Congenitally Corrected Transposition of the Great Arteries (CCTGA)
Key Points
- Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart defect that is often associated with other heart defects.
- It can sometimes be diagnosed before birth using a fetal echocardiogram, but some people with it also may not show symptoms until later in life.
- Treatment depends on each individual patient’s condition, but all patients with CCTGA will need to be followed by a cardiologist throughout their life.
What is congenitally corrected transposition of the great arteries?
Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart defect in which two abnormalities occur during a child’s development in the womb:
- The lower pumping chambers of the heart, called the ventricles, are reversed.
- The two main arteries (coronary arteries) that carry blood to the heart are also reversed.
In a normally developed heart, the left ventricle sends blood out to the body, and the right ventricle sends blood to the lungs. The left ventricle is stronger than the right because it has to pump more blood.
In cases of CCTGA, the reversal of both the ventricles and the coronary arteries cause the blood to flow through the heart on the correct path. This is why it is called “congenitally corrected.” However, even though blood flows correctly through the body, the smaller right ventricle must now pump blood to the entire body, leading to its overuse.
Less than 1% of people with congenital heart defects have CCTGA.
What are some additional heart conditions people have with CCTGA?
Patients with CCTGA also often have an additional existing heart condition, such as:
- Abnormal tricuspid valve
- Heart block
- Heart failure
- Pulmonary valve stenosis
- Ventricle septal defect
- Dextrocardia
What causes CCTGA?
The cause of CCTGA is unknown.
What are the symptoms of CCTGA?
When babies are found to have CCTGA, the symptoms are often from the additional heart conditions. These symptoms can include:
- Low oxygen levels
- Heart murmurs
- Heart arrhythmias
- Cyanosis
Some people who have CCTGA without other heart defects may not show symptoms until they are teens or adults. This is usually when issues have developed during childhood, such as when the right ventricle becomes unable to function properly from many years of overuse.
Symptoms in these instances can include:
- Fainting
- Dizziness
- Fatigue
How is CCTGA diagnosed?
Congenitally corrected transposition of the great arteries can sometimes be detected before birth with a fetal echocardiogram.
After birth, tests used for diagnosis may include:
Diagnosis for Adults
Adult patients who have not previously been diagnosed may show signs of heart block or heart failure. Diagnosis is typically made with an echocardiogram.
How is CCTGA Treated?
Children with CCTGA who do not have other heart defects may not require treatment. However, they should be monitored for the rest of their lives. Medication may be needed to treat symptoms of heart failure and arrhythmias (irregular heartbeat).
Babies who have CCTGA as well as other associated heart defects will typically need surgery early in life to treat any present defects. The type and timing of the surgery needed will depend on factors such as the type of defect(s) they have, how well or poorly the ventricles are functioning, and the rhythm of the heart.
For example, babies with a ventricular septal defect will need surgery to repair a hole that is present between the ventricles, typically with stitches or a special patch. Those with pulmonary valve stenosis will need a cardiac catheterization procedure or surgery to repair the pulmonary valve. Additional surgeries may be needed later in life to replace the valves.
A double-switch operation may be recommended for some patients. This is done to redirect blood flow and switch the heart’s major arteries. Some patients may also later need a pacemaker if they develop heart block.
What is the outlook for children with CCTGA?
The outlook for children with CCTGA varies depending on whether they have additional heart defects. Generally, all people with CCTGA will need lifelong monitoring and care from a cardiologist in case of complications later in life.
The risk of developing heart block increases as patients with CCTGA age, at a rate of about 2% each year of life. The risk of developing heart failure also increases with age because the right ventricle is not as well equipped as the left ventricle to pump blood to the entire body.
Medically reviewed by Benjamin Barnes, MD, MS, June 15, 2026
