From Palo Nidal to CDH and ECM, um, so, uh, we've got, it's not quite international, but Seattle, uh, it's like, uh, um. Uh, we have, uh, Rebecca Stark and Steven Lee coming from Seattle Children's, and it's about CDH but also the role of ECMO and timing of ECMO. So, uh, we'll see what we'll see what we can learn today. Thanks, guys. Great. The, uh, travel here seemed like it was an international trip, so we, uh, we missed last night's event. So, um, don't you love pediatric surgery when we went from cholidocholithiasis to Wilms to pyloidol now to CDH. So just a very, just some variations on the spectrum of diseases. So, um, I'm gonna hand it over to Doctor Stark to get started. Hi, um, I'm so excited to be here, although a bit nervous, um, this is my favorite thing to talk about and this is going to be very controversial because the disease itself is controversial so just that's my disclaimer that's my disclaimer, um, we'll, we'll see how people feel about these questions and my answers, um, OK, next slide please. These are the two basic things that we're going to be talking about, um, when and how to do ECMO to treat CDH and also when to repair and how to repair. And I'll talk about anticoagulation at the same time. Next slide, please. So this is our first clinical scenario. I purposely made it a little controversial. Um, so full-term neonate, known diagnosis of a left-sided, very severe CDH. You can see the numbers up there. Um, less than 25% for each of those are severe. So this is a very severe CDH. No known cardiac anomalies, no genetic anomalies delivered about an hour ago. Let's say it's an inborn situation, intubated and resuscitated per your institutional protocol. So we're not going to go into vent settings, nothing like that. Just imagine you trust your protocol. These are the first vitals. So, preductal sats are in the 70s, heart rate is 150, blood pressure is 35/25 on 100% FIO2. This is your first gas. So PH is 6.8, PACO2 is 130, and PAO2 is 28. So then I'm going to ask, what is your next move for this patient? Um, and we can talk about each of these answers, and I wonder if some audience members would like to say what they would do at their institution. All right, so while we're waiting for the polls, I know people are strongly opinionated about this. Yes, forgive me in advance to comment on their preference, thoughts on this? Have they changed their practice, Mac. Why me? If you're Uh OK, don't pick D. Don't pick D. So, so I have to read the thing again because I wasn't really OK, I don't have to put you on the, OK, anyone else want to answer. Jose, what would, what would you do in, in Chile? So in Chile there's one big neonatal ECMO center that takes care of all of these babies. They usually put babies in ECMO with an oxygenation index over 40 and after, um, reassessment of, so usually they do first, um. VAFO and nitric oxide before putting someone in ECMO and also you usually want to see uh uh oxygen saturation above 80 at some point before putting someone on ECMO so I think. Well, I'll take the top policy approach. Maybe this will be wrong, but I'll take some time and reassess. Yeah, Katie, what would you do? Utah. I mean, I think that that kid is, uh. From the prenatal, sorry, from the prenatal parameters that the child is definitely at risk for ECMO, um, I think our rates of ECMO have gone down dramatically over the years and we're at less than 10% overall. But I think that is likely a kid that would go on ECMO and our strategy is to go on VV first, um, with the ability to convert to VA if we decide that's necessary. And I would be the old school person that still does VA because I'm not bold enough to go VV. It's not that old school actually. You, you will see, but, um, it's, it is, yeah, I mean the vast majority of centers that report to the CDH study group, so like over 90 centers, thousands of patients do VA ECMO, um, but can I have the next slide? Quickly, just to talk about A and B, um, they, they're basically equivocal in terms of outcomes. So there's not a survival difference proponents of VV, uh, there are lots of benefits to VV. There are lots of benefits to VA. The majority of centers, as I said, still do VA ECMO primarily for CDH babies. Um, and there is a conversion rate. So it's, uh, both answers are totally reasonable. I think the baby needs an intervention. I think you know from the prenatal imaging that the baby is high risk, needs for, is at a high risk for needing ECMO, and I think the time to intervene is probably earlier rather than later. Do you feel that VV cannula availability and also concerns with certain VV cannulas, um, has played a role in that, and now that there are different cannulas available for VV do you think those numbers will change? I think it's possible. I think the other thing that people really think about with VV is size of the venous cannula, um, and some of these babies we know have smaller vessels than than their normal cohort. And so I think that is part of the question. I, I do think that if you, if you were at an institution that did VV and you couldn't cannulate because the 13 French is just too big, you'd probably do VA. And I do think that there are better cannulas coming out and that this is something that the needle might move on. Things move very fast, as I'll show you in, in this world of CDH where, you know, one day you have everyone doing VA and you can take a poll two years later and everyone's converted