Congenitally Corrected Transposition of the Great Arteries (ccTGA) and Atrioventricular (AV) Discordant Connections

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with congenitally corrected transposition of the great arteries (or “ccTGA”) (also called “levo-transposition of the great arteries,” or “LTGA”) and atrioventricular (or “AV”) discordant connections, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

Congenitally corrected transposition of the great arteries (ccTGA) and atrioventricular (AV) discordant connections are a group of problems in which the aorta and pulmonary artery (which are the main blood vessels leaving the heart) leave from the wrong ventricle. (Ventricles are the heart’s two lower chambers that receive blood from the atria — the top chambers of the heart. “Congenital” means that a child was born with the condition.

In congenitally corrected transposition of the great arteries, there’s also twisting within the heart — the blue blood is correctly going to the lungs and the red blood is correctly going to the body, but it flows through the wrong ventricle to get there. The challenge is that, anatomically, the left ventricle is designed to pump to the body in the long run and the right ventricle is designed to pump to the lungs. (In “levo-transposition of the great arteries, “levo” is Latin for “left.”) So when they pump to the wrong place, the ventricles may not work as well or for as long.

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

These conditions are typically diagnosed at birth or often early in life, but children can get to and through adulthood without having any symptoms or being diagnosed (the right ventricle may just adapt and be able to handle it).

 
How & When Is It Treated?

Depending on the exact anatomy, our Nemours Cardiac Center surgeons may suggest multiple different surgical procedures. These options range from switching both the atria and the arteries so that the proper ventricle is pumping to the proper location (this is called a “double switch procedure”) or just closing the holes in the heart while leaving the right ventricle pumping to the body. In fact, some children can live the rest of their life like that. The exact surgical procedure offered and the timing of that procedure will vary depending on the child’s specific anatomy.


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How Will It Affect My Child Long Term?

Children may need only a single operation or they may end up needing multiple operations. Again, because the right ventricle isn’t designed to pump against the blood pressure in the body, it may not be able to do it as well or for as long as the left ventricle could. Because of this, some children will develop heart failure during adulthood as the right ventricle stops working well.

Children with levo-transposition of the great arteries (LTGA) are also at risk for having problems with the electrical system in the heart. Because of the abnormal twisting the heart went through when it was growing, the electrical system connecting the upper and lower chambers may not have developed normally. These children are at high risk for disruptions of the connections of the upper and lower chambers resulting in complete heart block where the electrical impulses don’t pass between chambers. This may happen at the time of surgical or catheter procedures or years later.

The result is that some children require permanent pacemakers. (Connected to the heart by a wire, a pacemaker is a small, battery-operated device implanted into the body, near the collarbone or at the bottom of the ribcage, through a surgical procedure. Through a sensing device, a pacemaker can detect if the heart rate is too slow and sends electrical signals to the heart to speed up the heartbeat.)

If your child has levo-transposition of the great arteries (LTGA) and atrioventricular discordant connections, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

Make an Appointment

(800) 416-4441

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


International
Patients

+1 (302) 651-4993

Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »