Double outlet right ventricle (DORV) is a congenital heart defect made up of several parts.

Double outlet means both of the large arteries that come from the heart – the aorta (vessel going to the body) and pulmonary artery (vessel to the lungs) – are coming from the same ventricle, or pumping chamber. Most commonly, they both come from the right ventricle so it is called double outlet right ventricle.

Frequently, there is also a hole in the wall between the two ventricles, or ventricular septal defect (VSD), which allows an “outlet” for blood from the left ventricle. When there is a VSD, the blood from the left ventricle exits the left ventricle through the VSD, goes to the right ventricle, and then can be pumped out of the heart through the aorta or the pulmonary artery.

DORV can behave very differently depending on where the VSD is located. If the VSD is near the aortic valve, then most of the blood coming from the left ventricle will go the aorta (like normal). If the VSD is near the pulmonary blood, the blood coming from the left ventricle will go to the pulmonary artery. Sometimes the VSD is very large, and the blood from the left ventricle mixes with the blood from the right ventricle before it leaves the heart through the aorta or pulmonary valve.

Often DORV is associated with abnormalities of the valves. These valve abnormalities can lead to blockage of blood to the aorta or pulmonary artery. The combination of these abnormalities determines what kind of symptoms patients will have and what surgery may be needed.

DORV Causes and Diagnosis

In most cases, there is no known cause for DORV. There are genetic syndromes seen with DORV, but in many patients there are no known genetic abnormalities.

DORV is usually diagnosed with:

  • Echocardiogram - an ultrasound of the heart.
  • Fetal echocardiogram - an ultrasound of the heart before birth
  • Others may not be detected until after birth

Sometimes, the baby may have a decrease in oxygen level or murmur that indicates the problem. Then there's a small number of babies could fail their newborn pulse oximetry screen. The diagnosis would be confirmed with an echocardiogram.

What care will my child need?

If the diagnosis of DORV is discovered before birth, then your baby’s delivery may be planned. He or she will go to the neonatal intensive care unit (NICU) after birth. An echocardiogram will be done and the baby’s breathing and oxygen level will be monitored.

The same things would happen if the diagnosis is made after birth. If the baby has close to normal oxygen levels and is not expected to have any symptoms right away, he or she can go home soon after birth with frequent monitoring as an outpatient.

Eventually the baby will need to have surgery to address the issues.

Surgery for DORV

The surgery for DORV depends on all the factors discussed above. The goal is to divide the heart into two separate ventricles, with the aorta coming from the left ventricle and the pulmonary artery coming from the right ventricle. Valve abnormalities would also be addressed.

View our pediatric cardiac surgery outcomes.

The most common surgery, done for patients with DORV and a VSD near the aortic valve, is done by sewing a patch from the ventricular septum to the aorta. This closes the VSD in a way that the blood coming from the left ventricle can go out the aorta. These patients often have abnormalities of the pulmonary valve, so the surgeon may need to fix the pulmonary valve.

Patients with a Taussig-Bing anomaly, where the VSD is near the pulmonary valve, need a complex heart surgery. In order to reach the goal of separating the two ventricles and having the aorta come from the left ventricle and pulmonary artery come from the right ventricle, multiple steps are needed. The artery attachments need to be switched and the VSD must be closed so that the left ventricle is connected to the new aorta. Most of these patients have a narrowing in the aorta that requires further surgery.

Some patients have VSDs in locations where the surgeon may not be able to divide the heart into two ventricles. In those patients, other surgeries would be done to use the heart as a single pumping chamber (see single ventricle defects).

Some patients who have surgery for DORV will need repeat surgeries in the future. Fortunately this is not the case for most patients. Some may need a cardiac catheterization to evaluate the repair or to fix areas of narrowing.

All patients need lifelong follow up with a cardiologist.

What is the long-term prognosis for DORV?

In general, the long-term prognosis for DORV is good.

Although patients may need additional surgeries or procedures, they are not typically on a lot of medications. Their activities are typically not limited.

Some patients may develop heart rhythm abnormalities later in life, requiring medications or pacemakers.

When one family member has a congenital heart defect, there is an increased risk in other family members. A genetic counselor can better determine what the risk would be for future pregnancies.

To make an appointment with a Children's Heart Program physician, please call 410-328-4348.

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