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Congenitally Corrected Transposition of the Great Arteries (CCTGA)
Congenitally corrected transposition of the great arteries (CCTGA or L-TGA) is different and less common than “regular” transposition of the great arteries (D-TGA), affecting fewer than 1% of people with heart defects. In this rare condition, the anatomy of the heart’s main pumping chambers is reversed like a mirror image, flipping the usual position and roles. The right ventricle and its accompanying tricuspid valve usually pump blood to the lungs. The left ventricle and its accompanying mitral valve usually pump blood to the rest of the body. In CCTGA, these roles are reversed. Because the arteries are also reversed, it’s like the heart has “corrected” itself, allowing it to still pump blue blood to the lungs and red oxygenated blood out to the body. However, the CCTGA heart does not function normally and often develops problems over time.
In a normal heart, the stronger, more muscular left ventricle pumps oxygen-rich (red) blood out to the body to deliver that oxygen to the tissues and organs of the body. The mitral valve is coupled with the left ventricle and is capable of withstanding the high pressure of the left ventricle. The right ventricle and tricuspid valve have an easier job, pumping low-pressure blood to the lungs.
In CCTGA, the ventricles and valves are reversed, which means the left ventricle and mitral valve do the easy job of pumping blood to the lungs and the right ventricle and tricuspid valve are required to do the harder job of pumping blood to the rest of the body. This role reversal can work in the short term, but when the heart’s weaker parts handle the hardest jobs, it makes the heart less efficient and more prone to wearing down prematurely.
Experts in treating CCTGA
Congenitally corrected transposition of the great arteries (L-TGA) is a complex condition that should be treated at a center that specializes in congenital heart defects. The Herma Heart Institute is one of the nation’s top pediatric heart programs and is internationally known for its expertise in treating children with CCTGA. We consistently deliver some of the best congenital heart surgery outcomes for even the most complex types of heart disease. If your child has already been diagnosed at another center, we invite you to get a second opinion for CCTGA at the Herma Heart Institute.The Herma Heart Institute is also home to our Fetal Heart Program and Adult Congenital Heart Disease Program, allowing for seamless, high quality care for CCTGA across your child’s lifespan.
What causes CCTGA? Is CCTGA hereditary?
In the earliest weeks of pregnancy, a baby’s heart starts as a tube that folds to create the heart’s left and right sides. In CCTGA, it folds the other way, flipping the usual position of the ventricles and attached valves. We do not know enough about what causes this defect, but research shows that it rarely runs in families. Sometimes a baby’s anatomy spontaneously develops abnormally, and we need more research to understand possible causes of CCTGA and other congenital heart defects.
Why is CCTGA a concern?CCTGA can lead to abnormal heart rhythms, valve problems or heart failure. These problems might appear early on or develop later in life. Children with this condition might also have a ventricular septal defect (VSD), which is a hole between the two ventricles, or pulmonary stenosis, a blockage leading to the lungs.
What are the symptoms of CCTGA?
Sometimes children with this condition show no symptoms, although that can change if the heart starts to fail. Symptoms could include:
- Abnormal heart rhythm
- Child tires easily when playing
- Shortness of breath
Always consult your child’s doctor if you have concerns or notice anything new.
How is CCTGA diagnosed?
Your child’s doctor might detect a heart murmur or rhythm problem during a physical exam. Some babies who also have a VSD (hole in the heart) might have rapid breathing and failure to thrive. However, sometimes children with CCTGA show no symptoms initially and might not be diagnosed until later in childhood or even adulthood, during routine tests or when new symptoms appear.
