What a congenital heart defect could look like after delivery: Amy's birth story + Ultrasound insights, Part II

In today's episode we hear part 2 (of 3) of Amy's story--she talks about her induction and her experience becoming the parent of a baby with a significant heart condition, the operation that saved his life, and tips for parents who might find themselves the new minders of a fetus with a congenital heart defect. 

I also include the insights of a doctor who performs fetal cardiac ultrasound to understand that technology better. 

And the last thing I'll add in the interest of transparency, is that like Amy, my partner and I had a child with a significant congenital heart defect that required emergency surgery when she was born, so a lot of what she says really resonates with my experience. 

We pick up where we left off last week. Amy is learning about her future son's congenital heart defect from her doctor.

Transcript

Paulette  0:02  
Welcome to what you didn't expect in fertility, pregnancy and birth, how we think and feel about this enormous transition often lives in the gap between what we expected and what we actually experienced. This gap exists in part because of how we tend to talk about and portray these events on all kinds of media in a one dimensional way, everything was amazing, but it's more often the case that there are beautiful things that happen, and at the same time, really challenging things that happen. This show shares true experiences, both the easy parts and the difficult parts, and how we manage what we didn't expect, the intense things that can happen in the course of this transition, it impacts how you see yourself, how you see your partner and how you parent. The better we understand what happened to us, the better we can manage all the things that follow. I'm your host, Paulette Kamenecka, a writer and an economist, a mother of two girls, and I met many, many challenges in this process, none of which I expected in today's episode, we hear part two of three of A's story. She talks about her induction and her experience between the parent of a baby with a significant heart condition, the operation that saved his life, and tips for parents who might find themselves the new minders of a fetus with a congenital heart defect. I also include the insights of a doctor who performs fetal cardiac ultrasound to better understand this technology. And the last thing I'll add, in the interest of transparency, is that, like Amy, my partner and I had a child with a significant congenital heart defect that required emergency surgery when she was born. So a lot of what she says really resonates with my experience, we pick up where we left off last week, Amy is learning about her future son's congenital heart defect from her doctor.

Amy  1:49  
So I had to go to that appointment alone because of all the covid regulations. I walk into the room, and doctor comes in and she says, I'm really sorry, but it looks like your son has a very rare heart defect called transposition of the great arteries. Wow, that is a rare one. And they said basically, what that means is that his arteries grew in backwards, and I didn't want pun world to crumble around me. I feel and cope with stress through research and information. I figure if I know a lot about it, then I can't be scared about it. Yeah. And so I looked at her, and I said, what are his chances? And she said, I don't know. And I said, Okay, is it fixable? And she said, I don't know.

Paulette  2:40  
So what level, kind of hospital is this teaching hospital, or

Amy  2:44  
small? I am in a big city, Edmonton. I'm definitely in the right place for child with a heart problem, okay, the Stollery does approximately half of Canada's heart transplants for children in the country, okay? And we have a world class Heart Clinic in general. The hospital that I went to is partnered with the Stollery. It's called the Royal Alexandra, and they have loads of world class doctors who do lots of work there. So I definitely felt that I was in the best of times. And you know, some of the first people that I reached out to when I got this diagnosis, where those friends who have had similar experiences at the end of the year in crisis, you're looking for somebody who's had something similar, and they said, I know this is really scary, but you are in the right place, and they're going to take care of you as best as they can. Good. The waiting time in between there was almost unbearable. It was about a week between that doctor appointment and the diagnosis. But in that time, I went and googled as much as I could, look at all the scholarly research articles that I could find, and what I learned was that transposition of the great arteries is repairable with a surgery called an arterial switch, and it has a 96% success rate. So I felt confident going into that appointment. I show up and they're like, you're in a really good mood. And yeah, I know what to expect. I've done lots of research. I want to look at plenty of videos that walk you through the surgery and any other medical procedures that might happen only on transposition of great arteries. And sure enough, they confirm that that's what it is. And I get pulled into one of those counseling rooms and they said, Okay, your son has a fatal heart defect called transposition of the great arteries. And I'm like, yep. And she says, because it is a fatal heart defect, you do have the option of terminating your pregnancy.

Paulette  4:59  
Wait, let me pause right here for a second. Why are they calling it fatal if we can fix it?

Amy  5:04  
Because it is fatal if left untreated. Okay, not all heart defects will have the same amount of success,

Paulette  5:16  
of course, but they must know that yours has a great rate of success.

Amy  5:20  
They have to give that option, because that's the standard of care that they have. I know going into this that our care afterward is pretty simple, but not everybody who has a fatal heart defect or critical heart defect has the same quality of life that my son will have.

