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IPCCC ICD-11 Congenital Heart Atlas

You are here: Parchment right ventricle Congenital right ventricular anomaly Congenital anomaly of a ventricle or the ventricular septum Structural developmental anomaly of heart or great vessels

IPCCC Term Parchment right ventricle
IPCCC Code 07.01.06
ICD-11 Code 240652322


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


Uhl's anomaly; Uhl anomaly

STS-EACTS-derived IPCCC term

RV abnormality, RV wall paper thin (Uhl's anomaly)

EPCC-derived IPCCC term

Parchment right ventricle (including Uhl anomaly)

ICD-11 MMS code or crossmap

LA88.Y*: This Foundation term is not in ICD-11 MMS. If coding in MMS use term “Other specified congenital anomaly of a ventricle or the ventricular septum” with code LA88.Y

ICD-10 MMS code or crossmap

Q24.8**: If coding using ICD-10, crossmap to higher order term “Other specified congenital malformations of heart” with code Q24.8 (which includes "Uhl disease")


Congenital right ventricular anomaly


Coding Notes

Parchment right ventricle can occur in association with a normal tricuspid valve (Uhl anomaly) or with an abnormal tricuspid valve, such as Ebstein Anomaly. This should be distinguished from arrhythmogenic right ventricular cardiomyopathy by the presence of fatty infiltration in the latter.

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