Before you begin reading about congenitally corrected transposition of the great arteries, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
In a normal heart, the left-side pumping chamber (left ventricle) sends blood to the entire body, whereas the right-side ventricle pumps blood only a short distance, to the lungs. The left ventricle is built stronger than the right ventricle, because it has to work harder.
In congenitally corrected transposition of the great arteries (CCTGA), the heart twists abnormally during fetal development, and the ventricles are reversed: The stronger left ventricle pumps blood to the lungs and the weaker right ventricle has the harder chore of pumping blood to the entire body.
CCTGA can cause problems, particularly for the right ventricle, which must work harder than it was meant to. For many patients, in the long run, the ability of the right ventricle to continue to function effectively will determine how well they are.
There are several problems that co-exist in many patients with CCTGA and affect how well they do and what kind of treatment or surgical repair may be suitable. The most common associated lesions are:
CCTGA is a rare heart defect. Only 0.5 to 1 percent of all people with heart defects have CCTGA. This means there are about 5,000 to 10,000 people in the United States with this condition.
In CCTGA both ventricles (pumping chambers) are reversed and the arteries coming off them are inverted as well. The end result is paradoxically a blood flow pathway which is normal. This led to the confusing term “corrected” for a complex malformation in which the heart is far from being normal.
People with CCTGA experience a wide variety of symptoms, depending on the associated defects they have. There are sick newborns, with low oxygen level, who need care immediately after birth, and there are healthy people who can live a normal life for many years, without any intervention.
In one case, a woman was diagnosed with CCTGA at age 83 having had five children. On the other hand, some babies born with CCTGA die in infancy. Many people with CCTGA have no or few symptoms as children and young adults, but develop problems as they age. Outcomes are better for those born without additional heart defects.
CCTGA is often not diagnosed until adolescence or adulthood, when problems develop because of the leaky heart valve, abnormal electrical conduction in the heart, or inability of the right ventricle to continue the hard work of pumping blood to the entire body.
The symptoms may include fainting from the block in the electrical conduction, or fatigue from inability of the heart to pump enough blood to the body and backward flow through the leaky valve.
Diagnosis of CCTGA may require some or all of these tests.
Echocardiogram and cardiac MRI are most commonly used in the diagnosis of CCTGA.
Treatment for CCTGA varies according to the type and severity of symptoms and associated defects. In some patients, especially those that have no additional heart defects, there may not be a need to do anything.
For patients that do need surgery the type of operation will vary according to the associated defects. There are several operations used in this condition, including:
Any of the above operations may also have to include replacement of the leaky tricuspid valve with an artificial valve, and insertion of a pacemaker.
Because those operations are complex and difficult they are best performed in centers with large patient volume, resources and experience, like the Cardiac Center at CHOP.
Congenitally corrected transposition of the great arteries is a complex heart condition. Some related complications begin and can be treated early in childhood, but others develop later in life. CCTGA requires life-long care by a cardiologist with expertise in congenital heart disease.
Our pediatric cardiologists follow patients until they are young adults. The care is then transitioned to a special service we have for adults with congenital heart disease (ACHD). The American College of Cardiology has recommended that CCTGA patients be seen regularly at regional ACHD centers.
We will help patients transition their care to our ACHD program, or to a cardiologist with congenital heart disease expertise in another location.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Because of enormous strides in medicine and technology, today many children born with congenitally corrected transposition of the great arteries go on to lead healthy, productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Alex Davidson, MD
Date: January 2010
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