Uhl anomaly

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.

http://www.orpha.net/ORDO/Orphanet_3403

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

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3403

Infancy

Neonatal

Not applicable

ICD-11:LA88.Y

MeSH:C536932

MedDRA:10048951

OMIM:107970

UMLS:C0265857

ICD-10:Q24.8

Uhl anomaly

ORPHA:3403

http://www.orpha.net/ORDO/Orphanet_217678

ORDO:Orphanet_3403

Uhl anomaly

Worldwide AND has_point_prevalence_range : <1 / 1 000 000

Worldwide AND has_cases/families_value : 84.0 (Case)

Worldwide AND has_birth_prevalence_average_value : 1.0 AND has_birth_prevalence_range : 1-9 / 100 000

http://www.orpha.net/ORDO/Orphanet_217678

http://www.orpha.net/ORDO/Orphanet_557493

http://www.orpha.net/ORDO/Orphanet_377791