Double outlet right ventricle (DORV) is a condition when both of the large arteries that come from the heart — the aorta and pulmonary artery  — are from the same ventricle, or pumping chamber, usually the right ventricle. 

There are different heart abnormalities with DORV. There is also often a hole in the wall between the two ventricles called a ventricular septal defect (VSD) that allows blood to move from the left ventricle into the right ventricle. 

If the VSD is near the aortic valve, then most of the blood coming from the left ventricle will go the aorta, which is the correct process. But if the VSD is near the pulmonary blood, a condition known as a Taussig-Bing anomaly, the blood coming from the left ventricle will go to the pulmonary artery. 

The combination of these abnormalities determines what kind of symptoms patients will have, what surgery may be needed and when the surgery will take place.

Read our Patient Guidebook on DORV

Our Patient Guidebooks provide additional information about the conditions we treat. They are not diagnoses and should never be used instead of medical advice.

DORV – Causes and Diagnosis

In most cases, there is no known cause for DORV. There may be genetic syndromes, but in many patients there are no known genetic abnormalities. Diagnoses can occur during pregnancy or after birth.

DORV is usually diagnosed with the following tests:

  • Echocardiogram – an ultrasound of the heart
  • Fetal echocardiogram – an ultrasound of the heart before birth

The baby may have a decrease in oxygen level or a murmur that indicates a problem. A small number of babies could fail their newborn pulse oximetry screening and then will require an echocardiogram.

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.

Care Providers

The University of Maryland Children's Heart Program team that treats ASD includes a pediatric cardiac surgeon, pediatric cardiologists, pediatric and neonatal intensive care doctors and nurses, pediatric cardiac anesthesiologists, cardiac operating room staff, cardiac nurses and many others with expertise in providing this specialized cardiac care. 

If DORV is diagnosed while you are pregnant, you will see a maternal fetal medicine specialist who will treat you during your pregnancy. 

Treating DORV 

Surgery for DORV depends on the type of heart issues. The most common surgery for patients with DORV and a VSD near the aortic valve involves sewing a patch from the ventricular septum to the aorta. This closes the VSD so that blood from the left ventricle exit through the aorta. If there are abnormalities of the pulmonary valve, surgery may be required there, too.

When the VSD is near the pulmonary valve, the patient will need a more complex heart surgery to switch the arteries. The VSD must also 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. Other surgeries would be done to use the heart as a single pumping chamber.

Follow-up care may include a cardiac catheterization to evaluate the repair or to fix areas of narrowing and additional surgeries. All patients need lifelong follow up with a cardiologist.

Contact Us

  • To learn more about Maternal Fetal Heart medicine, please call 410-328-4348.
  • To make an appointment with a Children's Heart Program physician, please call 410-328-3865.
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