Tetralogy of Fallot Video Series

Transcript: Life After TOF Surgery

Transcripts in This Video Series
Diagnosis What is Tetralogy of Fallot (TOF)? How TOF Develops Preparing for your Baby's Delivery TOF Surgery Life After TOF Surgery

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Peter Gruber, MD: Tetralogy of Fallot is one of those diseases we've actually recognized for a very long period of time. From a historical standpoint it was first recognized by a series of children which were blue. Interestingly, many of those children got better when they squatted down or got into particular positions.

Thomas Spray, MD: Because they have to drive more blood to the lungs and that's what happens when you squat down.

J. William Gaynor, MD: The first successful operation was done in the mid-1940's at Johns Hopkins which was a shunt.

Thomas Spray, MD: The so called Blalock-Taussig shunt, which was one of the first operations devised for children with cyanotic congenital heart disease, that is patients who were blue.

J. William Gaynor, MD:

: It was a tube to carry blood flow from the aorta to the lungs and all of a sudden these blue babies would become pink. This did not correct Tetralogy of Fallot, it was a palliative operation.

Thomas Spray, MD: But it made a huge difference. Children, who were dying of blueness, or cyanosis, actually were able to survive and get into adulthood. And so it was a major advance. Then in about the early 1950s, the heart-lung machine was developed, and when that came along, it was possible to actually fix this heart defect.

Kim Persick, Mother: Robert goes back once a year for follow up now. We usually have our annual echocardiograms. And he's on no medication. And he has no restrictions. And he's just an active little boy.

J. William Gaynor, MD: Now, as with other complex heart lesions, we've moved towards early complete repair.

Peter Gruber, MD: Such that the vast majority of kids with Tetralogy of Fallot at The Children's Hospital of Philadelphia get a full, or what we call complete repair, upon diagnosis.

Jack Rychik, MD: We are quite fortunate to have some patients who are now in their 30s and 40s, even to the extent that it's not uncommon for us to have a mother who's had repair of Tetralogy of Fallot performed maybe in the 60s and 70s, who will now come to the Fetal Heart Program carrying a fetus for evaluation for congenital heart disease.

J. William Gaynor, MD: We recently looked at a series of 100 consecutive children at CHOP having elective tetralogy repair between-- at under 6 months of age, which means they had been out of the hospital, they came back electively for their operation. And every one of those children survived the operation. Now, that doesn't mean the mortality is zero. It means some of those children were very sick. But it does mean that survival for straightforward tetralogy should be excellent. And then the long-term survival also appears to be very, very good.

Thomas Spray, MD: The vast majority of children with Tetralogy of Fallot will have what is essentially a normal life.

Jack Rychik, MD: By closing the hole and relieving the outflow tract obstruction, one is recreating a situation that's relatively close to the normal heart. However, because of the initial element of narrowing in the right ventricular outflow tract and the need to enlarge the narrowing, there can be leakage of the pulmonary valve.

Thomas Spray, MD: And while that is very well tolerated there are some people, as they get into adulthood, where the amount of that leakage causes the right side of the heart to enlarge, and they need to have an artificial valve put in on the right side of the heart. So there is some risk of needing additional surgery after these repairs.

Robert Shaddy, MD: Unfortunately, as part of Tetralogy of Fallot, you're left with a problem related to a valve that did not develop normally and often needs to either be replaced or dealt with as the child and young adult grow.

Gil Wernovsky, MD: I am now telling families to expect that something will be needed to be done to the pulmonary valve over the lifetime of a child. Now, had I said that 15 years ago it always meant reoperation, but there's amazing new strides being done in catheter techniques. In fact, valves can now be put in in a same-day procedure through a heart catheterization, rather than open-heart surgery.

Robert Shaddy, MD: The idea is to put the valve in through a catheter and to place it into the position, in the pulmonary position, and then to let this valve be seated there and then to see how this valve does long term.

Gil Wernovsky, MD: And what will be available to these children 20 years from now is only speculation, but I'm sure it's going to continue to improve.

Jack Rychik, MD: There are some patients who continue to have some evidence for residual narrowing, even after initial relief of the pulmonary stenosis. In a small percentage of patients, there can be difficulties and problems with narrowing that occurs downstream of the pulmonary artery in the vessels within the lung itself. And those narrowings can oftentimes be treated with catheter-type techniques, or sometimes they may even require placement of a stent through a catheter.

Gil Wernovsky, MD: In addition, we've been concerned over the years about rhythm problems in some patients that have had tetralogy.

Kim Persick: I have a sister that is six years younger than I, and she was born with tetralogy. She was operated on at 15 months, and she herself needed a pacemaker.

Gil Wernovsky, MD: The incidence of palpitations or the need for rhythm medicine has not been uncommon in the older patients. And the techniques of the surgeons have gotten so much better that they actually can repair the tetralogy in very small hearts with very small incisions. So we think the likelihood of the substrate for the rhythm problem is going to be less in children repaired today compared to even a generation ago.

Kim Persick: Her heart disease was a little bit more than Robert's. And growing up knowing what a normal life she had, I knew that he would be okay.

Jack Rychik, MD: So repair after Tetralogy of Fallot can result in a normal quality of life, and in the vast majority of these patients there is very little residual problem or issue that they need to deal with. Certainly, these patients need to be followed by a cardiologist, and they need to be seen and evaluated at regular intervals.

Thomas Spray, MD: But, in terms of the ability to do things and to have children of your own and grow up and be employed, people with Tetralogy of Fallot do extremely well. And there are many adults now with congenital heart disease. In fact, 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 at 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.

Kim Persick: Robert, he's nine years old now. He is very active. He plays baseball. He plays soccer. He rides his bike. He always wants to be outside. Robert is lovable. He's funny. He's a great kid.

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: nd 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 20s. 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.