to VV. So it's definitely a moving target, um, in terms of C, I, I definitely don't think that that is a. Totally incorrect answer. Um, you know, there are lots of protocols that I've seen that take time to reassess and ECMO cannulation within the first hour is, is a really hard thing for people to decide to do. I will tell you that there's data from the study group, so big numbers that show that high performance centers cannulate earlier on lower event settings than, um, than other places, so they're getting higher survival, and that's the main thing that's, um, unique to, to all of those, um. Protocols. So I think this baby doesn't need more time. You could reassess if there was a, a big thing you could change. So you look at the chest X-ray and the ET tube is almost out or it's really mainstemmed or there's something that you know could make a big difference. Please intervene, reassess, um, but if event change isn't gonna help this, this baby's kind of too far and then in terms of comfort care, there, there is a lot of data, um, especially within the last 5 years that really supports, um. Trying to get survivors out of all of these babies, mostly because we don't have a great way of deciding which babies will survive and which babies don't survive and if we had a really good way of figuring that out, then it would make sense to not offer ECMO to some patient populations or if you're in a place where you can't offer ECMO readily, you have to transfer babies many hours or those sorts of things, then some of these, um, if you can go to the next slide, some of these, um, ideas about. Screening before you put babies on ECMO makes sense to me, and it all comes from a really good place, you know, it, it is a struggle to have babies die on ECMO. Um, it's hard for the families, it's hard for the care providers, but I do think that, um, and next slide, I won't go through all of these, sorry, um, this paper is, is really good and it really drives home this point. So, you know, at Michigan, um, they had a sphere protocol that protocol helped them decide which babies to offer comfort care to and which babies to, um. For ECMO too, they were prenatally selected so you can see the parameters here. If they had a severe CDH, so all of the normal qualifications for severe CDH with liver up, the parents were educated that they would go on this pathway at the delivery in the delivery room, they had ECMO on standby. They would intubate, they would do ventilation. Also, needle has moved on this, and it is not considered gentle ventilation anymore and be resuscitated within 2 hours if the PH did not meet 7, PCO2 wasn't less than 100. Preductal sets weren't above 80, they were offered comfort measures only. And if they could achieve some of those things, they were offered ECMO. So to analyze this, um, uh, Erin Perroni actually looked at the study group database, got lots of numbers, was able to get two groups of patients that exactly matched these two clinical scenarios. And so the, the failed group is the group that was offered comfort measures, so didn't meet any of those criteria. And The success group was offered ECMO. She compared these two groups of patients. They had equivalent survival amongst those two groups. It's that means that half of the patients that were being offered comfort measures could have been survivors. It also means that half of the patients that were offered ECMO didn't survive. So we don't, we are really bad at choosing. And if you make this decision to choose beforehand, you're going to be wrong 50% of the time, and I really strongly believe that we've all had clinical scenarios where. You don't expect a baby with CDH to live and they live and we've had scenarios where you expect a CDH to die or sorry to um opposite survive exactly um and so I, I feel very passionate about this. I know it's um controversial but I I do think that if you had. Good prenatal counseling with the parents and they're all in for this, um, you should pursue ECMO for for every unilateral isolated CDH. So I'm, I'm not talking about genetic syndromes, cardiac syndromes, those are, that's a whole different ballgame. Um, this is, uh, this is an isolated unilateral CDH. So now you have, um, another clinical scenario. Thank you for moving me along. Um, this is the same baby, and, um, you've placed the baby on VA ECMO, um, and what anticoagulant are you going to be using? What does your institution use? Um, what do you think? You can move the slide forward to show the answers. Yes, I put other, but I don't even know what other is. I just wanted 1/4 answer. We use bivalve. Yes, oh, great. Yeah, we, we use bivalve as well. Do you like it? Yeah, we love it. I think one thing you can get out of the, uh, get out of the mind is you never have to worry about hit. You never have to worry about following 83. It's really a struggle always getting heparin into a, uh, even if you're in a subtherapeutic or therapeutic range. Bivalve is pretty much a no-brainer, and we use predominantly as the sole anticoagulant, uh, drip for most of my gosh, I love that you're saying that. Is this a black diamond or is this like a diamond. No, I think, I think most places still use heparin, but I think this is one of those times when the needle is like really moving quickly because a lot of places are switching to bivalerudin as an, yes. Is this a black diamond or like is that the correct? I think you cannot say that because it's, it's some