Preferred Name

Uhl anomaly

Synonyms

Uhl's anomaly

parchment right ventricle

Definitions

Uhl anomaly is characterized by an almost complete absence of the myocardium in the right ventricle resulting in a thin walled nonfunctional right ventricle manifesting with cardiac arrhythmias and right ventricular failure. Cases of partial absence of right ventricular myocardium which remains asymptomatic or mildly symptomatic until adulthood have also been reported. Patients presenting with complete Uhl anomaly should be considered for cardiac transplantation. Editor note: check xrefs

ID

http://purl.obolibrary.org/obo/MONDO_0018084

comment

Editor note: check xrefs

altLabel

Uhl's anomaly

parchment right ventricle

definition

Uhl anomaly is characterized by an almost complete absence of the myocardium in the right ventricle resulting in a thin walled nonfunctional right ventricle manifesting with cardiac arrhythmias and right ventricular failure. Cases of partial absence of right ventricular myocardium which remains asymptomatic or mildly symptomatic until adulthood have also been reported. Patients presenting with complete Uhl anomaly should be considered for cardiac transplantation.

Editor note: check xrefs

has_related_synonym

Uhl's anomaly

parchment right ventricle

label

Uhl anomaly

prefixIRI

MONDO:0018084

prefLabel

Uhl anomaly

textual definition

Uhl anomaly is characterized by an almost complete absence of the myocardium in the right ventricle resulting in a thin walled nonfunctional right ventricle manifesting with cardiac arrhythmias and right ventricular failure. Cases of partial absence of right ventricular myocardium which remains asymptomatic or mildly symptomatic until adulthood have also been reported. Patients presenting with complete Uhl anomaly should be considered for cardiac transplantation.

subClassOf

http://purl.obolibrary.org/obo/MONDO_0004994

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