Cardiac Sympathectomy: Update Course 2017

Thank you everybody for tuning in. So we're gonna talk about cardiac sympathectomy for ventricular arrhythmias. And then. The bottom. Yeah, there you go. Um, so I know a lot of pediatric surgeons do sympathectomies for hyperhydrosis, but how many actually have performed a cardiac sympathectomy for ventricular arrhythmias as we have a poll up. So let's ask here, has anyone here ever performed a sympathectomy? So let's start with this who has done a sympathectomy for hyperhydrosis? OK. And uh Dan, what levels do you usually go to or wet either of you do you go to 2 from 2 to 4? I go 2 to 42 to 4. OK. All right. Uh, anyone do anything different and you do, it's a great case and you can do it bilaterally by putting them prone prone. It's, it's if you do one side, you slide them off to the side of it, or do you just leave them in the middle of the table prone and thoracoscopically prone. We put them into cubitus prone, put them prone. You're looking right at, you put the scope in and you're looking right at it. Wow. All right, I'll try that. Uh, all right, so, Sophia, let's see if, so, so it sounds like no one in this room has ever done it for ventricular arrhythmias and from the virtual audience. 86.7% said no. OK, so, uh, we're actually gonna, we've actually had two experiences here at Akron Children's where we've done cardiac sympathectomies for two different patients that have had ventricular arrhythmias or life threatening ventricular arrhythmia. So we're actually going to start off by showing you a video that gives a little bit of synopsis of when you would perform a cardiac sympathectomy as well as show you the technique that we use for those two patients. This video will discuss the use of left thoracoscopic cardiac sympathectomy for ventricular arrhythmias in two pediatric patients. Left cardiac sympathectomy is an effective treatment for patients with life threatening ventricular arrhythmias are resistant to medical therapy and in patients who've had frequent ICD firings. Unlike the procedure for hyperhidrosis, which involves a ligation of T2 to T4 ganglia, a cardiac sympathectomy also requires partial resection of the steellate ganglion. This is thought to increase the threshold for ventricular fibrillation. Our first case. Of a 14-year-old male with catecholaminergic polymorphic ventricular tachycardia. He was diagnosed after having multiple seizures and syncopal episodes. His symptoms persisted despite beta blockade to an ICD placed that resulted in multiple firings. He was brought back to the operating room for a left thoracoscopic cardiac sympathectomy. He was placed in the right lateral decubitus position. A double lumen endotracheal tube was placed, and defibrillator. pads were also placed in case the patient went into ventricular tachycardia during the dissection. The procedure is performed with 35 millimeter ports. The camera port was placed in the fifth rib space just anterior to the scapula, and two additional working ports are placed in the mid-axillary line in the 4th and 6th rib space. Upon entering the chest, we first identify our landmarks. This image shows approximate locations of ribs 2 and 3. The sympathetic chain and the steellate ganglion. The first rib is often very difficult to identify, so the highest most ribs seen is usually the second rib. We start that procedure by lifting up the pleura just above rib 4 and opening up using hook cautery. After identifying the sympathetic chain, we open the pleura approximately towards rib 2. And then distally towards route 5. Prior to resecting the sympathetic chain, we injected with lidocaine to avoid cardiac dysrhythmias during our resection. We then resect the distal sympathetic chain using electrocautery just past the T-four ganglion. At this point, we free up the rest of the sympathetic chain from its attachments until reaching the steellate ganglion. We again inject lidocaine to the stellate ganglion to avoid cardiac dysrhythmias during our resection. We now perform the partial resection of the stellate ganglion using clips and scissors. It is important to avoid the use of electrocautery on the steellate ganglion, as it may damage the remaining portion of it and result in Honner's syndrome. We then complete the case by evacuating the air using a red rubber catheter and re-inflating the lung. Our second case is of a 13-year-old female with hypertrophic cardiomyopathy and history of multiple cardiac arrests. Like the previous patient, she's had persistent symptoms despite beta blockade and had multiple ICD firings, so she was. Taken back to the operating room for a left thoracoscopic cardiac sympathectomy. Upon entering the chest, we first identify ribs 234, and the approximate location of the sympathetic chain and stellate ganglion. Again, the first rib is often very difficult to identify, so the highest most ribs seen is usually the second rib. Like with the first patient, we start our procedure by entering the pleura using hook cautery just above row 4. We then dissect proximally and distally to identify the sympathetic chain. We inject lidocaine to the sympathetic chain to avoid cardiac dysrhythmias during our resection. We resect the distal sympathetic