DORV (Double Outlet Right Ventricle)

What is DORV?

Double outlet right ventricle (DORV) is a rare congenital heart defect where the pulmonary artery and the aorta — the heart’s two major arteries — both connect to the right ventricle. In a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the left ventricle. A double outlet right ventricle (DORV) creates a problem because the right ventricle carries oxygen-poor blood, which then gets circulated around the body.

Another heart condition, called a ventricular septal defect (VSD), always occurs with DORV. This is a hole in the wall (septum) that normally separates the right and left ventricles. At first, this VSD is very helpful in children with DORV because this is the only way that oxygen-rich blood can get to the aorta and into the body. The VSD allows oxygen-rich blood to pass from the left ventricle to the aorta and pulmonary artery. But even with this added oxygen, the body may still not get enough, causing the heart to work harder. This extra work can cause the heart to become enlarged.

A child with DORV may also have other heart problems, including:

• pulmonary (valve) stenosis (PVS, PS)
• transposition of the great arteries (TGA)
• pulmonary atresia
• coarctation of the aorta
• mitral valve abnormalities

How can DORV be spotted?

This condition may be suspected at the 20 week anatomy scan and then confirmed by the cardiology team by fetal echocardiogram (or echo). This is an ultrasound scan of the heart – which can show the structure of the heart and the pattern of blood flow through the VSD. This will determine how large the opening is, as well as much blood is passing through it. Sometimes, this condition may only be detected after birth.

Common symptoms of double outlet right ventricle (DORV) in babies after birth include:

• rapid breathing
• rapid heartbeat
• sweating
• disinterest in feeding or tiring while feeding
• poor weight gain
• blue color of the skin, lips and nail beds (cyanosis)
• heart murmur (detected by doctor)

How can DORV be treated?

Babies with DORV will need surgery to connect the aorta to the left ventricle and the pulmonary artery to the right ventricle, and also to close the hole in the heart. This is done by open heart surgery. The type of surgical repair will depend upon the position of the great arteries in relation to the VSD.

Surgical options include: 

• Creating a tunnel through the VSD to connect the left ventricle to the aorta
• Switching the aorta and pulmonary artery positions (if needed) to connect the pulmonary artery to the right ventricle and the aorta to the left ventricle
• Inserting a patch between the ventricles to close the hole between the VSD

Your baby will be under general anaesthetic for the procedure, and, if all goes well, will only need to remain in hospital for a week or so after surgery.

General advice & information for the future

Your baby will have a scar down the middle of the chest, and there will be small scars where drain tubes were used, but these will fade over time. Your cardiologist will advise you about any restrictions on activity that you should be aware of as your baby grows up.

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