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).
It results from an unequal atrio-ventricular canal division and the right ventricle is typically very hypoplastic.
Recognised 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 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.
- 1. Fletcher BD, Jacobstein MD, Abramowsky CR et-al. Right atrioventricular valve atresia: anatomic evaluation with MR imaging. AJR Am J Roentgenol. 1987;148 (4): 671-4. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Goo HW, Park IS, Ko JK et-al. CT of congenital heart disease: normal anatomy and typical pathologic conditions. Radiographics. 2003;23 Spec No : S147-65. doi:10.1148/rg.23si035501 - Pubmed citation
- 3. Brown DL, Emerson DS, Cartier MS et-al. Congenital cardiac anomalies: prenatal sonographic diagnosis. AJR Am J Roentgenol. 1989;153 (1): 109-14. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Cook AL, Hurwitz LM, Valente AM et-al. Right heart dilatation in adults: congenital causes. AJR Am J Roentgenol. 2007;189 (3): 592-601. doi:10.2214/AJR.07.2420 - Pubmed citation
- 5. Siegel MJ, Bhalla S, Gutierrez FR et-al. MDCT of postoperative anatomy and complications in adults with cyanotic heart disease. AJR Am J Roentgenol. 2005;184 (1): 241-7. AJR Am J Roentgenol (full text) - Pubmed citation
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.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt