Transposition of the great arteries

Diagnosis

Transposition of the great arteries is most often diagnosed after a baby is born. But sometimes the condition may be seen before birth during a routine pregnancy ultrasound. If so, an ultrasound of the unborn baby's heart may be done to confirm the diagnosis. This test is called a fetal echocardiogram.

After birth, a health care provider may think about a diagnosis of a TGA if the baby has blue or gray skin, a weak pulse, or trouble breathing. The care provider may hear a heart sound, called a murmur, while listening to the baby's heart.

Tests

Tests are needed to confirm a diagnosis of transposition of the great arteries. They may include:

• Echocardiogram. This test uses sound waves to create moving pictures of the beating heart. It shows how blood flows through the heart, heart valves and blood vessels. It can show the positions of the two main arteries leaving the heart. An echocardiogram also can show if there are other heart problems present at birth, such as a hole in the heart.
• Chest X-ray. A chest X-ray shows the condition of the heart and lungs. It can't diagnose TGA by itself, but it does help the health care provider see the heart's size.
• Electrocardiogram (ECG or EKG). This simple, painless test records the electrical activity of the heart. Sticky patches called 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.

Treatment

All infants with complete transposition of the great arteries (D-TGA) need surgery to correct the heart problem. Treatment for congenitally corrected transposition (L-TGA) depends on when the condition is diagnosed and what other heart conditions exist.

Medications

Before surgery is done to fix the switched arteries, a medicine called alprostadil (Caverject, Edex, others) may be given to the baby. This medicine increases blood flow. It helps oxygen-poor and oxygen-rich blood better mix together.

Surgery or other procedures

Surgery for transposition of the great arteries (TGA) is usually done within the first days to weeks after birth. Options depend on the type of TGA. Not all people with congenitally corrected transposition need surgery.

Surgeries and other treatments used to treat transposition of the great arteries may include:

• Atrial septostomy. This treatment may be done urgently as a temporary fix before surgery. It uses thin tubes and small cuts to widen a natural connection between the heart's upper chambers. It helps mix oxygen-rich and oxygen-poor blood, improving oxygen levels in the baby's body.
• Arterial switch operation. This is the most common surgery used to correct transposition of the great arteries. During this surgery, the two main arteries leaving the heart are moved to their correct positions. Other heart problems present at birth may be repaired during this surgery.
• Atrial switch operation. The surgeon splits blood flow between the heart's two upper chambers. After this surgery, the right lower heart chamber must pump blood to the body, instead of just to the lungs.
• Rastelli procedure. This surgery may be done if a baby with TGA also has a hole in the heart called a ventricular septal defect. The surgeon patches the hole and redirects blood flow from the left lower heart chamber to the aorta. This lets oxygen-rich blood go to the body. An artificial valve connects the right lower heart chamber to the lung artery.
• Double switch procedure. This complex surgery is used to treat congenitally corrected transposition. It redirects blood flow coming into the heart. It switches the great artery connections so the left lower heart chamber can pump oxygen-rich blood to the aorta.

Babies born with TGA often have other heart problems. Other surgeries may be needed to fix those heart problems. Surgery also may be needed to treat complications of TGA. If TGA causes changes in the heartbeat, a device called a pacemaker may be recommended.

After surgery to fix TGA, lifelong care is needed with a provider trained in heart problems present at birth. This type of health care provider is called a congenital cardiologist.

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 tips that might be helpful:

• Seek support. Ask for help from family members and friends. Talk with your baby's health care providers about support groups and other types of assistance that are available near you.
• Record the baby's health history. Write down the diagnosis, medicines, surgery and other treatments. Include the dates of treatment or surgery and the health care providers' names and numbers. This record will be helpful for health care providers who are unfamiliar with your baby's health history.
• Encourage safe activities. After surgery to fix TGA, some high-energy activities might need to be avoided. Talk with a health care provider about which exercises or activities are safe.

Every situation 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 your baby has transposition of the great arteries (TGA), you'll likely have appointments with several types of health care providers. For example, you'll usually see a provider trained in heart problems present at birth, called a congenital cardiologist. Here's some information to help you prepare for the appointments.

What you can do

• Get a complete family history for both sides of your family. Ask if anyone in your family was born with a heart problem.
• 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 remember 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:

• Does my baby need surgery?
• What other treatments are available, and which do you recommend?
• How often are checkups needed after surgery?
• After surgery, will there be any remaining 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?

Don't hesitate to ask other questions you have.

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 problems at birth?
• Were there any known pregnancy complications?
• Does the person have blue or gray skin, difficulty feeding, or trouble breathing?
• Does the person have shortness of breath, swelling of the legs or irregular heartbeats?