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Nontraditional Pacing in Congenital Heart Disease ...
Nontraditional Pacing in Congenital Heart Disease (Presenter: Jeremy P Moore, MD, MS, FHRS, CCDS, CEPS-P)
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I'd like to introduce the first talker. I'm actually Thomas Greenwood, the current chair of the Working Group for Pediatrics for Dysrhythmia and Lectrophysiology of the APC. And the first talker will be Jeremy Moore with nontraditional pacing in congenital heart disease. Jeremy Moore Okay. I'd like to thank the moderators. So I'm going to get started here talking about nontraditional pacing in congenital heart disease. I have no relevant disclosures. I'm just going to go quickly here because I've got a lot of slides and I've got 10 minutes. So first of all, I'm going to define traditional pacing. Traditional pacing in congenital heart disease, basically we're talking about either surgical leads where we have epicardial approach or even sometimes a transmural approach to get the leads into the atrium of the ventricle, or transvenous approaches where we have direct access to an atrium and a ventricle regardless of what morphology it is. That's traditional pacing in terms of congenital heart disease. And also when we're doing CRT, putting in a CS lead, if the CS is in a roughly normal location, I would consider that a traditional approach. Anytime we have a hybrid system with either surgical and transvenous put together, that's also, I would say, traditional, at least for congenital heart disease. But everything else is pretty much on the table in terms of nontraditional, so there's a lot of different stuff here. These are the major categories that I could come up with in talking about this topic where you might need a nontraditional approach to get pacing. So we have single ventricle anatomy, systemic RVs for CRT, tricuspid valve surgery, intracardiac shunts, and atrial scar. And I'll go over pretty much all of these. So single ventricle anatomy, one of the first palliations is the glen shunt. Obviously, it's going to be difficult to go into the atrium or the ventricle with the glen shunt, so a variety of approaches have been described over the years. The approaches from below the diaphragm is one, so you can go transhepatic, you can go IVC, you can even go from the femoral, the iliac veins. The other one that's actually kind of takes advantage of anti-grade pulmonary blood flow is you can go retrograde from the SVC through the glen shunt and then retrograde through the pulmonary valve and get ventricular pacing or even atrial pacing from that approach. And then this last category here, this transthoracic, transatrial, I would consider traditional because it's surgical, basically. So the original transhepatic approach was described in 1996 by Steve Fischberger. Basically, you get hepatic vein access, which many of us do anyway for interventional procedures, EP procedures. You can place a lead and then you can tunnel the device. You can basically make it a subcutaneous device, and this works for patients who have glen physiology. One caveat is, you know, I think that you probably want to do this in patients who are one and a half ventricular repairs and not true mixing lesions because there is a risk for stroke and thromboembolism when you do that. The IVC approach has been described as well. This requires, this is very invasive, requires basically a laparotomy and a direct IVC puncture. So to my knowledge, this has not been done except for in this particular case report, but it is, it's been described as getting a lead into the heart from a glen patient. This is that retrograde approach I mentioned before, basically from the SVC through the glen into the PA, retrograde through the pulmonary valve, and then you can get ventricular pacing that way. So these are all viable approaches. And then finally, the transiliac approach. We actually published our experience on this. I'm not sure this is always the best thing to do, but if the surgeon refuses to operate and you need to get pacing, this is one way to do it in a glen shot. And the main thing is to put the device above the inguinal ligament so that there's no flexure or stress on the leads and no pain for the patient when they're walking around. Just, you know, the downsides of this is there's potential thrombotic complications and extraction would be extremely difficult for this approach. So we haven't had to deal with that, fortunately, but that's certainly a consideration. And then if you have a hemifontan, a lot of times these patients do have little fenestrations in the patch. You can even puncture through the patch, and those are all been described for patients with hemifontans to get transvenous pacing leads in. Moving on to the fontan circulation, there's obviously the different iterations of the fontan. The atrial pulmonary fontan is probably the most straightforward because you actually do have direct access from the SVC into the atrium. And to get ventricular pacing, though, you need to go into the CS basically for most of these. Most of these patients have a CS that drains to the atrial portion of the fontan. So this can be done, and it's actually not that tricky. When it gets trickier is the extracardiac fontans, if you're talking about transvenous pacing. There's a variety of approaches, but again, because the conduit is separate, the systemic venous circulation is separate from the rest of the heart, you have to get creative. And so there's the variety of approaches that have been described includes transpulmonary puncture. Sometimes you might take advantage of a residual venous connection like an LSVC to the heart, take advantage of a fenestration, or do even do a combination of things like a transhepatic approach with fenestration. So the transpulmonary approach was basically borrowed from the interventional cardiology literature. It was realized that you could actually go from the PA into the atrium directly to create a fenestration in these patients