General Information of Disease (ID: DISOMW0K)

Disease Name Tricuspid atresia
Synonyms congenital agenesis of the tricuspid valve; tricuspid atresia; tricuspid atresia (disease); congenital atresia of tricuspid valve; tricuspid valve atresia
Definition
Tricuspid atresia is (TA) a rare congenital heart malformation characterized by the congenital agenesis of tricuspid valve leading to severe hypoplasia of right ventricle (functionally univentricular). TA is associated with normally related or transposed great vessels (TGV), an obligatory interatrial connection that is crucial for survival (patent foramen ovale or atrial septal defect, osteum secondum type), ventricular septal defect (in 90% cases), pulmonary outflow obstruction - pulmonary atresia, stenosis or hypoplasia (usually in TA with normally related vessels but also in TGV), aortic coarctation and/or aortic arch interruption (usually in TA with TGV).
Disease Hierarchy
DIS2ANVO: Congenital tricuspid malformation
DISMT2VZ: Cardiogenetic disease
DISQBA23: Congenital heart disease
DISOMW0K: Tricuspid atresia
Disease Identifiers
MONDO ID
MONDO_0011514
MESH ID
D018785
UMLS CUI
C0243002
OMIM ID
605067
MedGen ID
67034
HPO ID
HP:0011662
Orphanet ID
1209
SNOMED CT ID
253455004