cardiomyopathy Uhl anomaly UMLS:C0265857 MedDRA:10048951 GARD:0005393 MEDGEN:78576 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. MONDO:0018084 Editor note: check xrefs icd11.foundation:240652322 parchment right ventricle MESH:C536932 Uhl's anomaly Orphanet:3403