Pectus - Physiologic Consequences and Research

Um, I think, uh, well, we have Dawn here now, and so, hey, Dawn. Good morning. How are you? Good, good to have you. Um, uh, thank you. Uh, we were gonna, uh, since we have you, we have Doctor Taylor, we thought maybe you might wanna talk about, um, some of your, you know, uh, VO2 testing and things like that as far as like the physiology, physiologic, uh, you know, consequences of Pus, and, uh, I'll go ahead and. I don't know if you can move your slides or if I need to move them for you. So before, before Dawn gets started, let me just, uh, embarrass her, uh, by saying that, um, uh, Becky and I and our team went to visit, uh, Dawn at the Mayo Clinic, and, uh, we came away, uh, impressed with the observation that she's one of the most talented, if not the most talented, uh, uh, adult thoracic surgeons that I've seen, uh, in my. In my short lifetime and uh we came away with a lot of learnings and changed our approach. uh so when we thought about having this uh global cast, uh the first person that came to mind, uh, that needed to be included outside of our Cincinnati family was Don and Don, I wanna thank you for being up so early in the morning, uh, to join us. It's all yours. All right. I think that you guys have to advance the slides cause um from there. OK, just tell me when to advance and I'll advance for you. OK, good. So, you know, for me, what I see are mostly older patients that come into me and, and say, uh, you know, my whole life I was fine, and now in the last decade or so, I can't exercise, I can't breathe, it's getting worse every year. And, um, you know, they have this series of symptoms which, um, In the older population can be a lot of things more so than the children. And many of them have seen a cardiologist and had a workup, but a lot of them don't. And so we started getting a very, you know, detailed dedicated uh echoes and um stress workups. And we're not so much looking for ischemia, we're looking at what is their pattern of um What is happening to them when they're saying, I, I, I've lost my exercise endurance. So, The best test so far that we found has been this cardiopulmonary exercise test, and it's been very critical in our ability to get insurance over the last years, and I think in the overall practice of Pus is documenting what happens to these patients as they get older and they lose their ability to exercise. So, I'm, I'm just gonna go through a few slides. Um, these kind of, if you can, um, go to the next one, the echo. The question is, you know, um, what is normal in this patient? And I can tell you that 99% of the patients that I get have a transthoracic echo that says normal, no issues. And what we started, what we've realized is it's very difficult to get windows to see properly the right side of the heart, um, transthoracic. So we started doing intraoperative, um, looking at all these patients with the TEE. And so in our next slide, you can see, um, this is an example of the pre-op, looking at the, the white band is your chest wall. Causing compression on the right side of the heart. And then this is post-op and you can see a marked difference in the right side, the, the dimensions of the chambers, uh, and actually the output as I'll show you. So on the next one slide is you're looking at velocities. So you can see almost a triple in the amount of velocity or volume that's going through your right track. And then people will argue, so how does this transmit to the left side? The left is not usually compressed, it's the right. But as you know, the heart is a pump, so when you look at your right, now switch to the next side should be the left. Your left side also has a significant improvement when you increase the flow to the right. And so, this is part of the kind of Physiologic aspects of why these patients are, are, are noticing less endurance. So moving into our next slide, we, we started looking at a little more detailed things. We looked, started looking at, you know, three dimensional aspects of the heart and in our older population. You can improve their cardiac output from like 50, you know, 55%, which is quote unquote normal to 77%, and they notice this, their ability to exercise, their, their, their fatigue level with exercise is hugely improved. On the next slide, we looked at a couple of other interesting aspects which um uh um get, get very complicated, so I'm gonna give you my simplified um thoracic understanding of it. But when you look at contractility of the heart, the synchrony, the amount of excursions that um and, and mobility of the heart, there's a big difference between pre and post-op in these patients. And then on the next slide, you can see um your segmental excursion. And it's basically the efficiency of the pump improves. And I think part of this is just because of the distortion of the heart over into that left chest, you know, the, the actual deflection of that right-sided chamber. And then on the final slide here, you know, we looked at um about 168 of our patients pre and post-op, and it's interesting, you know, the, the open group doesn't improve as much as the um minimally invasive, and I think that's because the open is more of a mixed batch of, you know, malunion and other