Tests to confirm the diagnosis could include:
- Chest x-ray - Uses electromagnetic energy beams to produce images of internal tissues, bones and organs onto film
- Electrocardiogram (ECG or EKG) - Records the electrical activity of the heart, shows abnormal rhythms and detects heart muscle stress
- Echocardiogram (echo) - Evaluates the heart’s structure and function by using sound waves recorded on an electronic sensor to produce a moving picture of the heart and heart valves
- Cardiac catheterization - A small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the heart to get very detailed information about the structures inside the heart
- Cardiac MRI - Uses a magnet, radio waves and a computer to create very detailed, 3D still and moving images of major blood vessels and the heart as it beats
Treatment for CCTGA
Children’s CCTGA specialists will recommend the best treatment for your child based on:
- Your child’s age, overall health and medical history
- Extent of the disease
- Your child’s tolerance for specific medications, procedures or therapies
- Expectations for the course of the disease
- Your opinion or preference
CCTGA treatment could include:
Medical management - Some children with CCTGA might need medications, including:
- Digoxin - To strengthen the heart muscle, enabling it to pump more efficiently
- Diuretics - To help the kidneys remove excess fluid from the body, which is sometimes needed when the heart isn’t working as well as it could
- Antibiotics - To prevent a heart infection called bacterial endocarditis after routine dental work or other procedures, which can be a risk for children with certain heart defects
Surgical repair - For the best outcomes, the Herma Heart Institute’s experts recommend surgically correcting CCTGA early in life, before a child’s heart starts to fail. Depending on your child’s age and heart condition, your child’s cardiologist might recommend the Fontan procedure to bring blood directly to the lungs, or a “double-switch” operation, which surgically reverses the defect and restores the right and left ventricles to their proper functions. The Herma Heart Institute is internationally known for its expertise in the double-switch procedure and has taught other heart specialists from around the world how to best repair the CCTGA heart.
Your child might develop other heart issues that could require treatment, including:
- Heart block - This blockage in the heart’s electronic signals prevents the heart from being able to beat as quickly or efficiently. In these cases, your cardiologist might recommend a pacemaker to regulate the heart’s beating.
- Valve problems - If your child has a malformed tricuspid valve or if the valve becomes leaky (when blood flows backward into the chamber it just left) because of the heart’s increased demands, your child’s cardiologist might recommend valve replacement surgery.
- Heart failure - If congestive heart failure is a concern, medications can help, and valve replacement and/or a pacemaker might also be recommended to improve the heart’s functioning.
Surgical repairs and some other procedures (like pacemaker surgeries) are performed in the operating room while your child is under general anesthesia. A mitral valve repair or other minor procedures might be done through a cardiac catheterization procedure while your child is sedated. Your cardiologist will advise you on the best options for your child.
Postoperative care for your child
After surgery, your child will spend time in the cardiac intensive care unit (CICU). During the first several hours after the procedure, your child will likely be drowsy from the anesthesia and medications given to ease anxiety and pain. Over time, your child will become more alert.
While your child is in the ICU, Children’s might use the following equipment:
- Ventilator - This machine helps your child breathe. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while they are under anesthesia. Many children have the ventilator tube removed right after surgery, but some will benefit from remaining on the ventilator for a few hours or days afterward so they can rest.
- Intravenous (IV) catheters - These small, plastic tubes inserted into blood vessels provide IV fluids and important medications that help your child recover from the operation.
- Arterial line - A specialized IV placed in the wrist, or other area of the body where a pulse can be felt, measures blood pressure continuously during surgery and while your child is in the ICU.
- Nasogastric (NG) tube - A small, flexible tube keeps the stomach drained of acid and gas bubbles that could build up during surgery.
- Urinary catheter - This small, flexible tube drains urine out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. Some swelling and puffiness is common after surgery, and your child might be given diuretics to help the kidneys remove excess fluids.
- Chest tube - A drainage tube might be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding can occur for several hours or even a few days after surgery.
- Heart monitor - This machine constantly displays a picture of your child’s heart rhythm and monitors heart rate, arterial blood pressure and other values.
Your child might need other supportive equipment while in the CICU or afterward. They will be kept as comfortable as possible with medications that relieve pain and anxiety, and the staff might also ask for your input on how best to soothe and comfort your child.
After your child is discharged from the CICU, they will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home and will receive instructions on medications, activity limitations and follow-up appointments before your child is sent home.
Care for your child at home following CCTGA surgical repair
Most children feel fairly comfortable when they go home, and your physician might recommend pain medications, such as acetaminophen or ibuprofen, to keep them comfortable. Your child might become tired more quickly than before surgery, but they usually will be allowed to play with supervision. It’s important to avoid blows to the chest that might cause injury to the incision or breastbone. Within a few weeks, your child should be fully recovered and able to participate in normal activity.
Long-term outlook and life expectancy after CCTGA surgical repair
The prognosis for CCTGA can vary but is generally good with proper cardiology care. Your child will always need to be monitored by a cardiologist in case new problems develop. But sometimes CCTGA doesn’t cause any problems, and patients can live to old age with no issues. Once your child reaches adulthood, they should seek out a program that specializes in adult congenital heart disease like Children’s ACHD program.