Conditions

Transposition of the Great Arteries

  • 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 transposition of the great arteries?

Transposition of the great arteries is a serious birth defect where your baby’s body does not get enough oxygen through their blood. This happens when 2 large blood vessels leaving their heart are transposed (change places with each other).

Normally, oxygen-poor (blue) blood flows out of the right ventricle of the heart through the pulmonary artery to the lungs. The lungs oxygenate (give oxygen to) the blood. Then, the oxygen-rich (red) blood returns to the heart. From there, it flows out of the left ventricle through the aorta to the body.

In babies with this birth defect, the pulmonary artery and aorta are reversed. Oxygen-poor blood flows out of the right ventricle through the aorta to the body. Then, it returns to the right ventricle without going to the lungs. Oxygen-rich blood flows out of the left ventricle through the pulmonary artery to the lungs. Then, it returns to the left ventricle without going to the rest of the body. As a result, your baby’s body is not getting enough oxygen.

Some babies with this condition have other heart defects too, like atrial septal defect (ASD) or ventricular septal defect (VSD).

Heart Illustration

This is a normal heart. It sends oxygen-poor blood to the lungs and oxygen-rich blood to the body. In transposition of the great arteries, the pulmonary artery and aorta are reversed. As a result, the heart sends oxygen-rich blood to the lungs and oxygen-poor blood to the body.

Transposition of the Great Arteries at Seattle Children’s

Consistently ranked one of the nation's best cardiology and cardiac surgery programs by U.S. News and World Report.Seattle Children’s outcomes for arterial switch surgery are among the best in the nation year after year.

    • Our team of more than 40 pediatric cardiologists has diagnosed and treated this condition in many babies — and we provide any ongoing care children need as they grow into young adults.
    • Our pediatric cardiac surgeons perform more than 500 procedures yearly. Each surgeon has deep experience correcting transposed arteries and related heart conditions in newborns and babies early in life.
    • We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.
    • 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 transposition of the great arteries before birth, our Prenatal Diagnosis and Treatment team works closely with you and your family to plan and prepare for the care your baby will need.
    • Your child’s treatment plan is custom-made. We plan and carry out their surgery based on the specific details of their heart defect, like if they have ASD or VSD along with transposed arteries. 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 the journey 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.
    • Seattle Children’s takes part in PC4, the Pediatric Cardiac Critical Care Consortium. This is a national group of leaders in treating children with serious heart conditions. PC4 focuses on advancing care by sharing and analyzing data to find what works best.
    • With other institutions in PC4, we are studying how long children need a breathing tube and how long they stay in the Cardiac Intensive Care Unit and the hospital after surgery for transposition of the great arteries. The goals are to reduce differences in care between children when it is safe to do so and get more children safely home sooner.

Symptoms of Transposition of the Great Arteries

Most babies with transposition of the great arteries are cyanotic (have a blue or purple tint to their skin, mouth or tongue) soon after birth due to a low oxygen level. They may also seem to be working hard to breathe and have trouble feeding. A few babies may appear quite normal and healthy for the first day or 2 after birth.

If their ductus arteriosus is helping oxygen-rich and oxygen-poor blood mix so that some oxygen can get to their body, their symptoms will get worse as the ductus begins to close (a normal step after birth). If they have a patent ductus arteriosus (ductus that does not close on its own), some oxygen-rich blood will continue to get through.

If they have a large ASD or VSD, their symptoms may be less severe. This hole between chambers of the heart provides a path for some oxygen-rich blood to get from their lungs to the rest of their body.

Diagnosing Transposition of the Great Arteries

Sometimes doctors can diagnose transposition of the great arteries in a developing baby before birth. They might first see something different about your baby’s great arteries on standard prenatal ultrasound. To learn more, you might have a fetal echocardiogram (an ultrasound done to check the heart more closely). Seattle Children’s Prenatal Diagnosis and Treatment team can care for you when you are pregnant if your developing baby has a known or suspected problem.

Most babies who are not diagnosed prenatally are diagnosed as newborns, before they go home from their birth center, because they show symptoms. A small number of newborns do not have clear symptoms right away, but a simple newborn screening test (pulse oximetry) shows their body is not getting as much oxygen as it needs. If this screening test shows a concern, more detailed testing will be done.

To diagnose this condition after birth, your baby’s doctor will examine your child and use a stethoscope to listen to their heart.

The doctor will consider your baby’s symptoms, their health history and your family health history.

Your baby will also need tests that provide information about how their heart and blood vessels look and work. These may include: 

Treating Transposition of the Great Arteries

Babies with transposed great arteries need surgery soon to change their blood flow. The surgery sends oxygen-poor blood to their lungs and oxygen-rich blood to the rest of their body.

  • For most babies, surgeons switch the transposed arteries to the correct places in the heart. They connect the pulmonary artery to the right ventricle and the aorta to the left ventricle. The coronary arteries, which bring blood to the heart muscle, also need to be moved so that they stay connected to the aorta. This is called arterial switch surgery. (See our outcomes for arterial switch surgery.)

    Some babies need a different surgery based on their body. For example, your baby might need a different surgery if one of their heart valves is small (stenotic) or their coronary arteries are very complex. Some of these other surgeries are called the Mustard, Senning, Rastelli and Nikaidoh procedures (named after the surgeons who developed them). Your baby’s team will talk with you about the surgery your baby needs and why.

  • Before your baby has surgery, they may need other steps. These may include: 

    • Getting medicine (prostaglandin) to keep their ductus arteriosus from closing
    • Having cardiac catheterization to enlarge a small hole between the atria that normally closes shortly after birth (patent foramen ovale) using a balloon 

    These steps help keep your baby’s condition stable in the days between birth and surgery. This can improve the outcome of surgery.

  • If your baby has other heart defects, like ASD or VSD, they will typically be repaired during the same surgery.

    After surgery, your child will need lifelong follow-up visits with a cardiologist to check that they are doing well and to see whether they need any further treatment.

    Children born with complex heart conditions are at risk for problems with the growth and development of their brain and central nervous system. At Seattle Children’s, we have a Cardiac Neurodevelopmental Clinic to assess your child and, if needed, create a plan to help them reach their full potential.

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.