chain using electrocautery just after the T4 ganglion. We then work proximately to free up the attachments to the sympathetic chain until reaching the steellate ganglion. We complete the case by performing the ganglionectomy using clips and scissors just like in the first case. Both patients had complete resolution of their symptoms without any further ICD firings and no evidence of Horner's syndrome on follow-up. The key points of this video are, first, left thochoscopic cardiac sympathectomy. is an effective treatment in pediatric patients for life threatening cardiac arrhythmias. It involves partial resection of the steellate ganglion and a T2 to T4 ganglionectomy. Second, the steellate ganglion is found by identifying the second rib, which is often the highest most ribs seen and dissecting above it. And third, avoid resecting the stelate ganglion using electric cautery, as it may further damage it and result in Horner's syndrome. It's great. Uh, all right, we can shut off the, uh, yeah, all right, well, so, Sophia, thank you for that presentation. Now John, are you gonna talk now or OK, yes, all right, perfect. So we're gonna pull up, uh, John's talk. So thank you, Todd, for the invitation to come lecture today, and, but more important, I wanna thank Todd for, um, providing my patients and myself, uh, the availability of this new technique, uh, which hopefully I can convey to you can sometimes be life saving. I also wanna thank Sophia for all of the work that she did to put this lecture together and specifically uh with the video uh when I watched the video I, I, I think wow that's a simple procedure and despite its simplicity, uh, it can have dramatic uh impact in the lives of the patients that can use it um. Most people are not familiar with a cardiac sympathectomy and, and figure it must be a brand new procedure, but in fact it's older than most of us in this room. Uh, it was first introduced in the 1940s as a treatment for angina. Uh, and it was very effective for that. It did nothing to address the patient's ischemic heart disease, but it did let them continue to have their MIs without pain. When cardiac surgery then came in the 1950s, there was really no need anymore for cardiac sympathectomy, and then in the 1970s there was a very mild resurgence of it when Long QT syndrome was defined. However, by the time Long QT syndrome became routinely identified by cardiologists, uh, we were already at the point of ICDs being available, and so ICD became the primary treatment for Long QT syndrome. Um, so sympathectomy sat on the back burner again for another 30 years, and it's only been in the last 10 years that the newer techniques of cardiac sympathectomy have come back into play. And the reason for that is some patients with long QT continue to have life threatening events despite appropriate medical treatment, despite ICDs, and so it was resurrected as one more possibility of how can we control these rhythms. And so I just wanna go into a little bit of detail of the two patients that were presented. Will that one work? I think it's. It's the big button. Oh sorry, OK. Um, so the first patient had catecholaminergic polymorphic VT, and as the name implies, adrenaline states are the risk for this patient population. It's quite rare. And the kids usually present in the 5 to 10 year age range with syncope or seizure during exercise or during emotional states, um, and that was the case with this child. He came to me via Walmart, um, he was shopping with his father. And he was wanting to stop and look at a toy. His dad was moving on to something else and I told him keep up. Well, he didn't keep up. He was lagging behind, realized where did dad go? and he was pushing the cart very quickly trying to find Dad, and he had a seizure, um, so he does not shop at Walmart anymore and he never pushes shopping carts and, and this was his conclusion, you know, he, he figured out this Walmart did this to me, um. And that's how he adapted. It's a quite lethal disease, 50% mortality by 40 years of age, um, and always related to sudden death, so difficult to treat and highly lethal. The treatment primarily is beta blockers, uh, secondarily a drug called flecainide, and in addition to that, ICD. And with this combination of therapy, most people can live, uh, despite ongoing events, will live a normal lifespan. The risks of the therapies that that we use uh for medications is noncompliance and for ICDs is inappropriate shock. ICDs are great at bringing you back from a life threatening rhythm, but they're not perfect. Um, we have a pretty big audience from all over the world. Can you define ICD? Yes, ICD is an implantable cardioverter defibrillator. It is a pacemaker type device that typically goes in the shoulder with a transvenous lead that goes down to the heart. It senses the heart rhythm continuously whenever it sees ventricular tachycardia or ventricular fibrillation. It can automatically shock the patient instead of having to wait for EMS. And I'll show you some of those rhythms. Uh, if a device inappropriately senses some something, and sometimes it could be electrical interference or sometimes a lead can break and it will see noise, it can deliver a shock that was not needed and