Hypoplastic left heart syndrome is when the left side of the heart, the part that pumps oxygenated blood to the rest of the body, is underdeveloped. Coming up, you'll hear from a leading pediatric cardiologist about how this condition is diagnosed and treated in little babies, next on Patient Power.
Hello and welcome to Patient Power sponsored by UCSF Medical Center and UCSF Benioff Children's Hospital. I'm Andrew Schorr. Well, I have three children, and we are so fortunate that the pregnancies went pretty well and we ended up with healthy babies. Hopefully that happens for everyone, but of course it doesn't always. One condition that can happen maybe one out of every 3,000 births in America is something called hypoplastic left heart syndrome. It can be a real shocker for mom and dad. It certainly was for Stephanie and Brent Stuckert of Petaluma, Calif. Stephanie, was pregnant with her first child, Olivia. We're going to hear her story. Stephanie, what happened six weeks before your due date?
About six weeks before my due date, my water broke unexpectedly. Brent and I went to Kaiser Santa Rosa where we were going to give birth to Olivia. When we were there, they did an ultrasound because I was so premature. I understand that's probably routine. They did an ultrasound and they saw something, that something was wrong with her heart.
Something didn't look right. So you find yourself first you thought you were going to another Kaiser facility, and then they said, uh-huh, you're going in the ambulance to UCSF Medical Center where they have specialized pediatric teams. What happened then?
When we got to UCSF, I was in active labor at that point and Dr. Grady from UCSF did a fetal echo on me and saw that Olivia had hypoplastic left heart syndrome.
Now, you'd never heard of that.
I'd never heard of that, and honestly, we just thought because we had had ultrasounds, prior ultrasounds, that everything was fine with the heart.
The baby had been developing normally, kicking, all the things moms look for?
Exactly. I was measuring a little small, but I was still within normal range, which is what I was told, and there didn't seem to be any other complications. I really thought that our biggest issue at that point was that she was six weeks premature, and it was quite a surprise to find out there was an issue with her heart.
A condition that could be fatal just within days or weeks of a baby being born with it. So there's this swarm of white coat people around you, and how did it go? So they explained that Olivia had this very serious heart condition. They had this big team. What happened next?
Well, at that point I think you're just in shock, and there's a whole array of emotions going on and shock and disbelief and anxiety and fear, and then also excitement because you're about to meet your little girl for the very first time.
She was then born but weighed, what, 3.5 pounds?
She was born and weighed 3 pounds, 12 ounces.
At that time ,they wanted to try to keep Olivia inside longer, for about a week, and they gave me medication to try to keep her in but she just, she wasn't . . .
She wanted to come out. You were there at UCSF how long?
We arrived at UCSF on Jan. 8, and we were there for two and a half months.
Now, the happy story that we're going to tell is Olivia is there in your house now, thriving and growing. She's about 17 months old, and I think she's having a tea party right now with your husband, Brent?
She is. She's downstairs with daddy having a tea party. Daddy and the doggie.
She's walking and babbling and thriving and growing.
She is. She's doing so well. She's just thriving and she looks healthy and she's got pink, cute little cheeks and you'd never know by looking at her all that she's been through.
Well, let's meet one of the doctors responsible for that. That's Dr. David Teitel. He's medical director of the Pediatric Heart Center at UCSF Children's Hospital. First of all, Dr. Teitel, you must love hearing stories like this.
Yes, that's what we work for and live for.
Let's understand this condition. So a baby like Olivia is born there or is quickly brought to UCSF with this condition that many people, as I said, have never heard of, hypoplastic left heart syndrome. What is that syndrome? What does it mean?
It basically means that the left side of the heart, which as you said at the beginning of the show, isn't developed enough to supply blood flow to the body, and the body needs the blood for oxygen to survive. As a fetus it's not important to have both sides of your heart because the lungs aren't doing anything. So either the right ventricle or the left ventricle can pump adequately to the body in the placenta.
But as soon as you're born and the right ventricle starts pumping blood to your lungs, you've got to have a pump to your body. If the left ventricle is not there, you're not going to live very long.
So if untreated, a baby would die in a short time.
Yes, usually within a day or two.
Oh, my. All right. Where are we now with what you're able to do and what you did for Olivia? Maybe you could describe what comes into play to have a child now be 17 months old and thriving.
