Double Outlet Right Ventricle

What is double outlet right ventricle?

Double outlet right ventricle is a rare birth defect of the heart.

The heart has 2 upper collecting chambers (right and left atrium), 2 lower pumping chambers (right and left ventricle), 1 artery leaving the right ventricle (pulmonary artery) and 1 leaving the left ventricle (aorta).

In a normal heart, the aorta leaves the left ventricle, carrying oxygen-rich (red) blood to the body. The pulmonary artery leaves the right ventricle, carrying oxygen-poor (blue) blood to the lungs.

In double outlet right ventricle, both of the heart’s “outlets” — the pulmonary artery and the aorta — exit abnormally from the right ventricle. This causes the oxygen-rich and oxygen-poor blood to mix together before leaving the heart.

Many kinds of heart defects fall into the category of double outlet right ventricle. They all share the feature that the aorta and pulmonary artery both exit from the right ventricle. In addition, other parts of the heart may be different.

In double outlet right ventricle, there is almost always a hole in the septum, or wall, between the right and left ventricles, called a ventricular septal defect, that allows blood to mix within the heart. Some children also have a hole in the septum between the right and left atria called an atrial septal defect. They can have other defects too, such as abnormal heart valves (pulmonary stenosisaortic stenosis or mitral valve abnormalities) or abnormal arteries (coarctation of the aorta or pulmonary atresia).

  • This is a congenital defect. Doctors do not know why it happens. They think it occurs very early in the pregnancy, when the baby’s heart is forming, before a woman may even know she is pregnant.

    Double outlet right ventricle is rare. About 1 in every 10,000 babies has this condition.

Double Outlet Right Ventricle at Seattle Children’s

  • Seattle Children’s Heart Center is one of the best pediatric cardiology programs in the United States and is the top-ranked program in the Northwest, according to U.S. News & World Report. With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    Our heart team has treated many children with double outlet right ventricle. We have extensive experience with the surgery these patients require. We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who undergo heart surgery.

    Seattle Children’s has been treating children since 1907. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.

    The Adult Congenital Heart Disease Program shared by Seattle Children’s and the University of Washington can help with care throughout your child’s life.

  • Children with double outlet right ventricle receive compassionate, comprehensive care through our Single Ventricle Program. We bring together experts in cardiology, nutrition, social work, feeding therapy and neurodevelopment to support your child’s health during the vulnerable transition between surgeries.

  • Care often begins before a child is born, when their condition is diagnosed through our Prenatal Diagnosis and Treatment Program.

    We also have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready. This program was developed by Seattle Children’s and the University of Washington to help with care throughout your child’s life.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s cardiologist, you are connected with surgeons, Cardiac Intensive Care Unit doctors, cardiac anesthesiologists, neonatologists, nurses, cardiac sonographers, dietitians, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child’s health needs and help your family through this experience.

    We’re committed to your child’s overall health and well-being. We’ll discuss treatments in ways you can understand and involve you in every decision.

    Read more about the supportive care we offer.

Symptoms of Double Outlet Right Ventricle

A wide range of heart problems fit into the category of double outlet right ventricle. Symptoms will vary, depending on how the blood moves through and out of the heart.

In many patients, double outlet right ventricle allows oxygen-poor (blue) blood to leave the right ventricle through the aorta and get pumped out to your child’s body. So this heart defect causes symptoms linked to lower-than-normal oxygen supply. Your child may appear to have bluish skin, for example.

Also, in children with double outlet right ventricle, the lungs may get more blood than usual. This can cause your baby to breathe very fast or work hard to breathe. It may result in high blood pressure in the lungs and cause heart failure.

Your child may have symptoms like these: 

  • Bluish skin, lips or nailbeds (cyanosis) or pale skin
  • Trouble feeding
  • Failure to thrive
  • Fast breathing or working hard to breathe
  • Sweating more than normal

Diagnosing Double Outlet Right Ventricle

To diagnose this condition, your doctor will examine your child, check their heartbeat and listen to their heart. In children with double outlet right ventricle, doctors might be able to hear a heart murmur – the sound of blood moving in the heart in a way that’s not normal.

The doctor will ask for details about your child’s symptoms, their health history and your family health history.

Your child will also need tests that provide more information about how their heart looks and works. These will most likely include: 

Your child may need other tests that provide more information about how their heart looks and works. These tests might include: 

Treating Double Outlet Right Ventricle

This heart defect will not get better on its own. Children with double outlet right ventricle need surgery to correct the condition. The exact procedures and timing depend on your child’s condition, including how severe it is and whether they have other heart defects too, but is usually performed within the first several days to months of life. It may require more than 1 surgery or procedure.

Your child will need lifelong follow-up with a cardiologist who specializes in birth defects of the heart. To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

  • In general, children need surgery to do the following: 

    • Direct oxygen-rich (red) blood, which comes from the lungs, into the aorta. From there, it can be delivered to the rest of their body.
    • Direct oxygen-poor (blue) blood, which comes back from the body, out of their aorta. This blood needs to go to their lungs first to pick up oxygen.
    • Close any additional holes in the heart to keep the oxygen-rich (red) blood separate from the oxygen-poor (blue) blood. 

    How these objectives are achieved depends on the kind of double outlet right ventricle your child has. The cardiologist and surgeon taking care of your child will talk with you about the best approach for your child’s heart.

  • To get ready for surgery, your child may need medicines like these: 

    • Diuretics, which help the kidneys rid the body of extra water
    • Angiotensin-converting enzyme (ACE) inhibitors, which lower blood pressure
    • Digoxin, which makes the heart beat slower and with more force
  • In rare cases, the heart cannot be repaired and a heart transplant may be recommended. The heart transplant team at Seattle Children’s is one of the best in the nation, caring for children with this or other heart problems that cannot be controlled using other treatments. Read more about our Heart Transplant Program or see our statistics and outcomes.

    • After undergoing two heart transplants before she was 4 years old, Mya is now thriving. Read the full story.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

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