in a disease that is catecholamine driven. Um, if they get a shock when they're standing there perfectly healthy, it is hard to then suppress that adrenaline surge, and so there's a risk of sudden death due to inappropriate shocks in patients with CPVT. The benefits of sympathectomy is there is 100% compliance. Um, once they've had it, they can't not take their sympathectomy that day. This child was pretty compliant with taking his beta blocker when he was at mom's house and very noncompliant when he was at dad's house, and so he was difficult to manage that way and. Hopefully can potentially attenuate the effects of adrenaline if there is an inappropriate shock or any other adrenaline surge for any reason. So John, can I ask a question? Uh, so. Having heard about this problem and in operative treatment for 10 minutes now, the, the question I would have is why don't you just treat all of these patients with a with a thoracoscopic day surgery type syphectomy. And, and we're probably headed that way. Um, you know, in the, in the cardiac world, this is not a procedure that is routinely done everywhere. It is just coming into vogue and we're just starting to try and learn who's appropriate for this and, and who is not. And I think your point is well taken and from my standpoint, uh, that would be my approach. Uh, symp operate on Wednesdays. So, so Todd, we, we have a question back here, uh, David Rothstein from Buffalo. Uh, so I have a question about, is there a selection process? When, when I saw your video first, uh, Sophia, I was wondering, you were talking about how to choose levels and how much of the study to take. Is there sort of such a thing as a prone thoracoscopic EP study that you might do? No. OK, thanks. Not, not in terms of the surgery. The, the surgery is really based on landmark. You can't, there's no way to tell, uh, what effect you're gonna get. Um, The only, the only important thing for surgery is that we do anesthetize the ganglion before doing anything to it because you can create a ventricular storm if you just clamp it without numbing it first, um, so that's the one caveat that you did mention in in the video. So, you want to describe the needle you were using? It looks like it looked like it was a sclerotherapy needle that you were using for that. So I don't know if you want to just let people know what you're using to inject the lidocaine. I actually do not know. Yeah, it was. It was, uh, I, I believe that's exactly what it was. It was a laparoscopic long tube catheter with a needle. I don't know what you would call it, but they had him in our OR, and I will tell you. It was a little unsatisfying, uh, kind of bathed it in lidocaine more than actually injected it. Don't tell John that, but John was there with me. I mean, we were, you know, we kept trying to stick it to get it to like infuse the nerve, um, and it did a couple of times, but it wasn't. All that satisfying. Do you agree? You watched it, yeah, and you know, the event became a non-event really, but we had everything there, you know, we had ECMO backup. We had the CT surgeons ready to, to crash them on the ECMO because of the risk of when you do something to that ganglion, uh, you can induce ventricular. storms, is that a hydroical risk or is that actually that so you know that that's a known Todd, Todd, did you, uh, how do you know how much of the stellate ganglion to take? That's the technique. That's exactly, I think what David was alluding to also. We took the lower half. So if you look at the stellate, when it gets to the point where it widens, that's where we took it. And is there science to that? No. But that's what the previous reports showed that if they took the lower half of the steellate, they stopped getting firing. They stopped getting ICD firings, so we copied exactly what the previous reports did, and we did not get any, well, we did not get any horners or anything. What's the risk of horners with that? So I told the patients, you will almost certainly get a horner's, and none of them did. Well, none of them, but neither of them did. So time after time it didn't happen. Exactly, but, but, so I tell them you're going to get a horner's. I mean, well, you either have a horner's or you die. So I mean, to me a horner's is a little bit of an OK consolation. I mean, so I, I tell them you're gonna probably have ptosis, meiosis, and hydrosis. I tell them all of those things and I was surprised that neither of them did. It actually made me worried that I didn't do a sufficient. Inferior ganglionectomy, but they haven't had any firingclinically it's it's been very effective. I got lucky twice, so they're not on medicines anymore. No, no, no, they're on medicines. They still have an ICD. Yeah, I wouldn't trust this as a cure or a sole therapy. The risks are just too high. Um, so he's done well. He's 2.5 years now post sympathectomy with no ICD shocks, um, since that surgery. Um, the second girl's a teenager with hypertrophic cardiomyopathy. Um, it's an abnormal thickening of the heart. It often causes obstruction also. In her case, she does not have obstruction, she just has the abnormal thickening, and it puts them at risk for electrical chaos and ventricular fibrillation. Hypertrophic