Paulette  5:41  
On the one hand, I know what you're saying, but I feel like their response to you is not specific enough to your to your case, yeah,

Amy  5:49  
they just throw the option out there. They don't expect you to make a decision right away. Yeah. They just want to let you know that these are the options that we have. Yeah, and I don't judge anybody for making a choice in that direction,

Paulette  6:04  
because, yeah, it

Amy  6:08  
it's hard given any given family, right? Everybody has a different level of comfort. Yeah, everybody's capability of having a medically complex child is going to differ depending on availability of hospitals and medication and care, it can be a lot of work for some children. And I don't think it would be fair to say, Oh, well, only these ones were going to give the option for and these ones were not. They have to say, okay, critical heart defect in general, because it is a big branch. This is the option that we do. Yeah,

Paulette  6:46  
I guess I'm imagining they would say it is a critical heart defect. But

Amy  6:54  
they we did have that conversation later, so that was from the diagnosis itself. So they did basically a once over again, doing an anatomy scan at about 30 weeks pregnant. After that appointment, I had a fetal echo, he took very detailed pictures and look at his heart to see if there was anything else going on. And thankfully, according to that, there wasn't. And so the counselor came in, doctors came in, and all of them came the social worker, and they said, Okay, so this is the diagnosis that your son has, and they're expecting me to kind of this is what will happen, and this is what'll happen, and these are the options. Okay, so what is it that they do? I'm a teacher, but I've been working in early health education. I just really love research. Yeah, okay, so you know what's going on, and I'm glad I don't have to repeat myself or give you any surprising information, like we hope to induce you somewhere between 39 and 40 weeks, and then we'll have a very planned delivery. So we had a game plan. I had fetal echoes every other week, I had to be shifted to a high risk pregnancy facility for my OB appointments, even though, personally, I was not at high risk. Yeah, the pregnancy didn't change at all. On my end.

Paulette  8:15  
Did you have a fetal cardiologist?

Amy  8:19  
Kind of I have, my son has his own cardiologist, and I was doing research through the University of Alberta, so I opted to be a part of of that, because the heart is very different in utero than it is after they're born. So we were testing to see if the foramen o Valley, which is a valve in between the atriums. That's how oxygen gets between both sides of the heart. In utero, they were testing to see if it would remain open after birth or if it would close, because that that Well, eventually close after birth, and if it closes, then there's no way for oxygen to get to my son's extremities. So I went through multiple testing with that, and I didn't mind, because it meant that I was being checked on more often, and it looked as though his foramen o Valley would remain open after birth. So we're like, Great, that sounds good. We'll see what happens. But you know, 56% of the time that cold does close right after birth. You need expected,

Paulette  9:27  
if that thing doesn't feel about a mixing oxygenated and deoxygenated blood. The

Amy  9:32  
problem with my son's heart condition is that because the arteries are switched, there is no way for oxygen to get these extremities. So the right side of his heart is going body, heart, body. And the left side of his heart is going lungs, heart, lungs. They never cross, okay, that hole in between the atriums allows the oxygen to go to the other side of his heart so that it can reach the extremities. Okay, so that's like a short term fix Exactly. That was a big part of his his birth plan, and keeping him alive until surgery, is making sure that that hole remains open. And there's another part of fetal anatomy called the ductus arteriosus, and it's basically a shunt between the pulmonary artery and the aorta. So it's another little shortcut that oxygen can travel in utero, and that does eventually close up after birth as well. So in order to keep that open, he had to be given prostaglandin, which was a naturally occurring hormone inside of a fetus while they're in utero, and that signals that they still need to keep that open. So he had to be given that medication immediately after birth to help keep that open. In contrast,

Paulette  10:54  
what we know about fertility, what we know about cardiology, it's lucky to be on the cardiology side at this point, right where there's so there, there's so many details that they know well about how everything is working. So that feels lucky,

Amy  11:08  
absolutely. I mean, for having a child with a heart defect, I'm really grateful that it's happening now. And I mean, a lot of the successes of people with heart issues has only come to fruition in the last 35 years. Really recent? Yeah, it's almost startling how how recent it all is. And people who have had heart transplants and have survived heart defects, congenital heart defects, are only around 35 or 40 years old, so we still don't have the complete data show long term health. Yeah,

Paulette  11:45  
we we keep fingers crossed at this point. Yeah, exactly. So take us to the birth. How does that all go down? Yeah.