without having to puncture through a conduit. And keeping in mind the anatomical relationship here, you have the PA that sits right on top of the left atrium. These are both anterior to the both right and left main stem bronchi. You can actually get, you can actually puncture here and do this in a relatively safe fashion. Our first patient that we performed this for was in 2014. We had a lady with extracardiac fontan who came in with multi-organ failure with tachybrady syndrome and a really difficult to control SVT. Every time it terminated with adenosine, it would be profoundly bradycardic and then it would go back into SVT. So dialysis, multiple inotropes, and multianterrhythmics. And we decided we would see if we could just at least get some bradycardia pacing. So we put a lead in from the LPA and externalized the pacemaker and realized we could get reliable pacing long term from that approach. And in the meantime, she recovered completely and came off all of her drips and dialysis and everything. So we took her back to the lab and we said, why don't we just put in a, the surgeons refused to touch this patient. So we took her back to the lab. We did a puncture from the PA into the atrium and then placed an atrial lead in the base of the left atrial appendage using TE and angiography. This is what it looks like by TE. You have the PA is shown here and then this is the left atrial appendage. And you can see this close approximation between these two structures. And it can actually be done without a huge risk, although there is going to be some risk obviously. We're talking about extravascular space. This is another patient we did a few years later. And I think here we're highlighting that the PA is a little bit further away from the left atrium. This area is typically filled with connective, you know, sort of fibrous tissue. So you can get through this area relatively safely in most cases and get leads in. And this is another patient that was not deemed to be a surgical candidate where we went the transpulmonary approach. So this was published in 2014. This has actually been replicated by other centers with some variations. And, you know, I think it's, it is a viable option for the trans, for the extracardiac fontan. The Vanderbilt group described their experience with this a year or two ago with basically instead of putting the lead all the way into the left atrium, just delivering the screw in lead into the epicardial surface of the left atrium to try to avoid any possible problems with thromboembolism. And I think they've done this in a few patients too. It's not published to my knowledge, but it's another way to get there in terms of atrial pacing. The fenestration approach, you know, you can basically get your lead through an extracardiac fenestration for consistent pacing. It's a little more direct. It can take a little while to find that fenestration sometimes with the lead, but it's certainly doable. And finally, the Bossa group showed that in some cases you actually have atrial tissue in the extra, either in the extracardiac fontan or just below it. And I think this has to do with having a fenestration potentially that's kind of closed off or having tissue pulled up around the atrium from the IVC. And it makes sense to look for this. I think in some cases it may be a straightforward thing to do. If you have an atrial myocardium in the extracardiac or just below the extracardiac fontan, save you a lot of effort trying to puncture across different things to get your lead in. What about your systemic RV? So the literature on this is pretty scant in terms of mustards and settings. There is a case report describing CRT in a mustard patient who had the CS drained to the systemic venous side, and they were able to cannulate the CS and then get biventricular pacing. To my knowledge, this has not been reproduced, but it's something you can consider rather than going epicardial to get CRT for a mustard patient. And then much more commonly, though, we see CRT needed for CCTGA patients with the AV block. We know from pathologic studies that up to 20, 30% of these patients have major abnormalities in the CS drainage. And so it can be challenging in some cases to get leads in patients with CCTGA anatomy. What we found is that the major variation seems to be a vein that drains the RV outflow tract to the area of the base of the atrial appendage. And that's pretty much there in every single case. The size is very highly variable. The other thing we've seen is duplication of the CS in atresia of the typical CS with a vein of Marshall providing access to the system. And so these are just some venograms we've done over the years. The top panel, basically these all show some, these white arrows show the drainage of this RV outflow tract venous system to the base of the right atrial appendage. And it's variable size. And so, for instance, on some of these bottom panels here, we were actually able to get a catheter into this upper vein. And in some cases, even place leads into these upper veins rather than the diminutive posterior vein. This is what it looks like on a reconstructed CT scan. This is a vein that drains very superiorly to the base of the appendage, very anterior. And it actually courses around the right ventricle. So it looks like on a 3D map. And then when you put the lead in, it looks like this. You can actually get reasonably good separation. If you can get this thing posteriorly, you can get pretty good separation in your leads with this, using this anterior vein. The other major anomaly we've seen is the atresia of the coronary sinus os with a vein of Marshall up to the anominate vein. And one quick clue that this is there is when you do your anominate vein injection, you see negative contrast washout where this vein's connecting. And you can, actually, this is much more straightforward. You can cannulate this vein, get a lead in place, and you have nice separation in your leads with this approach. So, you know, there's going to be some variability here. In terms