cardiomyopathy is far and away the number one cause of sudden death in young athletes. It accounts for 50% of sudden deaths in the United States. Uh, there's about 20 other diagnoses that account for the other 50%, so it's way ahead of everything else. Um, this girl had her first arrest during a basketball game. Um, the school nurse happened to be there and they had an AED and it was used in a quick amount of time and she suffered no neurologic effects from her arrest. She was admitted, worked up. Her diagnosis was made. An ICD was implanted and she was started on a beta blocker and then she did very well for six months, um, and she wanted to play basketball and. I did everything I could to talk her out of that idea, but the, the current guidelines which came out in 2015 say if you've had an arrest and if you have an ICD you should not play competitive sports. But in 2015 they added a tail to that phrase and they added, but if you do, and, and that changed the whole nature of what we do around the country. And so they required no events for six months that they have a functional device and they're compliant with their medications, so she met all that she wanted to play and we said, OK, if you're going to play, I want to make sure that you're safe to play. So we put her on the treadmill in my office. And she had a non-sustained run of VT right around 150 beats a minute when, when she went into that and I stopped the test. Um, I switched her beta blocker. I put her on a more, more potent beta blocker, a higher dose of beta blocker, brought her back in a week, and repeated the stress test, and this is why they call them a stress test. It's very stressful for the staff in the room, um. I don't know if I can point out, but on the left of the screen, the top button and hold it down. Uh There you go, do it again. Right here is the tail end of her exercise. Her heart rate is just over 150 beats a minute, and then she fibrillates, and this is an OMG moment. Um, she was successfully cardioverted with a single shock in the stress lab. By the time the ICU staff got there, she was sitting up and talking to me. We switched her medications, adjusted her device, and, and sent her home. Um, 10 days later she wanted to go on a field trip to Washington DC and I strongly argue with you. I don't think this is a good idea right now, but she wanted to go, so we put a very specific plan in place. The same school nurse who was at the basketball game was going on the trip. She was gonna room with the school nurse. The school nurse had an AED in addition to the girl's ICD. She was not allowed to climb or run. If there was any climbing, she either had to take a wheelchair or an elevator. And no running. She had a momentary lapse in judgment and decided to run up the steps to the Lincoln Memorial. She made it to the top and arrested. She got 9 shocks from her ICD at the time and then got admitted to Children's National there and. What this is an electrogram from inside. The ICD, so this is what it sees. And whenever it sees an event trigger, it's not your pointer's not. Um, on the top, top plate. It just shows me the cycle length of her heart before the event and so her heart rate is going right between 145 and 150 beats a minute. And then as soon as it sees ventricular fibrillation ensue, then it records the electrograms for me to look at. On the bottom plate of the screen, you can see a, a big block artifact that is the shock being delivered. And then subsequent to that she's back in sinus rhythm. OK, so here is the. Here is where the shock is delivered. This is sinus rhythm ensuing. She got life flighted back to children. She sat in our ICU for 3 weeks after being in their ICU for 1 week. Um, I readjusted her medications again. She's now on multiple high potency medications, but I don't know when I can trust her to go home. Um, that when are you going to run up the steps again? So prior to sending her home, we brought a treadmill down to the ICU. We had half the hospital in the room ready to resuscitate her in case anything happened, and it did. Um, when her heart rate hit 140 beats a minute, she fibrillated again. It took several minutes of CPR, multiple medications, and 13 shocks between the device and external, and at that point. Um, so this is, uh, an internal tracing of some of those events. What you don't see here is she had just received a shock and that took her out of her rhythm. She was back into a normal rhythm for a few beats and then she fibrillated again. The device detected again. It shocked her again here for a couple of beats. She's out of it and then she's back into it again. So we went through this process for several minutes trying to get her back. Um, ultimately did with no neurologic damage, um, but we were, I know. Um, stuck with, OK, what are our options now? And, and conferences, um, between cardiologists both at our institution and Cleveland Clinic, the general consensus was she needs a transplant. Because she is going to die from a malignant ventricular arrhythmia, and Agreeing with she may need a transplant, it's just really hard to take a healthy 14 year old girl whose heart function is perfectly normal and she is asymptomatic except for these events and transplant