Tricuspid 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).

Also known as congenital atresia of tricuspid valve, tricuspid atresia, tricuspid atresia (disease), tricuspid valve atresia — per MONDO

Also identified as

• DOID 0080169 per MONDO
• MESH D018785 per MONDO
• NCIT C85202 per MONDO
• OMIM 605067 per MONDO
• Orphanet 1209 per MONDO
• SCTID 63042009 per MONDO
• UMLS C0243002 per MONDO

Affected Organs

Drugs indicated