Double outlet right ventricle (DORV) is a congenital cardiac anomaly where both the aorta and pulmonary trunk arise from the morphologically right ventricle. It is reported to account for ~2% of congenital cardiac defects 1. It is usually classed as a conotruncal anomaly. There is almost always a concurrent ventricular septal defect (VSD) 4.
The estimated incidence is at ~1:10,000 births.
Most cases are thought to have a sporadic occurrence.
According to the position of the great vessels 5:
- side by side positioning of great vessels
- right-sided malpositioning of great vessels
- left-sided malpositioning of great vessels
According to where the VSD is located about the great vessels 6:
- DORV with subaortic VSD
- VSD is located just below the aorta
- DORV with subpulmonary VSD (also called Taussig-Bing anomaly)
- VSD is located below the pulmonary artery
- DORV with doubly committed VSD
- VSD under both of the great arteries
- DORV with non-committed (or remote) VSD
- VSD is not located near the aorta or the pulmonary artery
Appearances on chest radiographs are variable depending on the subclassification and presence of concurrent other anomalies. May show evidence of right ventricular enlargement.
On echocardiography, there is typically a lack of communication between the posterior aortic root and the anterior mitral valve leaflet.
Allows direct visualization of abnormal anatomy.
- 1. Obler D, Juraszek AL, Smoot LB et-al. Double outlet right ventricle: aetiologies and associations. J. Med. Genet. 2008;45 (8): 481-97. doi:10.1136/jmg.2008.057984 - Pubmed citation
- 2. Yoo SJ, Lim TH, Park IS et-al. MR anatomy of ventricular septal defect in double-outlet right ventricle with situs solitus and atrioventricular concordance. Radiology. 1991;181 (2): 501-5. Radiology (abstract) - Pubmed citation
- 3. Wiant A, Nyberg E, Gilkeson RC. CT evaluation of congenital heart disease in adults. AJR Am J Roentgenol. 2009;193 (2): 388-96. doi:10.2214/AJR.08.2192 - Pubmed citation
- 4. Barboza JM, Dajani NK, Glenn LG et-al. Prenatal diagnosis of congenital cardiac anomalies: a practical approach using two basic views. Radiographics. 22 (5): 1125-37. Radiographics (full text) - Pubmed citation
- 5. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 6. Frank L, Dillman JR, Parish V et-al. Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 2010;30 (4): 1069-94. doi:10.1148/rg.304095158 - Pubmed citation
Related Radiopaedia articles
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorized, 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