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Double outlet right ventricle (DORV) is a congenital heart defect in which both the aorta and pulmonary artery arise entirely or predominantly from the morphologic right ventricle, instead of the aorta arising from the left ventricle. A ventricular septal defect (VSD) is always present and is essential for systemic perfusion.
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In 'Double Outlet Right Ventricle' (DORV) the two Great Arteries (Aorta and Pulmonary Artery) both originate from the right ventricle and blood from the left ventricle passes across a VSD into the RV to reach the great arteries. The lung circulation is often exposed to very high pressure and increased blood flow (as with a large VSD). There are many different varieties of this abnormality.
****Cardiology : Double Outlet Right Ventricle. Rch.org.au. Published 2025. Accessed July 11, 2025. https://www.rch.org.au/cardiology/heart_defects/Double_Outlet_Right_Ventricle/
Anatomic Variants of DORV
| Variant | Description / Mimics |
|---|---|
| DORV with subaortic VSD | Blood from LV crosses VSD to aorta → mimics VSD physiology |
| DORV with subpulmonary VSD | Aorta arises more anterior; mimics TGA physiology (aka Taussig-Bing anomaly) |
| DORV with doubly committed VSD | VSD lies between aorta and pulmonary artery |
| DORV with remote/non-committed VSD | VSD far from both arteries → causes obstruction and complex routing |
Associated Anomalies
| Age | Findings |
|---|---|
| Neonatal | Cyanosis, poor feeding, respiratory distress |
| Infants/children | Heart failure, tachypnea, diaphoresis, failure to thrive, murmur |
| Older children (rare) | Clubbing, polycythemia (if untreated) |