issues. But in, uh, in the, the MIRP group, we actually had a 30% increase in their overall output, cardiac output. Um, and, and in all patients together, it was 24%. But I think it's significant and um we've been working really hard to try to put these numbers out in the literature to show that this, this is a, a medical disease. It's not cosmetic and it does affect these patients. And I think as they get older, their ability to tolerate this compression gets less and less, that's why they become more symptomatic. So for this population, on the next slide, you know, we do a full cardiopulmonary stress testing. And it's, it's not the standard ischemic stress testing on the treadmill and um it, we, we ordered it, we ordered it on all the patients and many patients come in with an outside workup and they almost always have the standard Bruce protocol ischemic testing, which is not what, what you need, um, you know, unless you think a patient has ischemia. Um. The stress testing looks at the VO2, the delivery, you know, the efficiency of the pump, and it separates it out from the pulmonary component. And so it's a really nice, uh, test for PCus. Um, VO2 max, um, looking at anaerobic VO2 are, are excellent, and, and they're very abnormal in PCus patients and all the other parameters are normal. So when you look at before and afters, and, and we're working at acc accumulating our after data, it's a little bit hard in the states because insurance doesn't want to pay for it afterwards. Um, but the, the French did a really nice study looking at 125 adults and it statistically improves and normalizes after surgical repair. And the next slide is just an example of testing before and after. So what you see in Pus patients is, you know, their ability to increase stroke volume or their output. is limited. It increases initially by heart rate, but then when they need to increase volume, they're, they're restricted by their chest cage. And so you get like this flat line where everybody else has a slope that goes up and it peaks to a max. And you can see before and after in this patient, um, you know, very flat inability to um increase output, which improves stat statistically, uh, significant after repair. So that's kind of, um, that's my main workup. Everything else I do is pretty much the same as far as looking at, you know, imaging and EKGs and looking for obviously, usually in adults if they've got Erlos-Danlos or Marfan's, it's already been. been uh recognized and diagnosed. Thank you, thank you, Dan. Doctor Taylor, Michael, would you care to comment? No, I mean, I think, you know, we use exercise testing uh like that for lots of different congenital heart disease. It seems like we don't, we haven't done it as much and I think for patients that we see from you, partly because the decisions are being made on other bases and that it hasn't been required. I mean, I think, you know, we obviously do it 5 or 6 times a day looking at various patients we have. Um, when we're trying to make a decision about whether to, what palliation somebody's gonna have or whether to replace, uh, you know, X or Y conduit or that kind of thing. But, uh, I will say we don't, we haven't done it a lot in the, just for pectus. So, so, Michael, um, given the fact that we are seeing more older patients, uh, The data that Dawn has shared with us, would that should that influence our, yeah, I mean, I think especially for the cases where you think. There's a borderline question like we were talking about earlier. Uh, I, you know, I think, uh, we, we are, like I said, we are very quick to do this for just about everything that we see in our clinic, congenital heart disease-wise, cause that's what you really wanna know is what's it doing to their functional capacity. So sure, I, I think it, you know, it would be, it's a good tool. Sure, yeah, so, um, there's questions about, uh, Just from all sorts of genetic questions and also the exercise questions, so. Let me just fly through them. Would you operate on an adolescent that has Ellers Dan Lowes type 4? And if yes, what would be your pre-op evaluation plan? So, I guess what would your recommendation be and then what would you do as surgeons with the Ellers-Danlos 4? Well, with the Ehlers down 4. I guess the first question would be, you know, I don't tend to see a lot of patients who have pectus excavatum with that, so, um, that gets a little bit tricky. I mean, that's, it doesn't tend to be so much of the skeletal disorder and the hypermobile disorder that we typically think about, um. If they had that, then um I think I, I probably would think very long and hard about before proceeding with that because what we know is that there's internal uh tissue fragility and so the repairs um would really have to be um much more specially managed. Uh, we would really want to confirm that with genetic testing, um, and if we truly believe that even if the genetic testing was normal, then we actually may even want to go to skin biopsy, but I, uh, usually we don't have to go that far. Um, I think this is probably relevant for this, uh, because a lot of our patients out there actually read a lot about Ehlers-Danlos on the internet and then they get very confused about it. So I often hear this question of, oh