tricuspid atresia
Findings
No curated finding names tricuspid atresia yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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).
Definition from the Mondo Disease Ontology (MONDO:0011514), read 2026-09-29. CC BY 4.0.
Features
10 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Tricuspid atresiaHPOHP:0011662
- Obligate (100% of cases)
- CyanosisHPOHP:0000961
- Very frequent (80% to 99% of cases)
- Ventricular septal defectHPOHP:0001629
- Very frequent (80% to 99% of cases)
- Atrial septal defectHPOHP:0001631
- Frequent (30% to 79% of cases)
- Hypoplasia of right ventricleHPOHP:0004762
- Frequent (30% to 79% of cases)
- Patent foramen ovaleHPOHP:0001655
- Frequent (30% to 79% of cases)
- Persistent left superior vena cava
Where it sits
Other names
3 names
Resolves to: tricuspid atresia
- Also called
- congenital atresia of tricuspid valvetricuspid atresia (disease)tricuspid valve atresia