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Transposition of the Great Arteries: Grace's Story
Transposition of the Great Arteries (TGA) is a severe congenital condition in which the body’s largest arteries are incorrectly attached to the chambers of the heart. In this 23-minute video, you'll learn how experts from the Fetal Heart Program and Cardiac Center identify TGA during pregnancy, support the baby's delivery, and provide surgery and comprehensive care after birth.
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Transcript: TGA Surgery
J. William Gaynor, MD: In transposition what you obviously want to do is rearrange the blood flow in some way so that the blue blood goes to the lungs and the red blood goes out to the body to mimic what happens in a normal heart.
Robert Shaddy, MD: Many years ago, someone had the courage and the technical skill and insight to say, "Let's just switch the arteries and do an arterial switch operation with the complex part of getting the tiny coronary artery switched at the same time."
Thomas Spray, MD: Remember the anatomy of transposition where the arteries to the lungs in the body come off the wrong side of the heart, the arterial switch operation takes those arteries and switches them back where they should have been in the first place. Now, that's pretty straightforward, except for the fact that the small arteries that supply blood to the heart itself also have to be moved. Those are called the coronary arteries. And they're very small. Each one is about two millimeters.
Gil Wernovsky, MD: The size of lead in a lead pencil. And those coronary arteries are then moved to the correct location in the newborn.
Thomas Spray, MD: They have to be removed and rotated and sewn to the other arteries so that they get blood, otherwise the heart will not receive oxygenated blood and can't function. And if those arteries are in any way kinked or twisted or in any way abnormal because they're so small, then no blood goes to the heart and the heart doesn't work. So the biggest risk of surgery for transposition of the great arteries is related to moving those so-called coronary arteries.
Donna Lamborne, Mother: I remember walking down the hallway to where the anesthesiologist picks her up.
Unknown Speaker: It's very, very important that the family feels comfortable with you as an individual taking their child from them.
Donna Lamborne: That was a really difficult thing, and she just smiled at us and she said, you know, "Don't worry, we're going to take care of her."
Sarah Tabbutt, MD: For those particular babies with transposition, the surgery is usually done within the first week or so of life.
J. William Gaynor, MD: They'll go to the operating room. The anesthesiologist will put them to sleep. We'll then clean, prepare everything, their chest and abdomen sterilely so that we can do the operation without infection. It's performed through an incision in the front over the breastbone, and we put them on the heart-lung machine, and cool them down to take care of their brain and other organs while we do the repair.
Peter Gruber, MD: We're bypassing the heart and the lungs so that the operative field, the area where we're working, is clean, and we can see what's going on.
J. William Gaynor, MD: Like other lesions, there's a lot of variation in transposition. There's simple transposition where the only problem is that the arteries are switched. More complex forms there may be a ventricular septal defect, or a hole within the heart. There can also be blockage of blood either going to the lungs or going to the body.
Peter Gruber, MD: So the principles of the operation are to switch the vessels and the coronary arteries so they're coming off the right ventricles. And practically what you do is divide the aorta directly above where the coronary arteries would implant, and you actually cut the coronary arteries out.
Jack Rychik, MD: Cutting out very small buttons of tissue of where the coronary arteries insert into the aorta.
Peter Gruber, MD: It's made more complex by the fact that those arteries don't always come from exactly the same spot, nor are there always the same types of arteries. There's a great deal of variability in terms of the anatomy of those arteries. Sometimes there's only one. Sometimes they come off very close together--two of them. Sometimes they run in the wall of the artery to the body before they exit onto the surface of the heart. And each of those variations requires a variation in surgical technique to deal with it. Some of them are much more complex and difficult to move than others. And most of the risk of the repair of transposition of the great arteries is related to moving those little arteries that supply blood to the heart itself.
Thomas Spray, MD: After surgery, we will make sure that the heart is working okay, that the blood pressure is okay, that there's enough oxygen in the blood, that there's no bleeding. And then, usually, we leave a couple little tubes inside the heart that let us measure pressures in the heart and give drugs. These come out through the skin. There's also usually two little blue pacing wires which let us change the heart rhythm. We then leave a drainage tube, and once everything's stable, the baby will come back up to the Intensive Care Unit.
J. William Gaynor, MD: That operation, nowadays, takes about three hours to do from start to finish.
Thomas Spray, MD: It was quick.
Donna Lamborne: Yeah.
Trai Lamborne: For such a big important thing it was kind of like-- Are you sure you're done? They brought her back to the unit that she was in for that entire week where she was stable prior to having to have the surgery.
Donna Lamborne: With the treatment of transposition of the great arteries, most of these children are out of the hospital in a couple of weeks and doing quite well.
Robert Shaddy, MD: The results with that surgery now are very good. The risk of not surviving that operation, while not zero, is less than 1%. So children do extremely well with that particular surgery.
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