Amy  11:52  
So I was induced. My sister's very kind and took my daughter for the time when we were in the hospital. I said, I don't know how long it's going to be. And she said, We'll cross that bridge when we get to it. We'll make sure she has a lot of fun. And they did. They ended up taking her out camping, and she was having the time of her life. So we didn't have a child to worry about in and amongst going to the NICU, because we knew that that's where we would spend a lot of time. So I have my induction on July 31 I show up at the hospital with my wife at about eight o'clock in the morning. I go through the fetal stress test, and they check my cervix and all that, and they say, Okay, I think we're ready to go. They go to call the salary Children's Hospital, which is across the river. It's not in the same location as the delivery place. And I said, Oh, they're not sure if they can take you today, so we're on hold. Oh, okay, so

Paulette  12:51  
I I feel stressed by this. Are you stressed by this?

Amy  12:55  
I mean, at the point in time, what else could happen? So we're just like, Okay, well, I guess we'll see what happens. And my wife and I, we brought them to change. And, I mean, it kind of worked, but I've got butterflies. It's a big day or not, we don't know. Yeah, four hours roll by and I look at my wife, I'm like, it's not going to happen today. And the person comes by and pokes her head into the curtain, okay, we have to go ahead so we're gonna start now. Like, oh, okay, so this is actually happening. And it's very strange to be like, Okay, we're starting this whole process, and my uterus is not at all ready. I wasn't even close to being dilated. He was very cozy in there. So they end up doing the Foley catheter, which is a very fun process. They insert a water balloon basically against your child's head and your cervix and wait for you to be three centimeters dilated. That was probably the most uncomfortable thing I have ever experienced in my entire life.

Paulette  14:10  
That does not sound

Amy  14:11  
comfortable, no, it's awful. And they put a surprising amount of water in there,

Paulette  14:17  
and it's like opening your cervix essentially manually or with a

Amy  14:21  
balloon? Yes, exactly. So the hope is that the weight of the baby and you're supposed to get up and, like, walk around, and that gravity will slowly open up your cervix. But again, like I mentioned before, my body seems to get the message very quickly. They filled up the water. I was cringing in my seat. My wife's like, oh my gosh, she's not okay, because it was very uncomfortable, very painful, and 30 seconds after it being filled with water, it comes flying out and gets everybody wet in the room. What is happening? And sure enough, I was three centimeters dilated. Oh, wow, yeah. So I was very grateful to have had a very quick experience with the holy catheter. I don't know how long I would have survived otherwise, but it happened very quickly. And they checked and saw that I was three centimeters. They said, Okay, you're going to labor and delivery.

Paulette  15:13  
Oh, okay, I

Amy  15:14  
just started this whole process, and now I'm going to deliver a baby. So I walk over to the next ward, and very much a similar story. I said, okay with my first once we started Pitocin, she was out within four hours. So just be aware that my body does respond to things quickly. They definitely slowed things down than they did with my daughter, which is good. Had the epidural. I mentioned a it only works on half of my body. And they said, Oh, that's not a thing. So had to go through all of that again. I accept this. I don't know if they used a different epidural medication, but this time it I could feel the freezing slowly climbing up my body, and it froze me up into my arms. And at that point, I was starting to get a little bit worried, because I had to think about my breathing a bit more. It wasn't coming as easily and as naturally, but that eventually went away. So I think I started my Pitocin somewhere around one o'clock in the afternoon, around six, he started going a little bit into distress with each contraction is oxygen would drop, and they're like, Oh, this isn't very good. It wasn't getting any better. So they drew some blood and said, Okay, I'm just going to prep you, just in case you need to go in for a cesarean. We want to make sure we have everything that we need. And at that point, I'm starting to panic, because I know that recovery from a cesarean section is far more challenging than a natural birth, and that will inhibit my ability to see him afterward. So I'm like, okay, come on, buddy, we got to get through this. You can do it. And I roll around a bit, and shift change happens, or somebody goes on break, and he moved and then finally, everything kind of just went back to normal, so he wasn't in distress anymore. Thank goodness. That took a huge load off. Just before 10pm they come and check me. And I had heard before that, you know, when it's time to deliver, it's going to kind of feel like you need to poop. I didn't have that experience with my daughter. I was completely numb with her. I didn't feel anything. And they're like, Okay, well, let's put you up in the stirrups and take a look at you. And I'm like, oh, oh,

