double outlet right ventricle (DORV)

Double outlet
right ventricle • Double outlet right ventricle (with VSD and PAH) - Ganzer Fall bei Radiopaedia
Jha P, Double outlet right ventricle (with VSD and PAH). Case study, Radiopaedia.org (Accessed on 27 Dec 2022) https://doi.org/10.53347/rID-18770 • CC by-nc-sa 3.0 (modified)
Double outlet
right ventricle • Double outlet right ventricle and dextrocardia - Ganzer Fall bei Radiopaedia
Jones J, Double outlet right ventricle and dextrocardia. Case study, Radiopaedia.org (Accessed on 27 Dec 2022) https://doi.org/10.53347/rID-24087 • CC by-nc-sa 3.0 (modified)
Double outlet
right ventricle • Double outlet right ventricle with persistent left superior vena cava into coronary sinus (prenatal ultrasound) - Ganzer Fall bei Radiopaedia
Ho F, Double outlet right ventricle with persistent left superior vena cava into coronary sinus (prenatal ultrasound). Case study, Radiopaedia.org (Accessed on 27 Dec 2022) https://doi.org/10.53347/rID-73212 • CC by-nc-sa 3.0 (modified)
Congenital
coronary artery anomalies • Double outlet right ventricle, malposition of the great vessels and pulmonary hypoplasia - treated with Rastelli procedure - Ganzer Fall bei Radiopaedia
Ho F, Double outlet right ventricle, malposition of the great vessels and pulmonary hypoplasia - treated with Rastelli procedure. Case study, Radiopaedia.org (Accessed on 12 Dec 2022) https://doi.org/10.53347/rID-73230 • CC by-nc-sa 3.0 (modified)
Infant with
cyanosis and increased pulmonary blood flow on CXR. Coronal CT with contrast of the chest show a large monoatrium (above) and a large right ventricle (below) with the aorta (to the right of midline) and pulmonary artery (in the midline) arising from it. The left ventricle was small.The diagnosis was double outlet right ventricle.
pediatricimaging.org • CC-by-nc-sa 4.0
nicht verwechseln mit: double chamber right ventricle (DCRV)
Radiopaedia • CC-by-nc-sa 3.0 • de

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 . It is usually classed as a conotruncal anomaly. There is almost always a concurrent ventricular septal defect (VSD) .

Epidemiology

The estimated incidence is at ~1:10,000 births.

Pathology

Genetics

Most cases are thought to have a sporadic occurrence.

Types 

According to the position of the great vessels :

  • 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 :

  • 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
Associations

Radiographic features

Plain radiograph

Appearances on chest radiographs are variable depending on the subclassification and presence of concurrent other anomalies. May show evidence of right ventricular enlargement.

Ultrasound

On echocardiography, there is typically a lack of communication between the posterior aortic root and the anterior mitral valve leaflet.

CT/MRI

Allows direct visualization of abnormal anatomy.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu double outlet right ventricle (DORV):
ösophagotracheale
Fistel
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rechts
descendierende Aorta
Wikipedia • CC BY-SA 4.0
Ventrikelseptumdefekt
 
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