Transposition of the great arteries

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By Mayo Clinic staff

All infants with transposition of the great arteries need surgery to correct the defect.

Before surgery
Your baby's doctor may recommend several options to help manage the condition before corrective surgery. They include:

  • Medication. The medication prostaglandin E1 (Alprostadil) helps keep the connection between the aorta and pulmonary artery open, increasing blood flow and improving mixing of oxygen-poor and oxygen-rich blood until surgery can be performed.
  • Atrial septostomy. This procedure — usually done using cardiac catheterization rather than surgery — enlarges a natural connection between the heart's upper chambers (atria). It allows for the oxygen-rich and oxygen-poor blood to mix and results in improved oxygen delivery to your baby's body.

Surgery
Surgical options include:

  • Arterial switch operation. This is the surgery that doctors most often use to fix transposition of the great arteries. Doctors usually perform this surgery within the first month of life.

    During an arterial switch operation, the pulmonary artery and the aorta are moved to their normal positions: The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The coronary arteries also are reattached to the aorta.

    If your baby has a ventricular septal defect or an atrial septal defect, those holes usually are closed during surgery. In some cases, however, the doctor may leave small ventricular septal defects to close on their own.

  • Atrial switch operation. In this surgery, the surgeon makes a tunnel (baffle) between the heart's two upper chambers (atria). This diverts the oxygen-poor blood to the left ventricle and the pulmonary artery and the oxygen-rich blood to the right ventricle and the aorta. With this procedure, the right ventricle must pump blood to the entire body, instead of just to the lungs as it would do in a normal heart. Possible risks from the atrial switch operation include irregular heartbeats, baffle obstructions or leaks, and problems with right ventricle function.

After surgery
After corrective surgery, your baby will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease to monitor his or her heart health. The cardiologist may recommend that your child avoid certain activities, such as weightlifting, because they raise blood pressure and may stress the heart.

If your child had an atrial switch operation, he or she may need to take antibiotics before dental procedures and other surgical procedures to prevent infections. Those who've had the arterial switch operation generally won't need preventive antibiotics.

Many people who undergo the arterial switch operation don't need additional surgery. However, some complications, such as arrhythmias, heart valve leaks or problems with the heart's pumping, may require treatment.

Pregnancy
If you had transposition of the great arteries repaired in your infancy, it's possible for you to have a healthy pregnancy, but specialized care may be necessary. If you're thinking about becoming pregnant, talk to your cardiologist and obstetrician before conceiving. If you have complications such as arrhythmias or serious heart muscle problems, pregnancy may pose risks to both the mother and the fetus. In some situations, such as for women with severely reduced heart function, pregnancy isn't recommended even for those with a repaired transposition.

References
  1. Transposition of the great arteries. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/birthdefects/TranspositionGreatArteries.htm. Accessed Feb. 9, 2010.
  2. d-Transposition of the great arteries. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=11074. Accessed Feb. 9, 2010.
  3. Transposition of the great arteries. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/print/sec19/ch287/ch287h.html. Accessed Feb. 9, 2010.
  4. Congenital heart defects. The March of Dimes. http://www.marchofdimes.com/professionals/14332_1212.asp. Accessed Feb. 9, 2010.
  5. Aboulhosn JA, et al. Congenital heart disease in adults. In: Fuster V, et al. Hurst's The Heart. 12th ed. New York, N.Y.: McGraw-Hill Medical; 2008. http://www.accessmedicine.com/content.aspx?aID=3073065. Accessed Feb. 11, 2010.
  6. Grogan M (expert opinion). Mayo Clinic, Rochester, Minn. Feb. 16, 2010.
DS00733 May 6, 2010

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