Conditions

Double-Outlet Right Ventricle

  • If this is a medical emergency, call 911.

    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • No pre-referral work-up is required for most conditions. If you have already done testing such as an EKG, Holter monitor or echocardiogram, please fax this information as well as relevant clinic notes and the NARF to 206-985-3121 or 866-985-3121 (toll-free).
    • Your patient will be seen as quickly as possible by the provider who is the best match for managing the current problem.
    • View our complete Heart Center referral information.

What is double-outlet right ventricle?

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

Normally, the heart has 2 upper chambers (right and left atrium) and 2 lower chambers (right and left ventricle). The pulmonary artery leaves the right ventricle, carrying blue (oxygen-poor) blood to the lungs. The aorta leaves the left ventricle, carrying red (oxygen-rich) blood to the body.

In double-outlet right ventricle, both of the heart’s “outlets” — the pulmonary artery and the aorta — leave from the right ventricle.

heart illustration.jpgThis is a normal heart. In double-outlet right ventricle, both the pulmonary artery and the aorta leave the right ventricle.

Other parts of the heart may also be different. Children with this condition almost always have a hole in the wall between the ventricles (ventricular septal defect). This lets oxygen-rich and oxygen-poor blood mix within the heart.

Some children also have a hole between the atria (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).

What causes double-outlet right ventricle?

Babies are born with this heart defect. Doctors do not know why it happens. They think it occurs early in the pregnancy when the baby’s heart is forming.

How common is double-outlet right ventricle?

Doctors consider this a rare heart defect. About 1 in every 10,000 babies has double-outlet right ventricle.

Double-Outlet Right Ventricle at Seattle Children’s

Consistently ranked one of the nation's best cardiology and cardiac surgery programs by U.S. News and World Report.

    • The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many babies with this condition.
    • Our doctors and surgeons are experts in the treatments your child may need. These may include surgery to change blood flow in your child’s heart, medicine and a heart transplant.
    • Our 4 pediatric cardiac surgeons perform more than 500 procedures yearly. Seattle Children’s surgical outcomes are among the best in the nation year after year.
    • We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery. General anesthesia is a medicine we give to people before surgery so they are fully asleep during the procedure.
    • The transplant team does several heart transplants each year for children with this or other heart problems that cannot be controlled using other treatments. 
    • Your child’s team includes experts from other areas of Seattle Children’s based on their needs, like doctors who specialize in newborns (neonatologists) or lung health (pulmonologists).
    • If your developing baby is diagnosed with double-outlet right ventricle before birth, Seattle Children’s Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.
    • Your child’s treatment plan is custom-made. We plan and carry out their treatment based on the specific details of their heart defects. We closely check your child’s needs to make sure they get the care that is right for them at every age.
    • We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
    • We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
    • Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
    • Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
    • Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make your experience as smooth as possible.
    • Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
    • 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 depend on how the blood moves through and out of the heart.

In many children, double-outlet right ventricle allows oxygen-poor 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 oxygen supply than normal. For example, your child’s skin may appear blue or purple tinged, mottled (different shades or colors), grayish or paler than usual.

Also, in children with double-outlet right ventricle, the lungs may get more blood than usual. This can cause your child 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: 

  • Cyanosis
  • Trouble feeding
  • Poor weight gain or slow growth (failure to thrive)
  • Fast breathing or working hard to breathe
  • Sweating more than normal

Diagnosing Double-Outlet Right Ventricle

  • Usually, doctors can diagnose double-outlet right ventricle when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing baby’s heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease.

    Your obstetrician may refer you for a fetal echo if your family has a history of congenital heart disease or if a routine prenatal ultrasound shows a problem.

    Our Fetal Care and Treatment Center team can care for you when you are pregnant if your developing baby has a known or suspected problem.

  • To diagnose this condition, your child’s doctor will examine your child, check their heartbeat and pulse 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 need tests that provide more information about how their heart looks and works. These will most likely include:

    Your child may also need these tests: 

Treating Double-Outlet Right Ventricle

Children with double-outlet right ventricle need surgery to correct the condition. The exact procedures and timing depend on your child’s condition, how severe it is and whether they have other heart defects too. Usually, surgery is done within the first several days to months after birth. Often children need more than 1 surgery or procedure.

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

    • Direct oxygen-rich blood, which comes from the lungs, into the aorta. From there, it can be carried to the rest of their body.
    • Keep oxygen-poor blood, which comes back from the body, out of the aorta. This blood needs to go to your child’s lungs first to pick up oxygen.
    • Close any other abnormal holes in the heart. This step keeps oxygen-rich blood from mixing with oxygen-poor blood. 

    How doctors achieve these goals 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.

    Most children have surgery that allows both ventricles to pump blood where it needs to go. For children with some complex defects, the best approach is surgery that allows one ventricle to do all the pumping. The team in our Single Ventricle Program provides specialized care for these children.

    All children with double-outlet right ventricle will need lifelong follow-up with a cardiologist who specializes in congenital heart defects.

  • 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 doctors may recommend a heart transplant. 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 and see our exceptional outcomes.

    Mya and Jamie.jpg“I wondered if her care team could keep her alive despite all she went through, and they did an amazing job. I can’t thank them enough. They’re like a second family to her.” ~ Jamie Gonzalez, whose daughter Mya is thriving after 2 heart transplants

Contact Us 

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

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For Healthcare Professionals

  • If this is a medical emergency, call 911.

    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • No pre-referral work-up is required for most conditions. If you have already done testing such as an EKG, Holter monitor or echocardiogram, please fax this information as well as relevant clinic notes and the NARF to 206-985-3121 or 866-985-3121 (toll-free).
    • Your patient will be seen as quickly as possible by the provider who is the best match for managing the current problem.
    • View our complete Heart Center referral information.