Well, if you only have one pump, the right ventricle, what you've got to do is get that ventricle to pump blood to the body and get blood to the lungs by some other way. So immediately at birth, we use a medicine called prostaglandin E1, which is a naturally occurring hormone, that when given to a baby keeps the ductus arteriosus open, and that is a little tube that connects the artery to the lungs with the artery to the body. So while that tube is open with this intravenous medicine, the right ventricle can do the work of both pumping blood to the body and the lungs.
The next steps are to figure out a way without using an intravenous medicine to let the right ventricle do the work, and that basically is a series of three operations over a period of time — from immediately within the first week or two of life through the first three to four years of life where you get the right ventricle to do the work of pumping blood to the body and allow blood to go passively into the lungs.
So, Stephanie, your child, Olivia, has had two of those procedures so far, right?
She has. She had the first one, the Norwood procedure, at about 2 weeks old, and then she had the second procedure, the Glenn, in October of 2009, and I believe she was about 10 months old at that point. And she will likely have her third between the age of 3 and 5.
So, doctor, with those procedures being successful, hopefully a third one just as well with Olivia, what does that mean for the kid growing up?
Well, as long as that right ventricle stays healthy, it means that she'll have a normal active and healthy life. She'll have to continue under medical, especially pediatric cardiac care for her life because we have to do everything to support that right ventricle. It's not built to pump blood to the body. It's built as a much less strong pump for blood to the lungs. But we found that by doing these procedures in these stages and helping it get along, to get used to pumping blood to the body, that many of these kids can live for decades and have perfectly healthy lives. And then if the right ventricle has some problem , we do other things to help out.
Stephanie was very fortunate that you could get to a center such as UCSF where they know how to do all this really rapidly. I imagine you feel very thankful.
I can't even put into words how thankful I feel. One, we're so fortunate to live in this area and to be so close to UCSF, but mostly our gratitude is just . . . there's not a day that goes by that we don't think about the team at UCSF and how amazing they are and how they saved our little girl's life, and they . . . we are just so grateful.
Want to say anything, Dr. Teitel?
Well, when you hear Stephanie talk about Olivia, there's not really much more you need to say. As I said, this is what we really work and live for . . . to be able to help people like the Stuckerts and Olivia to give her a normal life.
Stephanie, is there anything you want to say to him here?
Oh. Absolutely. Dr. Teitel, Brent and I, we want to say thank you to you and to everybody at UCSF. Like I said, not a day goes by where we don't think about you, all of you, and are grateful for what you've done for her and for our family. We can't imagine our life without her, so we really appreciate it.
And now you have another one on the way, right?
I do. We have another little girl.
Oh, that will be so neat. Well, they'll have a tea party together.
So let me ask you this, Dr. Teitel. So not everyone is blessed with getting rapidly to your center or one like it so quickly or maybe they don't even know or maybe it's not recognized. So first of all for families concerned about this, what would be the signs? Would there be any signs in pregnancy that someone could be aware of that might give some indication?
There really aren't any signs because, as I mentioned, you really only need one ventricle as a fetus. You don't use your lungs to get oxygen to the body, so there's nothing wrong with the baby as a fetus. But if you do get good care throughout your pregnancy, more and more the fetal cardiologists are training ultrasonographers to recognize heart disease and if they do, to send them to us for evaluation.
Even about 10 years ago, there were almost no babies diagnosed prior to birth with hypoplastic left heart syndrome. Now I would say in California about 30 percent are diagnosed before they're born, like Olivia, and mostly, significantly earlier than Olivia. So I think the main thing is to get good obstetrical care, and with time I think more and more of these babies will be diagnosed before they're born, which is really important because they can get very, very sick right after birth and not have the type of wonderful outcome that Olivia has had.
Now, curiously, Stephanie's water broke six weeks early. Would that have been related, or in a sense was that a good thing or bad thing, but is it connected at all?
It's not connected at all, but I think it was very fortunate because as Stephanie said she had an ultrasound done because of the water breaking and then somebody very astutely picked up that Olivia's heart wasn't normal. If she had just gone through labor and delivered in Santa Clara, Olivia wouldn't have been diagnosed until she got very sick. As the ductus arteriosus starts to close the babies start breathing hard, they get clammy, they look like they're in shock. So they can have a lot of problems and organ damage during that time before you diagnose it. So I think it was serendipitous that her water broke.
Wow. The world works in curious ways.
Doesn't it Stephanie?
It sure does.
Okay. Well, we have a good story with Olivia. Now, is there anything — first of all, I imagine it this doesn't run in families — that happened during a pregnancy or mom didn't eat right or something that would lead to this?
