Transposition of the Great Arteries
Before you begin reading about this heart defect, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
- What is Transposition of the Great Arteries?
- What are the symptoms?
- How is it diagnosed?
- What are the treatment options?
- What kind of follow-up care is required?
What is Transposition of the Great Arteries?
Transposition of the Great Arteries
Enlarge+Transposition of the Great Arteries (TGA) is a severe congenital heart defect in which the two large arteries that carry blood out of the heart are connected to the heart abnormally:
- The aorta is attached to the right-sided pumping chamber (ventricle), instead of the left.
- The pulmonary artery is attached to the left-sided pumping chamber (ventricle), instead of the right.
Normally, blood flows in this pattern: body » right side of heart » lungs » left side of heart » back to body. The pulmonary artery carries blood from the right side of the heart to the lungs, and the aorta carries blood from the left side of the heart to the body. In children with TGA, the normal pattern of flow does not exist, and the body doesn't get enough oxygenated blood.
What are the symptoms?
The symptoms include:
- Blue or purple tint to lips, skin and nails (cyanosis)
- Rapid breathing
- Difficulty feeding; poor appetite and poor weight gain
How is it diagnosed?
TGA may be diagnosed before birth, with fetal echocardiogram (ultrasound). Our Fetal Heart Program will prepare a plan for delivery and care immediately after birth.
Sometimes TGA is diagnosed when an infant is a few hours or days old, and in some cases, infants may not have visible symptoms for weeks or months. Pediatricians from other hospitals refer newborns to the Cardiac Center when they notice symptoms or abnormal values on laboratory testing such as pulse oximetry.
Diagnosis may require some or all of these tests:
- echocardiogram (also called "echo" or ultrasound) - sound waves create an image of the heart
- electrocardiogram (ECG) - a record of the electrical activity of the heart
- chest X-ray
- pulse oximetry - a non-invasive way to monitor the oxygen content of the blood
- cardiac catheterization - a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus ("belly button")
- cardiac MRI - a three-dimensional image shows the heart's abnormalities
What are the treatment options?
Surgery is required to repair Transposition of the Great Arteries. Without surgical therapy, the overwhelming majority of children with TGA die before their first birthday. This condition requires an open-heart surgical procedure called the arterial switch operation. It is typically performed shortly after the diagnosis is made. Surgeons reconstruct the heart so that the aorta is attached to the left ventricle and the pulmonary artery is attached to the right ventricle.
After surgery your child will recover in our Tabas Cardiac Intensive Care Unit (CICU).
In some children, a temporary procedure called a balloon atrial septostomy (BAS) may be recommended prior to the arterial switch operation. BAS is performed either during a cardiac catheterization or in the intensive care unit. A balloon-tipped catheter, developed at The Children's Hospital of Philadelphia by Dr. William J. Rashkind, is used to create or enlarge a hole in the upper chambers of the heart. The procedure is known throughout the world as the "Rashkind Procedure" and has been used to stabilize babies with TGA since the1960s. Following the Rashkind procedure, most children show an immediate improvement in blood oxygen levels, and may be better candidates for the subsequent arterial switch operation. Surgeons will repair the hole during the arterial switch.
In our practice in the Cardiac Center, approximately one-fourth of children with TGA require a Rashkind procedure prior to the arterial switch operation.
What kind of follow-up care is required?
Through 18
Children who have had surgical repair of TGA require life-long care by a cardiologist. Ongoing medication use is uncommon. More surgery may be required as the child grows.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physicians.
Into adulthood
We will help patients transition to an adult cardiologist.
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.
Until approximately 25 years ago, infants with TGA were managed by alternative surgical procedures, sometimes referred to as the "Senning" or "Mustard" operations. As a result, we don't yet know the truly long-term effects of the arterial switch operation beyond young adulthood. It is anticipated that the overwhelming majority of children born with TGA will go on to lead healthy, productive lives. Limitations to day-to-day activities, including sports, are rare.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Gil Wernovsky, MD
Date: October 2008
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