well, I've got, or the statement, I have. The hypermobile type and the vascular type. Well, why do you have the vascular type? Well, because you can see my veins or um things like this. So, or so and so had uh my grandfather had an abdominal aortic aneurysm, so I must have the vascular type. And those are both findings that really do not indicate that disorder, but patients will come in saying that they have this disorder. So it actually gets very confusing if the surgeon were to take that at face value. So we actually have to make sure that we are dealing with that disorder directly in the first place. Mhm. They, they said they saw one guy today and sent him for genetic testing. Um, that would be the right thing to do. Is there, is there a genetic disorder where you, where you wouldn't, where do you think would be a contraindication for surgery? No, I don't think so, uh, um, from at least the patients that I have seen, um, I think the only thing that you would really have to take in consideration would be whether there's really relative contra indications, meaning that this person really seems to be at a different functional status than others. Um, Dan, can you hear me? I have a question for you. So, uh, from the audience, it says, uh, given your excellent observations, could you comment on the case for pectus repair being best in the greater than 10 year old slash teenager as opposed to a young child? Um, I, I think that probably, you know, you have to weigh cost-benefit ratio, and I think doing very young children, um, costs, you know, costs something to them and, you know, over outgrowing their bars, um, potentially is, you know, is there any risk of growth, disturbance of the chest wall versus the, the teenager that is, um, you know, nearly completely grown, uh, you know, as an adolescent. Um, there, you know, It, it's always, you know, if you, you get a seven year old that has severe defect with, that's extremely symptomatic, then yes, maybe that child needs to be repaired earlier, but I think you have to look at the cost-benefit ratio. Um, it's interesting when I, when I was at um UCLA Malik, who is, you know, a big, uh, cardiopulmonary exercise person. He had an Easter sales grant to look at all the kids there um using the cardio exercise testing, and his results were not as strong, that, you know, there were, there were classes of patients that showed deficits, but a large percentage of children didn't show problems. It wasn't, it's not until they get older that I think their compensation mechanisms don't work quite so well and, and they start showing up, uh, you know, stronger on testing. And I, I think that, you know, that's clearly what we see with the disease. We see people, kids that are completely asymptomatic that that progress and become symptomatic as they get older and then there's some that are symptomatic very young. So I think you have to balance that. but it sorry, I'm loving this because when there's, when there's absolute uniform agreement, it's very boring. This is exactly what we like because I can tell you no one is agreeing on the age here. So, and I, I'm gonna, I'm gonna introduce yet another variable, and, and that is something that I think scares the living hell out of me, and Central is gonna help us, uh, sort of, uh, perhaps better understand that, and that is, um, we are dealing here in the state of Ohio and in this Cincinnati area with a crisis as far as prescription drug abuse and addiction and heroin. And um my concern is is that are we, because we don't know the answer to this when we wait that we know that the operation is better tolerated when you're younger, those older patients need a lot of narcotics. You're gonna hear about the early exposure to narcotics as potentially being a risk. For addiction. Are we then sort of by delaying it, making the treatment worse than the disease? So this is so, I have to tell you, I got, there's, I'm, I'm gonna get MRIs, I'm gonna start getting consults for genetics, and now I'm totally, I would have told you before today, are you crazy? Is this 567 year old? No way. I would not ever do it before 12 or 13. So I am being convinced and Dawn, I, I, it's interesting if you read back up in these texts. The variation of what people do. Some people adamantly say, uh, you know, no, no younger than 14, and some people adamantly say, no, the younger, in fact, Mark Saxon just made your point, that the younger kids tolerate the pain so much better. Um, and so, it's, is that, there's definitely data to or is that not even been studied? Is that anecdotal? Well, we're going to wait to hear. OK, OK, OK. I don't want to I mean I think if you look at chest wall flexibility, I mean you can repair a young kid with a popsicle stick. I mean they're pliable. So I mean, I think, I mean if you look at adults, they have tremendous pain because they're so rigid. So flexibility is certainly on your side at younger kids and if you look at sites like Korea, you know, they do 345 year olds, their, their bar set, you know, looks like a little tiny Tinker toy. Um, You know, it's what you're if you look at trends in the United States, you know, they used to repair everybody young and it's moved to older populations, for a lot of the reasons that I discussed. So