Transposition of the great arteries is most often diagnosed after a baby is born. However, signs of the condition may be detected before birth during a routine pregnancy ultrasound. If so, a fetal echocardiogram may be recommended. A fetal echocardiogram uses sound waves to create detailed images of the unborn baby's heart. It can help a health care provider confirm the diagnosis of transposition of the great arteries.
After birth, a health care provider may suspect a heart defect such as transposition of the great arteries if the baby has blue skin, a weak pulse or trouble breathing.
The care provider may also suspect a heart problem if an irregular whooshing sound (heart murmur) is heard when listening to the baby's heart.
A physical exam alone can't accurately diagnose transposition of the great arteries. One or more of the following tests are necessary for an accurate diagnosis:
- Echocardiogram. An echocardiogram is an ultrasound of the heart. It uses sound waves to create moving images of the heart in motion. An echocardiogram can show the position of the aorta and the pulmonary artery. The test can also identify other associated congenital heart defects, such as a ventricular septal defect, atrial septal defect or patent ductus arteriosus.
- Chest X-ray. Although a chest X-ray doesn't provide a definitive diagnosis of transposition of the great arteries, it does allow the provider to see the baby's heart size and determine if blood flow is collecting in the lungs.
- Electrocardiogram (ECG or EKG). This simple, painless test records the electrical activity of the heart. Sticky patches (electrodes) are placed on the chest and sometimes the arms and legs. Wires connect the electrodes to a computer, which displays the test results. An ECG can show if the heart is beating too fast, too slow or not at all.
All infants with complete transposition of the great arteries (D-TGA) need surgery to correct the congenital heart defect. Treatment for congenitally corrected transposition (L-TGA) depends on when the condition is diagnosed and what other heart conditions exist.
The baby's care provider may recommend medications or a catheter procedure to help manage the condition before corrective surgery.
Before surgery for transposition of the great arteries, a medication called alprostadil (Caverject, Edex, others) may be given to increase blood flow and improve mixing of oxygen-poor and oxygen-rich blood.
Surgery or other procedures
Surgery for transposition of the great arteries is usually done within the first days to weeks after birth. Options depend on the type of transposition. Not all patients with congenitally corrected transposition need surgery.
Surgeries and procedures used to treat transposition of the great arteries may include:
- Atrial septostomy. This catheter procedure may be done urgently as a temporary treatment. The procedure, also called balloon atrial septostomy, widens a natural connection between the heart's upper chambers (atria). It helps mix oxygen-rich and oxygen-poor blood, increasing oxygen delivery to the baby's body.
Arterial switch operation. This is the most common surgery used to correct transposition of the great arteries. During an arterial switch operation, the pulmonary artery and the aorta are moved to their correct positions. The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The heart arteries also are reattached to the aorta.
If your baby also has a ventricular septal defect or an atrial septal defect, those holes usually are closed during surgery. But sometimes a heart surgeon may leave small ventricular septal defects to close on their own.
Atrial switch operation. In this surgery, the surgeon divides blood flow between the heart's two upper chambers (atria). Oxygen-rich blood coming back to the heart from the lungs is directed to the right lower chamber (right ventricle), which then pumps the blood to the aorta. The oxygen-poor blood returning to the heart from the body is directed to the left lower chamber (left ventricle), where it's pumped to the pulmonary artery.
After the atrial switch procedure, the right ventricle must pump blood to the body, instead of just to the lungs as it would do in a typical heart. Possible complications of the atrial switch operation include irregular heartbeats, obstructions or leaks, and heart failure due to long-term reduced pumping function.
- Rastelli procedure. This procedure may be recommended if a baby has a ventricular septal defect with transposition of the great arteries. The surgeon closes the hole (septal defect) in the heart using a synthetic patch and redirects blood flow from the left ventricle to the aorta, allowing the oxygen-rich blood to go to the body. The connection between the left ventricle and the pulmonary artery is disrupted. A connection is then made with an artificial valve from the right ventricle to the artery connected to the lungs (pulmonary artery).
- Double switch procedure. This complex surgical procedure is used to treat congenitally corrected transposition. It redirects blood flow coming into the heart and switches the great artery connections, with the goal of placing the left lower heart chamber (ventricle) in the position to pump oxygen rich blood to the aorta.
Additional surgeries may be needed to correct other heart conditions, such as ventricular septal defect or pulmonary outflow obstruction. A pacemaker may be needed if transposition of the great arteries causes a disruption in heart signaling (heart block).
Some complications of transposition of the great arteries, such as irregular heart rhythms (arrhythmias) or heart valve problems may require additional treatment.
After corrective surgery for transposition of the great arteries, the person will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease.
- Strenuous activity. The cardiologist may recommend avoiding 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 provider about what type and amount of activity is safe.
Pregnancy. If you're thinking about becoming pregnant, talk to your cardiologist and obstetrician before conceiving. If you had surgery to correct transposition of the great arteries while a baby, it may be possible to have a healthy pregnancy, but specialized care may be needed.
Complications of transposition of the great arteries, such as irregular heart rhythms or serious heart muscle problems, may make pregnancy risky for both mom and the unborn baby. 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.
Coping and support
Caring for a baby with a serious heart condition, such as transposition of the great arteries, can be challenging. Here are some strategies that may help make it easier:
- Seek support. Ask for help from family members and friends. Talk with your child's cardiologist about support groups and other types of assistance that are available near you.
- Record your or your baby's health history. Write down the diagnosis, medications, surgery, and other procedures and the dates they were performed, along with any doctor's names and numbers. This record will be helpful for health care providers who are unfamiliar with your or your baby's health history.
- Encourage safe activities. After corrective surgery, you or your child may need to avoid certain types of vigorous activities. Talk with the cardiologist about which activities are safe. If some are off-limits, encourage other pursuits rather than focusing on what can't be done.
Every circumstance is different. But due to advances in surgical treatment, most babies with transposition of the great arteries grow up to lead active lives.
Preparing for your appointment
If a care provider suspects transposition of the arteries, you or your child will be referred to a cardiologist for diagnosis and treatment.
What you can do
- Get a complete family history for both sides of your family. Ask if anyone in your family has had a congenital heart defect or congenital heart disease.
- Take a family member or friend with you, if possible. Sometimes it can be difficult to remember all of the information provided to you. Someone who goes with you may be able to recall information if you forget some details.
- Write down questions to ask the health care provider.
For transposition of the great arteries, some basic questions to ask the health care provider include:
- What treatments are available, and which do you recommend?
- After surgery, will there be any lingering health concerns?
- Are there any activity restrictions?
- Are there any brochures or other printed material that I can take home with me? What websites do you recommend?
In addition to the questions that you've prepared to ask your health care provider, don't hesitate to ask questions during your appointment if you don't understand something.
What to expect from your doctor
Your health care provider is likely to ask you a number of questions, such as:
- Is there a family history of heart disease at birth?
- Were there any known pregnancy complications?
- Have you noticed that your baby has blue skin, difficulty feeding or difficulty breathing?
- If you are the patient, have you had shortness of breath, swelling of the legs or irregular heartbeats?