of response to CRT with CCTGA, the best responders are the ones that have chronically LV paste or have prolonged Q restoration to begin with. The ones that don't seem to respond as well are the ones that have AV block with junctional escape. And so, those are the ones that may be best with his pacing. This was described back in 2010. And, you know, you can get a pretty reasonably good response with mapping out the His-Purkinje system, placing a lead in these patients, and a nice, this is a non-selective His capture here with these patients. Am I running out of time? Okay. So, I'll skip through quickly the His capture. Tricuspid valve surgery, just real quickly, you know, there's a variety of approaches you could take. We've used micros in some of these cases when you can't get into the CS when the valve covers the CS ostium. And then, for intracardiac shunts, another option is the micro as well. We've done this in a number of cases now where we've used micro with septal shunts to avoid the complications of thromboembolism. And then, the last situation is when you have a lot of atrial scar. I think using 3D mapping systems can be very helpful. And oftentimes, the atrial septum is spared. So, I'll just wrap it up here and say that congenital heart disease may require non-conventional pacing approaches in a lot of cases. The common scenarios are single ventricle heart disease, coronary vein anomalies, tricuspid valve disease, shunts, and atrial scar. And we do have strategies to circumvent many of these limitations. And there's a lot of new technologies on the horizon including wireless LV endocardial leads and some other ones that are coming. And I think it's just a great, it's a great opportunity, it's a great time to be in interventional sort of EP and device therapies because there's a lot of options now. So, basically, that's all I have. Thanks, Mr. Mu, for your great talk. Are there any questions from the audience? Yes? Hey, Jeremy, real quick. Thank you very much. Actually, it was on the last slide that you kind of blew through nice and quick was on the mapping and then placing a lead at the same time. Is that what you were doing that you had mapped before and then you were using your mapping system to guide actual lead, pacing lead placement there? Yeah. So, we had done, gone from below. You could technically probably do this from above as well. But you can, yeah, we got a voltage map and then we connect the lead to the mapping system and then just use it to guide it into place. Excellent. And what, I mean, are you, that looks like a CARDO system. Is that what that is? This is CARDO. We've done it with CARDO. We did it with Rhythmia. You can use it pretty much any mapping system for this. Great. Excellent. Thank you. So, Jeremy, what's your opinion about anticoagulation with mixing lesions because there's mixed data about how effective that is at actually preventing strokes? Yeah. I mean, I don't think it's preventing strokes completely. I mean, we would do it, but I don't think that provides you absolute reassurance that you're not going to get a stroke. You put, what do you use? You put in Coumadin or? We're using Coumadin, yes. Good for that. Do you know, is there any data exist about this, about the risk of trauma embolism when you do, when you put the lead in the systemic ventricle? The literature on that, it basically comes from the, you know, the Canadian experience with LV, transeptal LV leads, endocardial leads, with CRT. They do have, I think it's like a 10 or 15% rate of stroke when you go into the LV side. They do that, I mean, people do that as just a bailout before transplant, essentially. So, we're talking about, you know, last resort sort of situations, really, for most of these things. Last question. I think often these approaches come up when the surgeons have declined or tried and failed to get leads on. Given what we know about the survival of epicardial leads in the first place, are there situations where you would go to some of these things up front before going through three rounds of epicardial leads and failing through them? I mean, it depends on which population we're talking about. I think for the extracardiac Fontans, if we find that we can get the lead on the epicardial side consistently and not have to worry about the stroke risk, I think we know that Fontan patients, you can anticoagulate and the risk for thrombus formation of the systemic venous side is low. That's been shown. And so, I think if we can do that reliably and safely and show that with larger numbers, I mean, we're talking about, you know, five worldwide cases or something at this point, I think we could, I think that would be a reasonable thing to do. Thank you.
Video Summary
In this talk, Dr. Jeremy Moore discusses nontraditional pacing in congenital heart disease. He explains that traditional pacing techniques involve surgical or transvenous approaches to access the atrium and ventricle. However, in certain cases, nontraditional approaches are needed due to anatomical abnormalities. Moore discusses several nontraditional pacing techniques for different scenarios, such as Glen shunts, Fontan circulation, systemic RVs, tricuspid valve surgery, intracardiac shunts, and atrial scar. He describes various approaches, including transhepatic, IVC, retrograde, transiliac, and fenestration, as well as the use of mapping systems for lead placement. Moore also addresses the issue of anticoagulation in mixing lesions and the risk of thromboembolism in nontraditional pacing. Overall, he emphasizes the importance of individualized approaches and the potential for new technologies in the field of interventional EP and device therapies.
Meta Tag
Lecture ID
3837
Location
Room 211
Presenter
Jeremy P Moore, MD, MS, FHRS, CCDS, CEPS-P
Role
Invited Speaker
Session Date and Time
May 09, 2019 1:30 PM - 3:00 PM
Session Number
S-032
Keywords
nontraditional pacing
congenital heart disease
anatomical abnormalities
Glen shunts
Fontan circulation
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