01.01.02 – Transposition of the great arteries with concordant atrioventricular connections and intact ventricular septum

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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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