Double Outlet Right Ventricle

What is a double outlet right ventricle?

Double outlet right ventricle (DORV) is a rare cardiac birth defect where both great arteries (the pulmonary artery and the aorta) arise from the right ventricle. No great arteries are connected to the left ventricle (the chamber that normally pumps blood to the body).

Heart defects associated with DORV

Most DORV defects are found with additional heart defects which may include:

• Ventricular septal defect (VSD)
• Obstruction of the aortic or pulmonary valve
• Abnormal positioning of the great arteries

There are different types of double outlet ventricles based on the location of the ventricular septal defect and the position of the great arteries. Prenatal diagnosis is important as babies with DORV may have additional organ or chromosomal abnormalities, such as 22q11 Deletion Syndrome.

Treatment for double outlet right ventricles

Depending on their stability, babies with DORV require heart surgery in the first few days to months of life to correct the heart defect.

In the model below:

A double outlet right ventricle (DORV) (1) forms when the aorta (2) and the pulmonary artery (3) both arise from the right ventricle (4). This creates a ventral septal defect (VSD) (5), which is a hole in the heart wall (septum) that separates the right and left ventricles. This opening (5) allows deoxygenated blood from the body and oxygenated blood from the lungs to mix. As a result, the other organs in the body do not receive enough oxygen.

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