to reach to the margins of the pericardial cavity, where it gives rise to the right and left pulmonary arteries.
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04.04.14 – Coronary sinus orifice atresia or stenosis

IPCCC Term CORONARY SINUS ORIFICE ATRESIA OR STENOSIS

IPCCC Code 04.04.14

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which the orifice of the coronary sinus is narrowed or absent.

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Image #1 shows an anterior view of the right atrioventricular junction in a heart with double inlet left ventricle and atresia of the coronary sinus (yellow brackets). The Eustachian valve is marked with red dots and there is a small deficiency in the flap valve or atrial septal defect at the floor of the oval fossa. The same heart is depicted from behind in Image #2 with a small vertical vein draining the coronary veins that were attached to the obstructed coronary sinus. This vertical vein is in the same position as a persistent left superior caval vein and drains the coronary venous blood to the left brachiocephalic vein.
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01.01.02 – Transposition of the great arteries with concordant atrioventricular connections and intact ventricular septum

IPCCC Term TRANSPOSITION OF THE GREAT ARTERIES WITH CONCORDANT ATRIOVENTRICULAR CONNECTIONS AND INTACT VENTRICULAR SEPTUM

IPCCC Code 01.01.02

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, and a ventricular septal defect is not present.

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These images show the right and left sides of a heart with transposition, or concordant atrioventricular connections and discordant ventriculo-arterial connections in the setting of an intact ventricular septum. Image #1 shows the morphologically right atrium connected to a morphologically right ventricle through a tricuspid valve with the aorta arising about a completely muscular infundibulum. In Image #2, the mitral valve guards the inlet of the morphologically left ventricle with the pulmonary trunk arising from the outlet in fibrous continuity with the leaflets of the mitral valve. The morphologically left atrium is not seen in this image.
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02.06.04 – Aortic root anterior and leftward to pulmonary root

IPCCC Term AORTIC ROOT ANTERIOR AND LEFTWARD TO PULMONARY ROOT

IPCCC Code 02.06.04

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

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

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02-06-04 alt This anterior anatomic view shows the aorta anterior and to the left of the pulmonary trunk. The aortic arch extends to the left with the normal branching pattern of the brachiocephalic arteries. Note the pulmonary artery band and the juxtaposed atrial appendages to the left of the arterial pedicle.
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01.03.00 – Usual atrial arrangement

IPCCC Term USUAL ATRIAL ARRANGEMENT

IPCCC Code 01.03.00

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac finding in which the atrial laterality (sidedness) is normal.

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01-03-00 alt This superior, short-axis view of the base of the heart shows usual atrial arrangement with the morphologically left atrium to the right and the morphologically left atrium to the left. The right atrium demonstrates pectinate muscles around the atrioventricular junction to the crux of the heart. The left atrial vestibule is smooth and the appendage has a narrow attachment with the pectinate muscles confined to the appendage. Note the coronary sinus running in the left atrioventricular groove.
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02.01.03 – Laevocardia

IPCCC Term LAEVOCARDIA

IPCCC Code 02.01.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

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

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02-01-03 This anterior in situ image shows the heart within the chest in its normal position which is predominantly to the left and with the apex pointing leftward. The yellow dots mark the pericardial reflection on the diaphragmatic aspect and the black dots mark its superior-most extent.
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09.43.04 – Anterior descending from right coronary artery across right ventricular outflow tract

IPCCC Term ANTERIOR DESCENDING FROM RIGHT CORONARY ARTERY ACROSS RIGHT VENTRICULAR OUTFLOW TRACT

IPCCC Code 09.43.04

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the left circumflex coronary artery arises normally and the anterior descending coronary artery arises from the proximal right coronary artery and courses across the right ventricular outflow tract.

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09-43-04 In this anterior superior view of a heart with repaired tetralogy of Fallot, the pulmonary conduit has been retracted forward to show the anterior interventricular coronary artery (anterior descending coronary artery) arising from the proximal right coronary artery and coursing across the right ventricular outflow tract. Note the sutures depressing the pate of the anterior interventricular artery as it takes its normal path along the interventricular septum. The circumflex artery (not seen) arises from its own orifice from the left-facing sinus and extends in the normal fashion along the left atrioventricular groove.
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09.10.30 – Congenitally discontinuous, non-confluent right and left pulmonary arteries

IPCCC Term CONGENITALLY DISCONTINUOUS, NON-CONFLUENT RIGHT AND LEFT PULMONARY ARTERIES

IPCCC Code 09.10.30

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is absence of luminal continuity between the right and left branch pulmonary arteries.

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09-10-30 alt The heart is tilted slightly to the right in this anterior superior view to show a solitary arterial trunk exiting the base of the ventricular mass. The pulmonary arteries are discontinuous and non-confluent, the branch pulmonary arteries supplied bilaterally via an arterial duct. The right pulmonary artery remains attached to the right lung while the left is suspended by a suture. On the left, the pulmonary artery arises from the usual position of the arterial duct. On the right, it arises from the brachiocephalic trunk (red star). The aortic arch extends to the left and the brachiocephalic arteries branch in the normal fashion. There is no identifiable intrapericardial pulmonary trunk.
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