Preferred Label : Parchment right ventricle;
ICD-11 definition : A congenital cardiovascular malformation in which there is almost complete absence
of right ventricular myocardium, a normal tricuspid valve, and preserved septal and
left ventricular myocardium. 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.;
ICD-11 synonym : Right ventricular wall paper thin; Uhl anomaly;
Origin ID : 240652322;
Automatic exact mappings (from CISMeF team)
A congenital cardiovascular malformation in which there is almost complete absence
of right ventricular myocardium, a normal tricuspid valve, and preserved septal and
left ventricular myocardium. 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.
