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, see this term), 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)(see these terms).
[Atresia of the tricuspid valve and total anomalous supracardiac pulmonary venous return. Description of a case associated with severe extracardiac malformations].
Pieri D, Rubino A, Pipitone S, Grillo R, La Monaca P, Sperandeo V. G Ital Cardiol. 1987;17(7):613-6.
The Authors report a case of tricuspid atresia and total anomalous pulmonary venous return of the supracardiac type, with mild pulmonary valve stenosis and extracardiac malformations as anal atresia, microphthalmia and irideo and chorioretinic coloboma.
