Double outlet right ventricle (DORV) is a rare form of congenital heart disease. In a normal heart, the pulmonary artery sends oxygen-poor blood from the right ventricle into the lungs, and the aorta sends oxygen-rich blood from the left ventricle to the rest of the body. Together, the pulmonary artery and the aorta are known as the great arteries. But with DORV, both of the great vessels connect to the right ventricle.

Infants born with DORV usually have a ventricular septal defect (VSD). A VSD is a hole in the wall that separates the lower chambers of the heart, allowing some of the blood to get pushed into the right ventricle instead of flowing normally to the rest of the body.

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. Still, the amount of oxygen-rich blood that reaches the body is not enough. The ventricles can become overworked. In an attempt to supply the body with enough blood, the left ventricle may pump harder and faster than normal. This extra work can cause the heart to become enlarged.

What are the different types of DORV?

There are 4 types of DORV. The type of DORV that occurs depends on where the VSD is located in relation to the great arteries.

• DORV with a subaortic VSD. This means that the VSD is located just below the aorta. With this type of DORV, blood flows from the left ventricle, through the VSD, and into the aorta. Blood from the right ventricle flows mainly into the pulmonary artery.
• DORV with a subpulmonary VSD. This means that the VSD is located below the pulmonary artery. This type of DORV is also called Taussig-Bing anomaly. With this type of DORV, oxygen-rich blood flows from the left ventricle, through the VSD, and into the pulmonary artery. Oxygen-poor blood from the right ventricle flows mainly into the aorta.
• DORV with a doubly committed VSD. This means that there are 2 VSDs–one below the aorta and one below the pulmonary artery.
• DORV with a non-committed (or remote) VSD. This means that the VSD is not located near the aorta or the pulmonary artery.

What are the symptoms of DORV?

Where the VSD is located will determine the signs and symptoms of DORV. The most common signs and symptoms of DORV may not appear for days or weeks after birth.

• Shortness of breath
• Heart murmur
• Sweating
• Extreme tiredness
• Fast or difficult breathing
• A bluish color to the skin (cyanosis)
• No interest in feeding
• Loss of weight or no weight gain

How is DORV treated?

The treatment for DORV depends on the type of DORV the child has, the child’s age, and how serious the DORV is. For most cases of DORV, surgery is the treatment of choice. The main goals of surgery are to connect the aorta to the left ventricle and the pulmonary artery to the right ventricle.

One type of surgery that is used to correct DORV is called intraventricular repair. With intraventricular repair, surgeons create a tunnel (called a baffle). The baffle is created through the VSD and connects the aorta to the left ventricle.

Another type of surgery is called an arterial switch, to reverse the positions of the arteries, so that the pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. Any VSDs are also closed during this surgery.