06.04.11 – Congenital anomaly of left-sided atrioventricular valve in double inlet ventricle

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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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06-04-11 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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