Treatment

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 (ductus arteriosus), 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 most common surgery used to correct transposition of the great arteries. Surgeons 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 body, instead of just to the lungs as it would do in a normal heart. Possible complications of the atrial switch operation include irregular heartbeats, baffle obstructions or leaks, and heart failure due to long-term 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 or competitive sports, because they raise blood pressure and may stress the heart.

Talk to your or your child's doctor about what type of physical activities you or your child can do, and how much and how often.

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 additional treatment.

Long-term care

You or your child will need to be monitored and treated throughout your life. Your child will be monitored and have regular follow-up appointments with a pediatric cardiologist. As your child grows older, his or her care will transition to an adult congenital cardiologist, who can monitor his or her condition over time. Transposition of the great arteries can affect your child's adult life, as it can contribute to other health problems. Adults who have congenital heart defects may need other treatments for their condition.

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 you and your fetus. In some situations, such as for women who have severe complications of their heart defect, pregnancy isn't recommended even for those with a repaired transposition.