I mean, I think if you look overall as a trend in the US, people are repairing. These patients older, but you know, no one knows what the right answer is. So Don, let me, so let me ask the question because in looking at Donald Nuss's series, right, when he first started doing the pectus repair, it was in very, very young patients, even as young as 3 years of age. Um, and so you look at trends, right, uh, uh, and is that really based on data, or is it somebody who had a couple of patients who outgrew their bars because the bars were too long and then they had to explant them and then put them back in, um, and but yet when you look at Donald's data, he, he didn't have very, very high recurrence rates. I don't recall at least in reading that those those series. That he had to explant them when he was doing them much, much younger. So why, why is it all of a sudden that we've now changed it to say, oh, we've got to do them much younger, much older, you know, older than 90, you've got to wait until they've grown, fully, you know, gone through puberty. Yeah, I don't know. I never have asked him specifically, but as an institution, you know, with thousands of patients there, they've definitely. Moved to the statement that they don't do them young. So I think they must have a lot more issues than what they reported in that initial series. Maybe that came with long term follow up. I don't know. But I know Korea, you know, if you talk to Yung Park, they repair them young all the time and say they don't have problems, so they think we're crazy to wait. Um, I was, I was in Peru, uh, last week, and they had this big experience with using the vacuum bell, um, and the question here from the audience is about that same thing, um, that for the smaller children, the vacuum bell works well. Uh, any quest, any comments about that? Um, I can only speak of one patient that I had, uh, who was using the vacuum valve, and, uh, the results were less than, uh, Uh, they were not compelling. They were, they were not compelling, um, and so that's that one patient that I have with a vacuum belt. Uh, so Dan, what, what's your experience, Becky? Do you have any experience with vacuum belt, oh sorry, I think the vacuum belt, um. Works if you have a very flexible chest and you wear it religiously. And there, there's a huge variety in um patient's ability to tolerate and use it. Um, when I see very young kids, I have their parents get a vacuum belt and put it on, and I think it definitely helps. Um, most of them will, will say, well, you know, he, he won't, he'll only wear it for 10 minutes, we have to bribe him with candy, or, you know, and, and so when you start getting into user ability to vary, a product, it gets hard to assess what is true, um. True value is, but I do think it helps and, and for me, when I see a 5 year old, I, I'll have him do a vacuum be versus taking him to the OR. I'm not, I'm not gonna operate on that small of a child. Um, but, you know, that, that's, that's our center and other centers don't feel that way, so, uh, you know. I think it's a reasonable response. Don, I, I have a question for you. Um, if, if you had like an, an asymptomatic, like 16 or 17 year old, uh, with a, a deep pectus, say a Heller index of, you know, 7 or 8 or something like that. And how would you counsel them, and they're saying, well, I'm fine. I, I don't need anything. I'm asymptomatic. How do you counsel them knowing what you know about, you know, the adults that you followed and the physiologic impacts of, of, of having a pectus, uh, of what's gonna happen with them in the future if they don't get it repaired? Sure, so, so I, I do exactly that. I, you know, you, I go through the data that, that we have. Um, we don't have randomized controlled tiles separating patients of who got fixed and who didn't. So, uh, you know, I always make the statement with everything that I say that I have a very biased opinion because most of what I see is symptomatic patients, and I'm sure there's, you know, thousands and thousands of adults out there with practice that may not be symptomatic. Um, so, so I have selection bias in what I see and I go through the data and I, and what I tell them and their parents is that here's your testing. You, you perform normal on the exercise test. Your echo is not showing, you know, any issues. You're not symptomatic, you know, there's nothing that medically says you have to be operated on right now. There's a good chance that you're going to become symptomatic. And here is my experience with older patients. And I let them make that choice. But I can tell you that 95% of the people that come into my office are, are coming cause they want surgery. So, when all those things are negative, it's, it's amazing how, like if you talk to them, all of a sudden they'll be like, well, you know, I'm actually not as good as my friends, you know, and so all of a sudden, they, they, whether, whether you implant those ideas that they're having symptoms or they, they truly have them and don't realize it until you talk to them in depth, uh, that's hard to assess. But, you know, if they have a normal cardiopulmonary stress test, it's still a bell curve and, and what's normal, you know, they, low normal may not be normal for them.