institutions will not offer bivalve and that's something you can't change in your own institution so you're gonna use heparin, but I, I will say there are a lot of benefits to bivallirubinin, you know, that it's a little bit more expensive, but it, the benefits actually outweigh that cost because you give less blood transfusions. Um, it, everything is better about it, and I think people, once you try it, um, will, will switch pretty quickly. Um, but heparin is still the most commonly used, although a lot of places have switched. OK, so most of the world, but that's, that's way more than 2 years ago when we did the poll. Yeah, but that was after Bargava said, OK, so. You know, I forgot to look at the audience poll for the first question. OK. I mean, in the room here, I mean, who, Greg, what do we do? Oh yeah, we do buy that. Switched over to bivalve. you Yes, that's exactly what happened, and they were using it for EVADs at our institution, so it was really easy to switch over. Next slide. This is the protocol that um kind of came out really early on um and people started adopting pretty quickly um next slide. In in that prior paper, you know, they use PTT to monitor bivalve level, and, um, and this is a paper that came out of our institution, you know, like I said, we were using it historically for LVADs and so they had to figure out a way to really track bivalve levels. You can do it with a dilute thrombin time and any lab can create this. It's 5 times more um specific and sensitive um as PTT and so it's something. That we can share and um it's a great way to monitor the bivalve levels as you go through. Um, I will just say two more things or maybe 3 more things about bivalve and it's a very short half-life, so it's really easy to turn on and off. You see immediate effects. It's like a 20 minute half-life, um, and it, um, is something that is, there is much less variability between patients in terms of how effective it is at anticoagulation. Um, and, uh, let's move on, because I have a lot more slides. Um, this is just our protocol. You can go through it. Um, OK, so now the same baby, um, severe CDH on ECMO, you're using bivalve, um, when are you going to repair? So can you move the slide forward to the answers? OK. So what, what are people doing in this room? Ever. OK Wait, did Sean, did you say late, Sean? 08, OK. Anyone doing, uh, Later repair. COD A or B, so no one else is. Repairing late or if they can't come off ECMO, not repairing. So maybe this could be like you gotta do it because people are still doing deeds. Yeah, oh look at our, let's look at the poll. Yeah, I gosh, I keep forgetting these polls. So it's controversial yes. It is controversial. You know, I think, I mean, I, I already said like we've really decreased our ECMO rates like dramatically, um, but I definitely think there are very vocal voices at my institution that would wait weeks before repairing. I would say repair the 48 hour window is nice because the ECMO circuit gets broken in. You get time to get broken in. You give time to give product, give time to all those fluid shifts to kind of start to declare themselves. You don't wait so long to the baby, it looks like the Michelin baby. Um, and it just, I, I think that's the time to do it. You've already got a broken-in circuit. Hopefully not a lot of clot burden. You can turn off your bivalve, you know, start, yeah, start your Amicar. Don't give Amicar. Don't turn off the bivalve. Just turn it down. But whatever you do, you got like a broken-in circuit and you got a baby that's not too, and you just start to fix things and start to move things forward. Wait, so I remember this discussion about 10 or 15 years ago at our institution, and uh I remember somebody brought up a really good point, uh, at least at our institution, uh, the neonatologists were actually running the ECMO, so if we repaired them early then we didn't have to get calls from neonatologists telling us about all these problems with. With ECMO or the hernia, we're going to fix the hernia. So it actually streamlined our approach. We would fix them, you know, early and then the, then the neonatologist could manage the rest of them. I wanna, I wanna just bring up one thing. Um, I think in this room we have experts from major institutions and so for so forth and going back to what your institution offers and, and there are many institutions that are lower volume where neonatologists are very involved with, with the care and, and switching to an early repair if you're only putting one or two children on ECMO per year for CDH is a is a challenge and you need a, a whole buy-in. So the question is how do you. How do you get this by and what kind of protocols and, and how do you ease this transition? Well, I think you show a lot of data. Luckily, we were already using Balve for our LVAD, so that was a kind of easy transition. And then you really build a team. So we have anesthesiologists who do our repairs on ECMO, who they're two of them, they do almost all of the repairs on ECMO. You get buy-in from the neonatologist who's running the pump to be with you in the operating room so they can manage those levels at the same time, um, and you get. People to agree to operate on weekends sometimes because, um, I, what I would just say is if you use bivalve, you, you don't have to wait for the circuit to kind of equilibrate. It, it's very fast. You can operate after 8 hours of putting them on bivalve as long as