No. It's not quite a random event, but, no, there's nothing that Stephanie did during her pregnancy that would have brought it on. These heart problems do run in families to a small degree. So Stephanie having had Olivia with heart disease probably has about a three- or four-fold higher chance of having a child with heart disease. But since her chance was only 1 percent before, she still has a 97 percent chance of having a normal baby. So any woman who has had a baby like Olivia or any baby with heart disease, we recommend doing fetal echocardiograms to see exactly what's going on with the other baby. And Stephanie, I believe you've had one, right?
We have, and everything is normal.
Okay. Stephanie, what would you say to other parents, let's say if something is spotted by that smart ultrasonographer, what would you say to the parents as far as getting the best care?
Wow. As far as getting the best care, that's hard to say. In a way, I think the best care kind of fell into our lap. And as far as getting through something like this, I can speak better to that.
I think looking for some sort of support group or network, I was put in touch with someone whose daughter is 12 with hypoplastic left heart syndrome, and was able to e-mail her throughout the process and ask questions, and her daughter is doing very well, and it was really helpful for us to just kind of ask her . . . she'd been through it, and ask her questions about — A, how she got through it or, B, you know what specifically we might expect as far as six months down the road or eight months. It just put our minds at ease in a way. It was nice to have a resource, somebody to reach out to who had been through that process.
Dr. Teitel, I imagine, and I alluded to this earlier, a pretty big team to help families afflicted by this.
Yeah, it's an enormous team, and that's why pretty well all the care is done at major university medical centers. You have neonatologists, intensive care doctors, cardiologists, cardiac surgeons and a variety of various nurse specialists and respiratory therapists, so it's a really big team to deal with a really tiny baby to get the outcomes that we get.
Now, where is technology headed and your techniques, because I noticed that there have been adaptations with these surgical procedures over time. So do you continue to refine it?
It's constantly being refined. One of the problems in pediatric cardiology is that we're changing things so quickly it's hard to know what the best technique is. Because as soon as we start studying one technique, somebody else comes up with a brilliant idea for a newer one, so we're moving on to doing less invasive, more with catheters and tubes, which is what I do. I'm a cardiologist, and I use catheters. I'm not a surgeon. Moving toward less invasive care for these children, doing work together with the surgeons to minimize the children's time in the hospital and the stress to their growing bodies, especially their brains and their hearts.
So there's a lot of change going on. And I've been doing this as a pediatric cardiologist since 1980, and when we started we didn't even offer care to babies with hypoplastic left heart syndrome. We felt there was nothing to do, and now over those 30 years we've gone to not only offering care but offering the really strong possibility of a long healthy life.
So the point is if someone, parents, hear this term applied to their baby who is about to be born or is being born, the truth is they want to have a connection with a center such as UCSF fast so that that life can be saved and that baby can thrive.
All right. Well, it is really a happy story, Olivia having her tea party right now as we speak and doing well, and, Stephanie, that you and Brent were able to connect with the right care at UCSF.
So, doctor, are you optimistic that you'll continue to perfect these procedures and when it comes to not just this problem with the heart but others really more broadly in little tiny babies or maybe even sometimes even in utero that you'll continue to refine what you're doing to save more lives?
Oh, absolutely. That's what our goals are for every day that I come to work . . . it's to be able to offer little children like Olivia the chance to have a healthy life, but we've gone a long way since I've started doing this, but there's a lot further we can go.
Well, that's a great story. Stephanie, you are going to dance at Olivia's wedding some day, and she'll be pouring tea or something for everybody, and then you can look forward to another daughter. Well, thank Brent, your husband, who does his own lifesaving work as an EMT in San Francisco. Thank him for being at the tea party while we get to have this conversation.
I will. I will. He's actually a lieutenant in the San Francisco Fire Department, but I will definitely thank him for you. He's disappointed he couldn't be here.
Okay. But what a joy to be with Olivia.
Oh, every day, we wake up and it's a joy.
Wait until she's a teenager. That's another story. Well, Stephanie Stuckert from Petaluma, thank you so much. All the best to your family. And Dr. David Teitel, medical director of the Pediatric Heart Center at UCSF Children's Hospital, thank you for being with us.
Thank you. Another inspiring story of medical advances, a talented team, and very devoted parents in this case on Patient Power. Thank you so much for joining us.
Recorded June 2010
Reviewed by health care specialists at UCSF Benioff Children's Hospital.
This information is for educational purposes only and is not intended to replace the advice of your doctor or health care provider. We encourage you to discuss with your doctor any questions or concerns you may have.
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