07.01.06 – Parchment right ventricle (including Uhl anomaly)

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IPCCC Term PARCHMENT RIGHT VENTRICLE (INCLUDING UHL ANOMALY)

IPCCC Code 07.01.06

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is almost complete absence of right ventricular myocardium and preserved septal and left ventricular myocardium.

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07-01-06 The right atrium and ventricle have been opened in clam-shell fashion and are viewed from the antero-lateral aspect. There is almost complete absence of the right ventricular myocardium in this heart with an unguarded right ventricular inlet (yellow dots mark the atrioventricular junction) and pulmonary atresia with intact ventricular septum. The septal and left ventricular myocardium are preserved (left ventricle not shown).

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