What is 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). The condition is also called dextro-transposition of the great arteries. A rarer type of this condition is called levo-transposition of the great arteries.
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 either prenatally or within the first hours to weeks of life.
How common is transposition of the great arteries?
Transposition of the great arteries is the second most common congenital heart defect that causes problems in early infancy. TGA occurs in about 3% of all congenital heart defects. Please discuss with your doctor for further information.
What are the symptoms of transposition of the great arteries?
The common symptoms of transposition of the great arteries are:
- Blue color of the skin (cyanosis)
- Shortness of breath
- Lack of appetite
- Poor weight gain
There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.
When should I see my 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 face and body.
What causes transposition of the great arteries?
The heart forms during the first 8 weeks of fetal development. The problem occurs in the middle of this time, allowing the aorta and pulmonary artery to become attached to the incorrect chamber.
Some congenital heart defects may have a genetic link causing heart problems to occur more often in certain families. Most of the time this heart defect occurs by chance, with no clear reason for its development.
What increases my risk for transposition of the great arteries?
There are many risk factors for transposition of the great arteries, such as:
- A history of German measles (rubella) or another viral illness in the mother during pregnancy
- 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
Diagnosis & treatment
The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.
How is transposition of the great arteries diagnosed?
A pediatric cardiologist and/or a neonatologist will be involved in your child’s care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.
Cyanosis is the major indication that there is a problem with your newborn. Your child’s doctor may have also heard a heart murmur during a physical examination. In this case, a heart murmur is a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix, such as the ventricular septal defect or patent ductus arteriosus.
Diagnostic testing for congenital heart disease varies by the child’s age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:
- Chest X-ray. A diagnostic test that uses invisible X-ray beams to produce images of internal tissues, bones, and organs onto film.
- Electrocardiogram (ECG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias), and detects heart muscle stress.
- Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor to produce a moving picture of the heart and heart valves.
- Cardiac catheterization. A cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.
How is transposition of the great arteries treated?
All infants with transposition of the great arteries need surgery to correct the defect.
Your baby’s doctor may recommend several options to help manage the condition before corrective surgery. They include:
- 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.
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 week 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 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.
Talk to your child’s doctor about what type of physical activities 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.
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.
Lifestyle changes & home remedies
What are some lifestyle changes or home remedies that can help me manage transposition of the great arteries?
The following lifestyles and home remedies might help you cope with transposition of the great arteries:
- 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 baby’s health history. You may want to write down your baby’s diagnosis, medications, surgery and other procedures and the dates they were performed, your child’s cardiologist’s name and phone number, and any other important information about your baby’s care. This record will help you recall the care your child has received, and it will be helpful for doctors who are unfamiliar with your baby to understand his or her health history.
- Talk about your concerns. You may worry about the risks of vigorous activity, even after your child has had corrective surgery. Talk with the cardiologist about which activities are safe for your child. If some are off-limits, encourage your child in other pursuits rather than focusing on what he or she can’t do.If you have other concerns about your child’s health, discuss them with your child’s cardiologist, too.
Although every circumstance is different, remember that due to advances in surgical treatment, most babies with transposition of the great arteries grow up to lead active lives.
If you have any questions, please consult with your doctor to better understand the best solution for you.
Hello Health Group does not provide medical advice, diagnosis or treatment.
Hello Health Group does not provide medical advice, diagnosis or treatment.