Tricuspid atresia

Last revised by Calum Worsley on 23 Oct 2022

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).

Presentation is in the neonatal period if not diagnosed antenatally, with progressive cyanosis, which is classically worse when crying 7.

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

Recognized extra-cardiac associations include:

Chest radiographic features may vary depending on the presence and extent of a ventricular septal defect or transposition of the great arteries. 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.

Treatment is surgical. Initially a shunt is placed to increase pulmonary blood flow (e.g. Blalock-Taussig shunt). Definitive repair is with a Fontan procedure (which has several indications but was first described to treat tricuspid atresia 8). Patients successfully corrected can live into adulthood.

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