Tricuspid atresia

Last revised by Dr Joachim Feger on 10 May 2020

Tricuspid atresia is a cyanotic congenital cardiac anomaly which is characterized 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, tricuspid atresia may also be associated with transposition of great arteries (TGA).

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

Recognized extra-cardiac associations include:

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.

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

Allows direct visualization of the 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.

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Cases and figures

  • Case 1
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  • Case 2: with Fontan circulation
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