JSON

Uhl anomaly

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


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. [ Orphanet:3403 ]

Term info

database cross reference
  • GARD:0005393 (MONDO:equivalentTo)
  • Orphanet:3403 (MONDO:equivalentTo)
  • MedDRA:10048951 (Orphanet:3403/e)
  • MESH:C536932 (Orphanet:3403/e)
Subsets

ordo_morphological_anomaly

closeMatch

http://identifiers.org/meddra/10048951

comment

Editor note: check xrefs

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.

exactMatch

http://identifiers.org/mesh/C536932, http://purl.obolibrary.org/obo/Orphanet_3403

has related synonym

Uhl's anomaly, parchment right ventricle

id

MONDO:0018084

Term relations

Subclass of: