06.01.34 – Ebstein malformation of tricuspid valve

IPCCC Term EBSTEIN MALFORMATION OF TRICUSPID VALVE

IPCCC Code

06.01.34

ICD-11 Code

PENDING

Synonyms

PENDING

Abbreviations

PENDING

IPCCC Definition

A congenital cardiac malformation of the tricuspid valve and right ventricle that is characterized by downward (apical) displacement of the functional annulus, usually involving the septal and inferior (posterior) leaflets.

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01.01.03 – Congenitally corrected transposition of great arteries

IPCCC Term CONGENITALLY CORRECTED TRANSPOSITION OF GREAT ARTERIES

IPCCC Code 01.01.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically left ventricle, the morphologically left atrium connects to the morphologically right ventricle, the morphologically right ventricle connects to the aorta, and the morphologically left ventricle connects to the pulmonary trunk.

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09.15.19 – congenital anomaly of aortic valve

IPCCC Term CONGENITAL ANOMALY OF AORTIC VALVE

IPCCC Code 09.15.19

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation where the aortic valve is abnormal.

07.11.12 – Trabecular muscular ventricular septal defect: postero-inferior

IPCCC Term TRABECULAR MUSCULAR VENTRICULAR SEPTAL DEFECT: POSTERO-INFERIOR

IPCCC Code 07.11.12

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 right ventricle in the postero-inferior region of the trabeculated component of the ventricular septum.

  • 07-11-12 The anterior free wall of the right atrium and right ventricle have been removed to demonstrate a muscular ventricular septal defect along the postero-inferior aspect of the trabecular component of the ventricular septum. This ventricular septal defect is well away from the septal leaflet of the tricuspid valve and lies near the diaphragmatic aspect of the right ventricle. There are tendinous cords passing anterior to the defect and attaching to the inferior papillary muscle. Note the atrial septal defect and perimembranous ventricular septal defects that were previously repaired.
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09.45.10 – Congenital coronary arterial fistula to right ventricle

IPCCC Term CONGENITAL CORONARY ARTERIAL FISTULA TO RIGHT VENTRICLE

IPCCC Code 09.45.10

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with the right ventricle.

  • Image #1: In this anterior view, the right ventricle is opened in clam-shell fashion to demonstrate a large, fistulous communication between the coronary arteries and the right ventricle (black arrows). The inferior interventricular coronary arteries both contribute to this fistulous communication. This heart demonstrates the most severe variant of pulmonary atresia with intact ventricular septum in which only the inlet component of the right ventricle is present. The apical trabecular component and the outlet have been mostly obliterated by muscular hypertrophy. Image #2: This short axis view shows the apical component of both the right and left ventricles with a large coronary artery to right ventricular fistula.
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09.04.07 – Congenital aortopulmonary window

IPCCC Term CONGENITAL AORTOPULMONARY WINDOW

IPCCC Code 09.04.07

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is side-to-side continuity of the lumens of the ascending aorta and pulmonary trunk in association with separate aortic and pulmonary valves or their atretic remnants.

  • Image #1 shows an anterior view of the normally related arterial trunks that are clearly separate as they exit the ventricular mass. The yellow star marks the outer surface of the aortopulmonary window. The brachiocephalic arteries branch normally from the aortic arch which extends to the left. Image #2 shows a similar view of the same heart with the aorta opened from just above the valve, along the ascending portion and into the arch. The aortic aspect of the aortopulmonary window is marked with yellow dots. The right pulmonary artery can be seen through the aortopulmonary window. Image #3 shows the opened right ventricular outflow tract, pulmonary valve and pulmonary trunk from the same heart shown in the other panels. The pulmonary aspect of the aortopulmonary window is marked with yellow dots and lies between the valve and the bifurcation of the left pulmonary artery.
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05.01.02 – Atrial septal defect within oval fossa

IPCCC Term ATRIAL SEPTAL DEFECT WITHIN OVAL FOSSA

IPCCC Code 05.01.02

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is an interatrial communication confined to the region of the oval fossa (fossa ovalis), most commonly due to a deficiency of the primary atrial septum (septum primum) but deficiency of the septum secundum (superior interatrial fold) may also contribute.

  • 05-04-02 The anterior wall of the right atrial appendage has been incised and folded away revealing an interatrial communication (atrial septal defect) secondary to a deficiency in the floor of the oval fossa or primary atrial septum (septum primum).
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09.02.01 – Dysplasia of truncal valve

IPCCC Term DYSPLASIA OF TRUNCAL VALVE

IPCCC Code 09.02.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation where the truncal valve leaflets are markedly thickened with restricted mobility, characterized by the presence of myxomatous tissue.

  • 09-02-01 This anterior view of the right ventricular outlet shows an opened common arterial trunk with a dysplastic valve consisting of four leaflets. The leaflets are markedly thickened with a myxomatous appearance. The common trunk arises almost exclusively from the right ventricle and there is a restrictive, interventricular communication (ventricular septal defect) that is subtruncal with perimembranous extension. The pulmonary trunk arises very close to a truncal valvar sinus.
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09.01.14 – Common arterial trunk with aortic dominance and both pulmonary arteries arising from trunk

IPCCC Term COMMON ARTERIAL TRUNK WITH AORTIC DOMINANCE AND BOTH PULMONARY ARTERIES ARISING FROM TRUNK

IPCCC Code 09.01.14

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a common arterial trunk is associated with an unobstructed aortic arch and both pulmonary arteries arise from the common arterial trunk.

  • The images demonstrate a common arterial trunk with separate origin of both the right and left pulmonary arteries. Image #1 is viewed from an anterior and slightly superior position with the anterior aspect of the arterial trunk incised and folded forward. The right and left pulmonary arteries clearly arise separately from the posterior aspect of the common trunk. The left coronary orifice arises slightly above the sinotubular junction and the right coronary orifice is not seen. Image #2 shows the posterior aspect of the common arterial trunk with separate origin of the right and left pulmonary arteries. The aortic arch extends leftward, and is unobstructed with the brachiocephalic arteries branching normally from the aortic arch.
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09.01.01 – Common arterial trunk

IPCCC Term COMMON ARTERIAL TRUNK

IPCCC Code 09.01.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a single arterial trunk arises from the heart, giving origin sequentially to the coronary arteries, one or more pulmonary arteries, and the systemic arterial circulation.

  • 09-01-01 This anterior view of the right ventricular outlet shows a common arterial trunk and an interventricular communication (ventricular septal defect) that is subtruncal with perimembranous extension. Along the inferior border of the ventricular septal defect the tricuspid, truncal and mitral valves are in fibrous continuity. The truncal valve consists of three, semilunar leaflets that are slightly thickened. The common arterial trunk supplies the coronary, pulmonary and systemic circulation with both pulmonary arteries arising separately from the left posterolateral aspect. The left coronary orifice arises slightly above the sinutubular junction and has a slit-like appearance. The right coronary artery is not visualized.
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