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Transposition of the great arteries

Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly and your child faces serious complications or death without treatment.

Transposition of the great arteries is usually detected within the first hours to weeks of life.

Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. Having a baby with transposition of the great arteries can be alarming, but with proper treatment, the outlook is promising.

Symptoms Causes Risk factors Complications Prevention

Transposition of the great arteries symptoms include:

  • Blue color of the skin (cyanosis)
  • Shortness of breath
  • Lack of appetite
  • Poor weight gain

When to see a doctor

Transposition of the great arteries is often detected as soon as your baby is born or during the first week of life. If signs and symptoms didn't appear in the hospital, seek emergency medical help if you notice that your baby develops bluish discoloration of the skin (cyanosis), especially involving the lips and face.

Transposition of the great arteries occurs during fetal growth when your baby's heart is developing. Why this defect occurs is unknown in most cases.

Normally, the pulmonary artery — which carries blood from your heart to your lungs to receive oxygen — is attached to the lower right chamber (right ventricle). From your lungs, the oxygen-rich blood goes to your heart's upper left chamber (left atrium), through the mitral valve into the lower left chamber (left ventricle). The aorta is normally attached to the left ventricle. It carries oxygen-rich blood out of your heart back to the rest of your body.

In transposition of the great arteries, the positions of the pulmonary artery and the aorta are switched. The pulmonary artery is connected to the left ventricle, and the aorta is connected to the right ventricle. Oxygen-poor blood circulates through the right side of the heart and back to the body without passing through the lungs. Oxygen-rich blood circulates through the left side of the heart and right back into the lungs without being circulated to the rest of the body.

Circulation of oxygen-poor blood through the body causes the skin to have a blue tint (cyanosis). Because of this, transposition of the great arteries is called a congenital cyanotic heart defect.

Although some factors, such as rubella or other viral illnesses during pregnancy, maternal age over 40, or maternal diabetes, may increase the risk of this condition, in most cases the cause is unknown.

Although the exact cause of transposition of the great arteries is unknown, several factors may increase the risk of a baby being born with this condition, including:

  • A history of German measles (rubella) or another viral illness in the mother during pregnancy
  • A family history of transposition of the great arteries or another congenital heart defect
  • Poor nutrition during pregnancy
  • Drinking alcohol during pregnancy
  • A mother older than age 40
  • A mother who has poorly controlled diabetes
  • Down syndrome in the baby

Potential complications of transposition of the great arteries include:

  • Lack of oxygen to tissues. Your baby's tissues will receive too little oxygen, (hypoxia). Unless there's some mixing of oxygen-rich blood and oxygen-poor blood within your baby's body, he or she won't be able to survive.
  • Heart failure. Heart failure — a condition in which the heart can't pump enough blood to meet the body's needs — may develop over time because the right ventricle is pumping under higher pressure than usual. This added stress may make the muscle of the right ventricle stiff or weak.
  • Lung damage. The lack of oxygenated blood causes damage to the lungs, making breathing difficult.

Surgery is required for all babies with transposition of the great arteries early in life, usually within the first week. Complications of surgery to correct transposition of the great arteries may occur later in life, including:

  • Narrowing of the arteries that supply blood to the heart (coronary arteries)
  • Heart rhythm abnormalities (arrhythmias)
  • Heart muscle weakness or stiffness leading to heart failure
  • Leaky heart valves

In most cases, transposition of the great arteries can't be prevented. If you have a family history of heart defects or if you already have a child with a congenital heart defect, before getting pregnant consider talking with a genetic counselor and a cardiologist experienced in congenital heart defects.

Additionally, it's important to take steps to have a healthy pregnancy. For example, before becoming pregnant, be sure you're up to date on all of your immunizations, and start taking a multivitamin with 400 micrograms of folic acid.

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