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In transposition of the great arteries (TGA), the “great” arteries, the aorta and the right ventricle, are reversed in their origins from the heart. The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle — exactly the opposite of a normal heart’s anatomy.
With these arteries reversed, oxygen-poor (blue) blood returns to the right atrium from the body, passes into the right ventricle, then goes into the aorta and back to the body. Oxygen-rich (red) blood returns to the left atrium from the lungs and passes into the left ventricle, which pumps it back to the lungs — the opposite of the way blood normally circulates.
Most babies with TGA, also known as transposition of the great vessels, are born with a small hole between their atria, which allows just enough red blood to get to the body to maintain life for a few hours. Typically diagnosed within the first hours after birth, TGA is life threatening, and in order to survive babies need special therapy urgently.
The most commonly used initial therapy is balloon atrial septostomy, where a balloon at the end of a catheter (small, flexible tube) is used to enlarge the opening between the atria. Complete open-heart repair generally takes place a few days later.
If there are no unusual risk factors, more than 98 percent of surgically-treated infants survive their infancy. Most children who’ve had TGA surgery recover and grow normally, although they can be at some risk in the future for arrhythmias, leaky valves and other heart issues.
While relatively rare (one in every 4,000 babies), TGA is among the most common cyanotic (“blue baby”) heart defects, and it affects boys more than girls.
Most babies with TGA have only that defect, but there are other defects that can occur with TGA:
ASD is an opening between the right and left atria. This defect occurs occasionally with TGA and is actually helpful; it easily can be corrected at the time of surgery.
VSD is an opening in the wall of tissue (ventricular septum) separating the right and left ventricles. This occurs in about 20 percent of patients with TGA. If large enough, a VSD will need to be surgically closed at the time of surgical correction.
The ductus arteriosus is a blood vessel that’s normally open (patent) while the baby is inside the mother, but which closes naturally shortly after birth. Because keeping this vessel open helps increase the blood oxygen level in babies with TGA, doctors often give patients a medicine (prostaglandin E1) to keep the ductus open.
At Boston Children’s Hospital, our team of experts in the Department of Cardiac Surgery has years of experience helping children who have been diagnosed with transposition of the greater arteries.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”