Paulette  17:31  
okay, I

Amy  17:33  
think I need to push. Okay, so that's what that's supposed to feel like. And they're like, Okay, well, you can't push yet. The doctor who's delivering your baby is busy catching another baby right now. Okay, my wife is trying to lighten the mood, and so she starts telling jokes and I laugh, nope, nope, nope, nope. If I laugh, this baby's gonna come flying out of me five minutes later. Okay, let's deliver a baby. How is NICU? Is NICU ready for the baby? NICU pops their head in the door. We need about five more minutes, they said. And I'm like, oh, okay, holding on to this thing for dear life, hoping I don't deliver too early. And everybody was finally ready to go. It was 10 o'clock. I start pushing, and 15 minutes later, he was out. Oh, wow, yeah, my body gets the message, which I'm so grateful for. We knew that everything was going to be really quick. He was delivered. He did not cry. They said he might be blue. I didn't know at the time, but he was blue. He looked pink enough to me, but I mean, you don't really have a before and after picture at that point in time. So I as soon as I delivered, I sat up and I looked at him at the end of the table until his umbilical cord stopped pulsating. They cut the cord, and then they took him to the NICU room next door with him. I was stitched up. I laid there for about an hour while they did all of the post delivery stuff. They hooked him up to monitors and gave him prostaglandin right away and put him in a transport box. And the next time I saw him was about an hour later, and he was ready to go. And I remember looking at him, and they're like, Okay, we're gonna get ready to go across the river. I knew that I wasn't going to have much time with him, but I didn't expect it to be like, almost no time, yeah. And I remember just kind of sitting back and wondering, Am I allowed to touch him? Had this inner dialog. I have no idea what they were talking about. I'm like, of course you can touch him. He's your baby. And so I reached out and I gave him a rub on the head. And they're like, Okay, we gotta go. So wave goodbye as they roll, my wife went with him. She couldn't go in the ambulance because of covid, so she drove behind as they went across the river. And I stayed at the hospital overnight. And it's a very strange place to be after you've delivered and you can hear other babies crying and you're just sitting in a room by yourself in the middle of the night knowing what's happening on the other side.

Paulette  20:29  
Yeah, my daughter was whisked away right after delivery, and my husband followed the ambulance, just like your wife did. And it's weird to be on labor delivery by yourself. Yeah, it's

Amy  20:39  
a strange place to be, like, Can I do anything for you? I'm like, Can I have another sandwich, please? How

Paulette  20:46  
did you feel? Physically at that point, I

Amy  20:48  
still had all of the epidural and stuff in my body. So physically, I felt okay. Emotionally, I was like, Okay, we're in this now. Yeah, it's a very sexy experience, because you know when you're pregnant that your baby is as safe as possible, but as soon as baby comes out and has a heart defect that all bets are off, yeah, yeah, and there's nothing else that I could do personally to keep them

Paulette  21:15  
safe. I felt that once she was out, it was harder than I anticipated. Yeah. Is that what you felt as well. It

Amy  21:23  
was for a while I had this very concrete idea of what was going to happen through the copious amounts of research that I had done. I'm like, Okay, this, and then this, and then this, and then this, and then this. And so I'm like, Okay, this is the game plan, and as long as we follow these steps, we will get to go home. And so I was just checking off boxes. And I think when I'm stressed out, I get very factual and very clinical, and I push away all of the emotional stuff so that I can deal with that later, which I have slowly been unpacking over the last year, through everything that I've learned in the meantime, question,

Paulette  22:02  
at no point during your pregnancy, do they say this will be a problem for fetal development? No, good. Okay, so you definitely were not worried until birth.

Amy  22:13  
No. Because while he was in utero, he was getting all of the oxygen that he needed, because the lungs are not yet a part of the game. Yeah, their lungs are completely and those secret passageways, the foramen o Valley and the ductus arteriosus, are what keep your baby alive with oxygen. So I knew that he was okay as long as he was in there.

Paulette  22:39  
And transposition of the great arteries comes alone. Doesn't come with anything else. Usually it depends,

Amy  22:45  
okay? CHD, which I'm sure you've learned in the meantime, can come in many different combinations and permutations, yeah, but it was the only problem with that his arteries were methods, okay,

Paulette  22:57  
and at 28 weeks, enough of the fetal heart is formed. Think anything else is going to happen?

Amy  23:04  
Yeah, there are some pieces that are definitely harder to see in negro. The game plan was after he's born, to do another another echo now that he's out, so they can get a better picture, and just to see exactly where all of the things are, where his coronary arteries are. How big his arteries are? His pulmonary emborda make sure there's nothing else that they missed and go from there.

Paulette  23:30  
Okay, so how long need to stay in your hospital?

