Transposition of the Great Arteries

Overview

What is a transposition of the great arteries?

Transposition of the great arteries is a congenital heart defect caused when a child’s heart develops incorrectly in the womb. This condition occurs when the heart’s two most important blood vessels—the aorta and the pulmonary artery—are reversed.

In a healthy heart, the aorta pumps oxygen-rich blood to the body, and the pulmonary artery sends blood to the lungs (where the blood absorbs oxygen so that the oxygen-rich blood can then be pumped to the body). With this condition, not enough oxygen-rich blood circulates from the lungs to the body. 

Children born with transposition of the great arteries are usually diagnosed shortly after birth, but some may have been diagnosed by fetal ultrasounds before birth. Babies with the condition often have a blue tint to their skin, a sign of cyanosis because oxygen-rich blood has no way to get to the body.

The most common form of transposition

Most infants with D-type transposition will have symptoms and be diagnosed shortly after birth. In this congenital abnormality, no oxygen-rich blood goes to the body and:

• The aorta and pulmonary artery are switched, but the ventricles developed in the correct place.
• Blood that returns to the heart from the body travels from the right atrium to the right ventricle, where it is pumped out to the body again, without ever going to the lungs.
• Blood from the lungs comes back to the left atrium and then to the left ventricle, where it is pumped back out to the lungs through the pulmonary artery.

Programs & Treatments

How do we treat transposition of the great arteries?

At Children’s Hospital Colorado, our pediatric cardiologists begin treating transposition of the great arteries right after birth.

Medication

If your child is born with D-type transposition, he or she may require medication and surgery very soon after birth. Most often, children are treated in the intensive care unit with intravenous (IV) medication and oxygen. Sometimes a breathing tube may be necessary for a short time.

If the hole between the atria (atrial septal defect) is not large enough, a minor procedure called an atrial septectomy or “Rashkind” procedure may be necessary to widen it so oxygen-rich and oxygen-poor blood can mix inside the heart. This is usually performed in the intensive care unit by your child’s cardiologist.

Surgery

The only treatment for D-type transposition of the great arteries is heart surgery. The best and most common surgery is the arterial switch procedure, which is performed by pediatric cardiac surgeons. During this surgery, the pulmonary artery and aorta are switched back to their normal positions and the coronary arteries are also switched along with the large blood vessels. Any holes in the heart will also be repaired. 

Learn more about pediatric heart surgery at Children’s Colorado.

After surgery, most children with transposition of the great arteries go on to live normal, healthy lives, but should be followed by a cardiologist for some time after surgery. 

Signs & Symptoms

What are some of the symptoms of transposition of the great arteries?

Children with transposition of the great arteries are usually diagnosed within a few weeks of birth.

The most common symptom is cyanosis, which is a blue tint to the skin that is caused when not enough oxygen gets to a child’s body.

Other symptoms to look for are:

• Trouble breathing
• Clubbed feet and toes
• Poor feeding
• Sweating during feeding

Who is at risk of transposition of the great arteries?

Transposition of the great arteries is a very rare congenital heart defect. Boys are more susceptible to D-type transposition, although the reason is not known. Children with a family history of transposition are more likely to develop the condition.

Diagnosis & Tests

How is transposition of the great arteries diagnosed?

If your doctor suspects your child has a transposition of the great arteries, he or she will order further tests. These will help a cardiologist identify the condition and evaluate its severity.

Common tests include:

• Chest x-ray
• EKG
• ECHO
• Diagnostic cardiac catheterization  

Helpful resources

To learn more about transposition of the great arteries, visit these websites: