01.05.01 + 01.04.00 + 07.10.00 + 07.09.01 – Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect and lev

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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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[su_carousel source=”media: 6263,6264″ limit=”27″ width=”460″ height=”510″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] 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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