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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.
Jack Rychik, MD: After the arterial switch operation, the aorta, which was sitting over the right ventricle, now sits over the left ventricle, and we use to term neoaorta for that vessel because it's now a new aorta. It's in a new position. The pulmonary artery, which was sitting over the left ventricle, now sits over the right ventricle. The vessels themselves are switched, but the base of the vessels, which contain the valves that lead to those vessels, remain on each of the ventricles. So, in fact, when you do an arterial switch operation, you bring the aorta over to the left ventricle, but you're putting that over what has developed and originated as the pulmonary valve. So the old pulmonary valve becomes the neo or new aortic valve as it now connects to the aorta. And, as we follow these patients forward in time, what we've learned is that sometimes that pulmonary valve, that neoaortic valve, natural pulmonary valve, can begin to leak a little bit. And so that's something we need to follow.
Peter Gruber, MD: Transposition of the great arteries is one of those lesions in which there are rarely other associated defects.
Jack Rychik, MD: In some after repair there can be development of narrowing of the pulmonary artery.
Peter Gruber, MD: Normally, the pulmonary artery is behind the aorta. But in this situation we now have to put the pulmonary artery in the front of the aorta because it's switched. That ends up draping the two pulmonary arteries that go to the right lung and the left lung on top of the aorta. And this can sometimes result in narrowing of those arteries. So that's one of the things we look at carefully and can be followed with echocardiography.
Jack Rychik, MD: And we've now seen in some of our adolescents that there can be narrowings of the coronary arteries following the switch itself.
Gil Wernovsky, MD: The real unknown in transposition surgery is what happens over the lifetime of a patient specifically related to the most difficult part of the operation and that's moving the tiny coronary arteries.
Jack Rychik, MD: There are no 40 or 50 or 70-year olds who have had an arterial switch procedure. So we don't know, really, what's going to happen to many of these patients when they get much older.
Gil Wernovsky, MD: As you know coronary artery problem is the most common cardiac problem in adults. They can get clogged with cholesterol and have atherosclerosis. And what we don't know, because the children have not gotten into the age when atherosclerosis is important is, will children with transposition surgery be more at risk than they would have been based on their lifestyle and genetics than the rest of the population? And it's for these reasons we encourage a healthy lifestyle, in particular, for children that have had transposition surgery. That they get routine exercise, that their diet is good, and that they'll need lifelong follow up just like all children with congenital heart disease.
J. William Gaynor, MD: But for what's called transposition with intact ventricular septum, or simple transposition, the survival should be excellent. You should have between a 95 and 100% chance of surviving the operation. And again, since this operation was only introduced in the mid-1980's, we've only got about 15 to 20 years of experience with it, but survival out to 15 to 20 years is excellent. And most kids have good exercise capacity, are going to school, and leading relatively normal lives.
Donna Lamborne, Mother: Grace is--
Trai Lamborne, Father: You would never know.
Donna Lamborne: No, you would never know in a million years--
Trai Lamborne: --that she ever had any type of heart defect or-- she's going to be 3 in May, and she is unbelievable.
Donna Lamborne: Quite a personality.
Trai Lamborne: Oh, yeah, she's a ham.
Donna Lamborne: She is very outgoing. She loves to dance. She loves to sing. She has so many bruises and bumps on her legs from falling and jumping and climbing and crawling and, you know, whatever, any kind of activity we really, you know, we've been told that she really should have no limitations.
Gil Wernovsky, MD: Basically, it is a reparative operation and, in every sense of the word, through childhood it's very unlikely the children need any further intervention. With the current approaches and the surgical techniques, maybe 5 to 10% of children will need an operation to patch an area that doesn't grow as the child grows from the newborn size to the full adult size. Now, the frequency that used to happen was about 20%, and know it's down to about 5%. So, in general, children are followed yearly or every other year that have had an arterial switch operation and have little to no restrictions on their activity.
Thomas Spray, MD: What I tell families is that there are more adults now than there are children living with congenital heart disease, which frankly is a testimony to the fact we've been pretty successful with dealing with these conditions.
Gil Wernovsky, MD: We don't cure congenital heart disease, just like we don't cure diabetes. We take a disease that's very complicated and lethal in some respects and turn that from an unmanageable disease into a very manageable disease with real hopeful outcomes. In general, the children who go through these complex surgical interventions are doing very, very well on a day-to-day basis and really indistinguishable from the other kids in the class.
Donna Lamborne: I hope she just stays happy and does whatever she wants.
Trai Lamborne: I hope she can look back in these pictures--look at them again in 20 years and realize what she put us through.
Donna Lamborne: Exactly. Right. So--
Trai Lamborne: She's just a great kid and like any kid. I mean, who's had a heart defect. Who hasn't had a heart defect. You just wish the world for them.
Elizabeth Goldmuntz, MD: What moves me is to work with the families and to work with the children and to give them the best possible care that we can provide. That's first and foremost.
Susan Nicolson, MD: And to see them grow and to see them integrated into the family and to see them interface with their parents and their siblings just as you would any other routine and normal child.
Thomas Spray, MD: To be able to take the heart of a child and fix it so that the physiology is better and the child has a chance at a long life, that's very gratifying.
J. William Gaynor, MD:: There's nothing better than seeing the kids come back when they're 4 or 5 years old.
Susan Nicolson, MD: And really see that they're just like any other kid.
Gil Wernovsky, MD: I see patients now still that are in their high teens and young 20's. I get prom pictures from them. I just got a wedding invitation from a patient that I followed as a fellow. And that's, you know, that's overwhelming. I mean, it's absolutely a beautiful thing.
Jack Rychik, MD: Through the Fetal Heart Program, we're now able to offer hope and promise for the future for these children to go on to lead happy and healthy lives. And that's what this is all about.
Contact the Fetal Heart Program for more information