Testing and diagnosis of CCTGA

Diagnosis of CCTGA may require some or all of these tests:

• Echocardiogram (also called echo or ultrasound), in which sound waves create an image of the heart
• Chest X-ray
• Electrocardiogram (ECG), which records of the electrical activity of the heart
• Cardiac MRI, a 3D image that shows the heart’s structure
• Cardiac catheterization, during which a thin, flexible tube (catheter) is inserted through a vein or artery in the leg and into the heart to provide detailed information about the structure and function of the heart and lungs

Echocardiogram and cardiac MRI are used most often to diagnosis CCTGA.

Treatment for CCTGA

Treatment for CCTGA depends on the type and severity of symptoms and the defects causing them. Some children, especially those that have no additional heart defects, may not need treatment. However, even these children should be followed by a cardiologist for the rest of their lives so the condition can be monitored. For other children, medication may be needed.

For children that do need surgery, the type of operation depends on the child’s specific heart defects. There are several operations used to repair this condition. Here are a few.

• If there’s a hole in the heart (VSD), or if pulmonary stenosis develops, doctors may need to close the hole or insert a tube (called a conduit) between the heart and lungs. The conduit may need to be replaced or made bigger as time passes, which would require another surgery. 
• If the VSD is left open, doctors might treat the heart as if it has only one pumping chamber (ventricle). This is called a “single ventricle” or Fontan repair. In this procedure, the veins that carry blood without oxygen are connected directly to the lungs, so the blood can pick up oxygen without having to pass through the heart. 
• Doctors may perform a double switch operation, during which surgeons redirect blood flow from one side of the heart to the other. The heart's major arteries are also switched during this operation. This corrects the blood flow patterns, so that the heart's stronger left side delivers blood to the body and the weaker right side sends blood to the lungs. This is a very complex operation and is usually only performed on infants and children. It is very rarely used in adults.

Any of these operations may also include valve replacement with an artificial valve and/or placing a pacemaker.

The Cardiac Center at CHOP is a high-volume cardiac center, which means our highly experienced surgeons are experts at complex and difficult surgeries like Fontan and the double switch operation, with outcomes among the best in the world.

Outlook for CCTGA

Because of enormous strides in medicine and technology, today many children born with CCTGA go on to lead healthy, productive lives as adults. Overall, outcomes are usually better for babies born without additional heart defects.

Follow-up care

CCTGA is a complex heart condition. Some related complications begin and can be treated early in childhood, but other complications develop later in life. CCTGA requires life-long care by a cardiologist experienced in congenital heart disease.

Our pediatric cardiologists follow patients until they are young adults. The American College of Cardiology has recommended that CCTGA patients be seen regularly at regional ACHD centers. The Philadelphia Adult Congenital Heart Center, a joint program of CHOP and Penn Medicine, meets the unique needs of adults who were born with heart defects.

At CHOP’s Cardiac Center, we support our adolescent and young adult patients through a smooth and seamless transition to adult cardiology care at the Philadelphia Adult Congenital Heart Center or, if desired, to a cardiologist with congenital heart disease expertise in another location.