Amy  23:33  
I was luckily able to be discharged the next morning, but we did have a very long and eventful night, so after they transported him to the hospital and they got him into his room, as soon as they laid him down in his NICU bed, his oxygen saturation plummeted dangerously low. His perimeter belly had closed. So in order to survive, they had to medically paralyze and sedate him, and they raced basically like my holy urine, a catheter up and into his heart, where they inflated a balloon and ripped the world back open between two, wow, yeah, between the two atriums. Wait. Where are they putting the catheter? I believe it's through the groin, or sometimes through the umbilical cord. Okay, yeah, so they had to get that up and in there. And Sam said it took three very violent polls for his oxygen finally to to get back up to an acceptable level. And it just goes to prove that early detection of congenital heart defects saves lives. Yeah, had we not known that he had a problem with his heart beforehand, he would have died. Yep, if he took him, yep, it wouldn't have even been at home. He would have still been at the hospital. Was it within three hours of birth, his oxygen would have plummeted, and the doctors would have been scrambling trying to figure out what was going on. Yeah. And it still remains today that half of heart defects are missed pre if not more, although 30 to 50% of heart defects are caught in utero,

Paulette  25:12  
Amy and I talked both about the importance of picking up these heart anomalies as early as possible, and some of the obstacles to doing this. I took this issue of fetal heart monitoring to an expert to learn more about the issues involved in detecting congenital heart issues. Today, we are lucky to have Dr Jawad Mustafa with us. Dr Mustafa, thanks so much for coming on. Can you introduce yourself and tell us about your experience? Thank you for the opportunity.

Dr. Mustafa  25:39  
I'm Dr jamad, I am from Pakistan. I'm working as a family physician for 18 years now, and I have done my M critical ultrasound. One reason I

Paulette  25:50  
wanted to talk to you today is about the technology of ultrasound as applied to pregnancy. So we're looking at fetal bodies and trying to decipher what's going on in them. So let's talk specifically about fetal hearts. Congenital heart defects are one of the most common defects of pregnancy, and I'm wondering how difficult or easy it is to determine if that exists. If you're looking at a fetus that's between 18 and 22 weeks developed,

Dr. Mustafa  26:23  
actually, there are four variables that are involved in any fetal heart scanning or any fetal structure. The one is the examiner, the practitioner or the doctor or the technician. The second is mother herself. Was through her abdomen. Those structures right from the skin till they notic throat sac in which the baby is present if it is present. So if the amount of fat is more, then it can reduce the amount of sound waves those are penetrating inside to gain the image. The third variable is the Amazon machine itself, because if it is an advanced, high level machine with higher capacity to send and receive, then it can prove a better image. And the force variable, which is the most tricky one, is the fetus itself, because it is sleeping, then it is easy to get the information, but if it's kicking, then it's very hard to get the proper image, because all the time the fetus is moving, the heart is also changing position. So it is very hard at times again, even if all the three variables are good, if the fetus is moving, then it is very hard to crack all the movement, to check all its valves, its chambers and HO Just oriented. So it gets tricky, and even trickier, if the fetus,

Paulette  27:48  
that sounds tricky, and there are a lot of different places where uncertainty can creep in, because you don't ultimately have control over all these things. I'm wondering, when we're looking at a fetal heart, is it the size of a tennis ball. Is it the size of something smaller? Is it the size of a golf ball? How big is that? So

Dr. Mustafa  28:06  
basically, the machine has two different knobs that help us. One is to increase or decrease the depth at which we are scanning. So the machine has a capability to make it closer and also to zoom it at a specific point. We can zoom the heart, it can appear like a golf ball, and it can appear like a tennis ball, depending on the machine the capability and how much it is able to zoom properly and how much clearer image we can get. So we can make it small and large depending on the capability of the machine. So we can, we utilize it as large as it can be, but at the cost of its resolution and the clarity of the image we are getting? Okay?

Paulette  28:55  
So it sounds like the size of the organ that you are looking for is not really what's at issue. It's the four things you talked about. It's the machine, the technician, the mother's body, and the willing to cooperate or not.

Unknown Speaker  29:08  
That's definitely,

Paulette  29:09  
I'd add to what Dr Mustafa said here, when he says the technician is one of the variables. There's a lot packed into that. What that includes is both training for sonographers, which isn't always standardized. Communication between sonographers and interpreting physicians, as well as variations in screening protocols across different regions. And not surprisingly, Experience matters. More experienced sonographers tend to have higher detection rates. I'm going to stop my conversations with both Amy and Dr Mustafa here for today, I appreciate Amy sharing what she's learned about the importance of early congenital identification with us today, and I appreciate Dr Mustafa's grounding and some of the issues that drive the ease or difficulty getting the best information in utero. I also asked him about the timing of heart defects, and he suggested that most organs are pretty well formed between 18 and 22 weeks. Which is why, that sounds schedules of afternoon. So, if there is an issue, and it's going to be caught. It's the best chance of finding it. Dr. Mustafa also suggested.

Transcribed by https://otter.ai