Treatment for DORV aims to improve blood flow from the heart to the body and lungs and open narrowed pathways. When possible, treatment also creates separate pathways so oxygen-rich blood flows to the body. Oxygen-poor blood flows to the lungs to help it pick up more oxygen.
Some newborns need treatment soon after birth to improve blood flow while the care team plans surgery. Prostaglandin E1 is a medicine that is given through an IV. It keeps a blood vessel called the ductus arteriosus open. This blood vessel is open before birth and usually closes soon after birth. In some babies with DORV, keeping it open allows more blood to reach the lungs or the rest of the body while the care team makes a treatment plan.
Depending on the heart structure, a baby also may need a procedure called atrial septostomy. This procedure uses a thin, flexible tube called a catheter to make or enlarge an opening between the two upper chambers of the heart. The opening allows blood to mix between these chambers, which can help increase the amount of oxygen in the blood that reaches the body. Some babies may need other procedures or an earlier surgery before DORV can be repaired.
Surgery
Most babies with DORV need surgery to improve how blood flows from the heart to the lungs and the rest of the body. The type of surgery your child needs depends on your child's heart structure.
The surgical team looks at:
- Where the VSD is located.
- The positions of the aorta and the pulmonary artery.
- The size of both ventricles and how well they pump.
- How well the heart valves work.
- Narrowing in the pathways that carry blood from the heart.
- Blood vessels that supply the heart muscle, called the coronary arteries.
These details help the team decide what type of repair is possible and right for your child.
Intraventricular tunnel repair
This surgery may be done when the VSD is close to the aorta. During an intraventricular tunnel repair, a pathway is created inside the heart from the left ventricle through the VSD and into the aorta. This allows the left ventricle to pump blood through the aorta to the body.
If the pathway from the right ventricle to the pulmonary artery is narrow, it also may need to be widened or rebuilt.
Arterial switch operation
An arterial switch operation may be part of the repair procedure for babies with Taussig-Bing type or other types of DORV in which the VSD is close to the pulmonary artery. During this surgery, a surgeon moves the aorta and pulmonary artery and creates a pathway through the VSD to direct blood from the left ventricle to the aorta. This allows the left ventricle to pump blood through the aorta to the body. It also allows the right ventricle to pump blood through the pulmonary artery to the lungs.
The blood vessels that supply the heart muscle, called the coronary arteries, also are moved to the aorta.
Rastelli procedure
The Rastelli procedure may be used when a pathway can be made from the left ventricle to the aorta, but the pathway from the right ventricle to the pulmonary artery is too narrow. This may happen when the valve that controls blood flow from the right ventricle to the pulmonary artery is narrowed. This is called pulmonary valve stenosis.
During the Rastelli procedure, a surgeon creates a pathway from the left ventricle through the VSD and into the aorta. This allows the left ventricle to pump blood to the body. A tube with a valve inside, called a conduit, is placed between the right ventricle and the pulmonary artery. The conduit is the pathway that allows blood to flow from the right ventricle to the pulmonary artery so the right ventricle can pump blood to the lungs.
The conduit does not grow as a child grows. Over time, it can narrow, harden or leak. Another catheter procedure or surgery may be needed to repair or replace the conduit.
Single-ventricle surgery options
Sometimes, the heart's structure does not allow both ventricles to work separately. When this happens, the care team may consider what is called a single-ventricle pathway. This is a more complex treatment that involves several surgeries done in stages to change how blood moves through the heart and lungs. After these surgeries, one ventricle does most of the heart's pumping work.
A single-ventricle pathway may be considered when:
- One ventricle is very small.
- A heart valve has complex attachments.
- The VSD is too far from the aorta or pulmonary artery to create the pathway needed for a two-ventricle repair.
Other complex types of repairs
Other, more complex types of surgery may be done in rare situations based on the unique features of DORV in some babies. Cardiologists and surgeons recommend the surgery that is right for each child.
Sept. 10, 2026