Tricuspid atresia

Tricuspid atresia is a cyanotic congenital cardiac anomaly which is characterised by agenesis of the tricuspid valve and right ventricular inlet. There is almost always an obligatory intra-atrial connection through either an ASD or patent foramen ovale (PFO) in order for circulation to be complete 5. A small VSD is often also present. In a proportion of cases they may also be associated with transposition of great arteries (TGA).

Pathology

It results from an unequal atrio-ventricular canal division and the right ventricle is typically very hypoplastic.

Associations

Recognised extra-cardiac associations include:

Radiographic features

Plain radiograph

Chest radiographic features may vary depending on the presence and extent of a VSD or TGA. May demonstrate decreased pulmonary vascularity (i.e. oligaemic appearance). Cardiac size may be normal or enlarged.

Echocardiography/ultrasound

Usually the 1st line imaging modality in utero. It allows direct visualisation of the anomaly. 

CT and MRI

Allows direct visualisation of anomaly and may typically show a fatty and/or muscular separation of the right atrium from the right ventricle. Cine MRI can offer functional information in addition to anatomy.


Congenital heart disease

There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.

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Article Information

rID: 7374
Synonyms or Alternate Spellings:
  • Right atrioventricular valve atresia
  • Atresia of the tricuspid valve

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