your levels are stable, which happens really soon. And it's, it's a much easier surgical repair. There's very little edema, no edema. It's, it's very similar to, um. To doing, um, a repair off ECMO. So the, I have 2 minutes left, so I just want to say the one thing I would not say is, uh, you, you, all these babies should have an opportunity to be repaired. You know that if you don't repair them, even if you're waiting till the end of the run, that they're not going to survive. So if you were trying to get as many survivors as possible, you should offer repair. The other thing I will just mention is that there is, there are groups out there that actually risk stratify once they go on to ECMO. So if the baby's high risk, they operate early. If they're lower risk, they try to come off and then repair. I think that's totally logical. The problem is you can't be 100% certain which babies will end up with a late repair, um, because they can't come off ECMO, and that's really the worst time to operate. Um, this is one of the papers that really popularized it. OK, next slide. Um, these are all the advantages, and I would love to tell you about all of them, but, um, you know, you get good lung growth. I think the cannula position thing is, is really important. Um, it's something that, you know, if you're 3 weeks on ECMO and that cannula is getting adjusted and moved and you're getting fluid for it, it can be really challenging. It's much easier if it's in the right position because the heart's in the right position. Left sided heart disease is a big contributor to overall cardiac function. We know that now. You can't predict. which babies can come off without repair reliably, and um you are in a much better position to come off ECMO if you need to urgently, like you have a head bleed, you have bacteremia, you can't clear. The baby's already repaired and you're 3 weeks in, you have a much better chance of survival coming off rather than coming off with a sick baby who still needs repair. And then it's very rare, but you still have this risk of ulvulus and bowel ischemia the entire time you're on ECMO if all that bowels in the chest. Um, Next slide. Next slide, um. OK, repair can be done safely. These are my take home points. Early repair is technically easiest. Um, and I think between 8 to 24 hours is really the right time. We use bivalve Rudin, we don't use Amicar, we turn down the rate, we can share our protocols, um, but I think that's what most centers are doing who use bivalve Rudin. And I think that it is helpful to have a smaller team doing the surgeries. It's very nice if they can be under, you know, 2 hours, um, And you pay meticulous attention to um to the surgical operation. I do want to say there's a slide that got slipped that um might be interesting to people because I, you know, reached out to Matt Harding because I wanted to know in, in out of the 90 centers that contribute to the CDH study group, um, which which patients are getting early repair in this group and, and now almost 80% of centers that contribute are doing early repair on ACMO and, and if you could see this graph that I put in there, but I think it got skipped, um. It's, it's pretty impressive, you know, the data has shifted just over the last 3 years, um, and, um, and so it's, it's a, it's an impressive shift, and I think that in the CDH world, you get shifts like that really quickly because if you're part of some of a, a big group of contributing centers, when some, someone's an early adopter and it works, you're able to pick up on that really quickly. And try to move the needle. Um, yeah, and so I think I will end there. OK, so great, yeah, yeah. So just really quickly, your last two end points, your two teaching points were what? Oh, I think that if you have a very severe isolated unilateral CDH, you should consider them a survivor until they don't survive and also that early repair on ECMO is safe and physiologically benefits the patient. Right, this is phenomenal. Do you have like guideline protocols that you guys use at your hospital? Um, love, if you we'd love to share share shareable, that would be phenomenal. Um, any final comments or questions before we end this session? This was, as we knew, pretty, pretty provocative. All right. Oh, we do, OK. Sorry, hi, uh, thank you very much. Um, I would like to ask you, do you have a minimum weight or minimum age for ECMO connection? We use, uh, less than 32 weeks, less than 1.8 kg. Um, those are relative contraindications. So if there were like particular reasons for or against, you know, we don't say absolute, but those are our cutoffs. They're probably pretty similar to what other people are using at their institution. Minimal invasive sur uh CDH repair. I find it really beautiful surgery and skilled hands, but have not seen a report giving equivalent results. What's your opinion on that? And are you doing it personally or as a group equivalent results in terms of recurrence? Oh, incurrence. It's, um, you know, there's so that's like such a big bag of, um, because, yeah, the needle is also moving on how to do the repair, you know, some places use patches, pages use muscle flaps, um, and so I think recurrence rates are different among those two things, and, um, yeah. It's a great question. I would love to talk to you about it. Yeah, thank you. We'll be back. That was phenomenal. Thank you so much. Thank you.
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