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Single ventricle malformations are a group of congenital heart defects in which one of the heart’s pumping chambers (ventricles) develops improperly and cannot effectively circulate blood. In this video series, you'll learn how experts in the Fetal Heart Program at The Children's Hospital of Philadelphia diagnose and monitor single ventricle malformations before birth, allowing effective treatment to begin right after delivery.
Thomas Spray, MD: We initially thought that this kind of heart defect was so severe that even if we could stabilize children and do these staged operations, that all of them would end up needing a heart transplant at some point.
Gil Wernovsky, MD: But, fortunately, for the last two decades, the frequency with which that happened has been surprisingly and gratifyingly low.
Robert Shaddy, MD: I think the single ventricle spectrum, which includes hypoplastic left heart syndrome, is an area that is always going to be at risk for problems related to heart failure, problems related to needing a heart transplant, problems related to multiple surgeries and even after all their surgeries are done, multiple medications, even possibly pacemakers, and then the ultimate of replacing the heart.
Thomas Spray, MD: In single ventricle malformations the Fontan operation has been around since about 1970. So there are some people now in their 30s and 40s that have had these operations and completed the Fontan.
Anthony Ramsay, Father: And they're doing fine, which is good, right? But we don't have anyone into, like, you now, long life — 50s, 60s. But, you know, we're hopeful. I think, you know, especially this hospital the way they're growing and the stuff, and the research they're doing, they're really looking into, you know, how can we help these kids?
J. William Gaynor, MD: They're basically on a medical frontier. They're on the cutting edge of medicine right now.
Gil Wernovsky, MD: About every four to five years, we make major strides in changing the operation and the way we approach these children.
J. William Gaynor, MD: So really a child who survived all this in the late 80's and is now late teenage or early adulthood is very different from a child having surgery today. So we have a moving target because we're getting better at what we do.
Thomas Spray, MD: Only time will tell what the long-term outcome really is.
Gil Wernovsky, MD: But what we have learned, especially over the last five to six years, is how these children are doing in school age, high school, and as young adults.
Lynne Ramsay: Joseph is a pip. He's very friendly. He says hello to everybody he sees. He is just very vivacious and very smart. We can't believe how much he picks up and — he's just a joy.
Gil Wernovsky, MD: The majority of children have normal learning, normal intelligence, and do well from an integration and social perspective. However, the frequency of either mild learning problems, attention deficit disorder, maybe some delays in speech and language is higher than the general population, maybe twice as high as the general population.
Robert Shaddy, MD: So it's not just limited to their heart. It's limited to the neurologic abnormalities that are beginning to show up that in some children are very minor in others are more significant.
Sarah Tabbutt, MD: We've really pushed the field in, you know, making this a heart disease that is not only not fatal, but is actually very survivable with good outcomes. And shouldered on that is the transition to trying to optimize neurodevelopmental outcomes —
Gil Wernovsky, MD: There are many reasons why the brain might not be the same as a child without congenital heart disease. It's not just the surgery. It's the effect of the oxygen levels. It's the effect of being in the Intensive Care Unit. It's the effect of having a chronic condition. It may even be the effects of the circulation when the baby's inside the womb. We're just beginning to tease out the causes of these increased incidences of learning problems.
The follow-up for children with single ventricle involves comprehensive evaluation of the heart muscle function, of the surgical connections that were made as part of the stages of reconstruction, of the pressures that are present in the various chambers of the heart and the lungs, the rhythm of the heart, and a comprehensive general health assessment of children with this very complex plumbing rearrangement.
The most common long-term issue that we've seen to date has been rhythm problems with the heart. And those rhythm problems could either be too slow or too fast or irregular. And we believe that the cause of those problems is the surgery that's necessary to do the Fontan operation is right in the area where the heart's natural pacemaker resides. About 15% of children, by the time they reach adulthood, either need a medicine to keep the heart rhythm normal or a pacemaker to keep the heart rhythm normal. And that's something that we'll need to follow as children get even older. What we see in children as they get older is no symptoms in day-to-day activities or even mild exertion, for the most part. But when they're doing high-exertional activities, such as youth sports, their endurance tends to be on average about two-thirds what other kids can do without a congenital heart problem.
Thomas Spray, MD: Many of these children will have issues when they get older.
Gil Wernovsky, MD: 1 in 5, 1 in 6 children may need an operation that's unplanned in addition to the staged reconstruction.
Thomas Spray, MD: Some will require additional procedures of various types. Some may have rhythm problems that need to be addressed. And some will probably eventually need a heart transplant. But 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.
Anthony Ramsay: After the first surgery, you know, you always talk about blue babies and all this other stuff. And he came home he was, you know, pink. He's warm, you know, he's running around. He's the only one out of the three of them that broke his arm. He climbs on everything. You know, you'd think he'd really be tired. He's not. He runs around like crazy.
Lynne Ramsay: We're so lucky. We're so blessed and lucky that he was born where he was and when he was and that he has these people taking care of him. We wouldn't want to be anywhere else but here.
Anthony Ramsay: It gives us a lot of hope. You know, that he'll live long into the future. And that's very comforting.
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