The Children's Hospital of Philadelphia
www.chop.edu
Hypoplastic Left Heart Syndrome — Life After HLHS Surgery

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: As the results of surgery become better and better, we think that heart transplantation is a less attractive option.

Sarah Tabbutt, MD: Probably the most difficult surgery that we do is the palliation for hypoplastic left heart syndrome. And the survival of those babies, if they don't have any other complications, is now like greater than 90%.

Shane Thrush, Father: At this point we're at an every-six-months appointment just for basic check up and--

Andrea Thrush, Mother: --it's the standard EKG, vital signs, oxygen levels, relatively brief checkups. They spend more time talking to each other and catching up.

Gil Wernovsky, MD: One of the most common questions we get asked is, "How are the older children with hypoplastic left heart syndrome doing?"

Thomas Spray, MD: Many children are now in their early teens with this kind of heart condition, and they're doing quite well.

J. William Gaynor, MD: They're basically on a medical frontier. They're on the cutting edge of medicine right now.

Thomas Spray, MD: We don't have any children with hypoplastic left heart syndrome who are over about 22, 23 years of age.

Peter Gruber, MD: Because it's only been done for a little over 20 years, per se, from the very first operation.

Thomas Spray, MD: The surgery for hypoplastic left heart syndrome has evolved since the early 1980s and continues to evolve.

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 80s 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.

Andrea Thrush: She is so full of energy. I mean, you can't even believe she has a heart defect. And people that don't know what she's been through look at her in awe of what she's capable of.

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.

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 particularly for babies with hypoplastic left heart syndrome.

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. 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.

Andrea Thrush: When you again go from being told a diagnosis like hypoplastic left heart and feeling a lot of uncertainty and anxiety, you visit here, and you realize there's knowledge and compassion and comfort at CHOP.

Shane Thrush: You walk in here and you just feel, you know, that Morgan's best interest is in their best hands. So--

Andrea Thrush: That's one of the reasons why we chose her middle name to be "Hope" because it was such a strong feeling and sense that we got every time we came and left.

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 C. 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 C. 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.