her because she will not be alive 20 years from now with a heart transplant. Um, and she definitely won't feel better with a heart transplant, so we talked about it but decided let's try a sympathectomy and see if that can make any difference before we head her down a path of a transplant. So we did that, um, I let her recover in the ICU for one week, put her back on the treadmill again in the ICU with the whole hospital there this time, um, and, and no surprise she was. Really scared to step on the treadmill. Um, it took a lot of, of coaxing, uh, but she did. I pushed her to a heart rate of 180 beats per minute, which on the multiple big drugs that she was on, that was about exhaustion, and she did not have a single PVC and she's now 10 months, uh, post sympathectomy and has not had an episode of VT, has not required a shock, so. Tremendous benefit to these two patients. Will it benefit everybody? No. Do I think CPVT patients. Um, uh, can use it from the outset, yes, I do. Um, it is a rare disease. I have one other patient right now with CPVT. That has neither an ICD nor a sympathectomy, and the reason being she's, she's asymptomatic on medications. It's an Amish family and their desire is she's well controlled, um, let's not do anything else because the community is gonna pay this so, so other than CPVT. So that we all know it can go back to our institutions. OK, there you go. There's your, there's your softball, uh, pitch. Can I ask you a couple of questions for you? What's the, do you have any idea what the durability of this is? No, really, this is, it's all anecdotal reports right now. There, there are no large studies saying how, how effective is this, um, who should have it, or when. It's just brand new. There's very few centers doing it, yeah. And then the other question will demonstrate my ignorance about the innervation of the heart. You only have to do the left side. You only have to do the left side. Yeah, yes, that surprised me too. Why is that? Because the, because the heart is a left-sided structure. The sympathetic chain comes from the left side to the heart. So, huh, interesting. What about these, these patients undergoing induction of anesthesia, is that a risk for them? Always, and you know, I, I face that a lot when they're, when they're getting ENT work done and it's gonna be in an office practice, um, that seemed like a good idea. It seems like a bad, you know, I, I do counsel the families and I talk with the ENT that, you know, anyone who's giving anesthetics to these patients needs to be capable of full resuscitation. And if you're not, then you need to rethink the location of where you're gonna be doing the, the procedure. So almost everybody who has an ICD is gonna get their, even their minor procedures done in our hospital, um, because if it requires an inhaled or an IV anesthetic, their risks are, are greater. And, and just to just to, uh. To, to, so when Sophia presented this in London, Oliver Munster was wondering why we injected the, the nerve with lidocaine. He said he's never injected them for hyperhydrosis, and I think the key to everyone understands is totally different, um, that we had this patient, we were ready for World War 3, and I mean we had ECMO ready. We had her padded. It was like totally prepared for, for badness, and it didn't happen, but. But the, yeah, hyperhydrosis patients don't have genetic malignant arrhythmia syndrome, so. It's work. No, I think it's the distance, OK. All right, well, that was great. I think, uh, that's something, I mean, this is one of those things that's just new. It's nothing that we really know about, but most of you already do sympathectomy anyway. And I, I think be aware of this because it is coming, um, so Ray, have you seen this in the adult world? No, most people aren't doing this for adult problems anymore as he knows. So I mean it just once you have your open heart surgery, you're actually kind of doing a sympathectomy anyhow, so I think 11 potential things that haven't been looked at yet is what is there any benefit to afib. Um, and, and if so, you're gonna get a lot of, you will be inundated. Yes, there's 30 million afib adults walking around this country. Um, so if anybody does that study and shows there is benefit to doing it, so is there a model for afib, an animal model, or I, I don't deal with afib being pediatric, so I, that's not something I keep up on. There is a, a big. Pushed though by large corporations on neuromodulation of the sympathetic and parasynthetic plexus right now. Uh, the alphabet company and Google, uh, partnered with GSK and a company called Verily with 100 million to look at neuromodulation of the sympathetic and parasympathetic plexuses with injectable neurostimulators. So the future will be, do you need to cut that or can you inject a neurostimulator to. Modulate the sympathetic and parasympathetic chains. Interesting. So that's the future of this for both hypertension, arrhythmias, and once you get the alphabet and Google involved, a lot of money is going into looking at this right now. Well, Ray, you're talking like you know about nerves. So, uh, so it might be true. So, uh, Ray Anders, uh, well, John, thank you very much, and Sophia, that was exciting.