OK. All right. Looks like it's a little past 7, so probably time to get started. Good morning, everyone. Uh, thanks for coming to Surgery Anesthesia Grand Rounds. Uh, we're gonna talk today about uh mid-aortic syndrome, and it's, uh, gonna be an interdisciplinary, uh, talk. So I have, uh, my colleagues, uh, uh, from the group, uh, all here. So we're all gonna give a sort of a short, uh, segment on, uh, our particular, uh, area of interest. I don't know how many of you know about our mid-aortic syndrome, uh, and renal vascular hypertension center, uh, but we are recently got center status about a year ago. Um, so we're able to hire, uh, some supportive resources and we've been trying to grow this program. So, our center, or our program effort actually started in 2011. Um, this is an organizational email I sent back then. And, um, uh, this basically started because we realized that we were individually seeing these patients with either renal artery stenosis or uh meteoric syndrome or combination. In our clinics, separate clinics, um, but we weren't really talking to each other too much. And so if they came to surgery, we'd plan some surgical procedure. If they went to interventional radiology, they'd get some angioplasty or something. And, um, uh, if they went to renal, uh, you know, same thing. So, Uh, it turned out we actually need to get together and talk about these patients cause they're quite complex and everyone's, every patient is, um, uh, very individual, um, in terms of their anatomy, and their, uh, medical problems. And so, uh, once we realized this, we formed the center and we, uh, started meeting, uh, regularly, uh, seeing patients and made an effort, uh, to, uh, first, uh, write our experience up, our joint experience, uh, which got us, um, sort of into literature as a group. And then, um, Uh, word of mouth, um, uh, between the families, uh, uh, started happening and, um, that's how our referral, um, uh, base, uh, basically increased. Um, just, just so you know, there was really one center, one major center in the US doing, doing this kind of work in Michigan. There are a couple of other groups that were doing a little bit of, um, uh, they may have had a cardiologist interested in this or an interventional radiologist, but there was really no, um, fully formed, uh, inter interdisciplinary group, and this Michigan group was primarily surgical. So, uh, this is our group and we started, um, and I was looking at this picture last night and boy, we've, it was 7 years ago, uh, things have changed. Um, but, um, uh, we did get a good group together, um, and, um, uh, so, um, we're, we're all gonna share with you our individual experiences. So, um, I just wanna start off by telling you what mid-aortic syndrome is. It's really a condition, um, that results in anatomic abnormalities of the aorta. Uh, they can be anywhere from the thoracic aorta all the way down, um, to the abdominal aorta. Um, and typically, they involve the, the visceral vessels, so the renal arteries or the mesenteric arteries. And, um, again, the anatomy is widely variable. And, um, the treatment, uh, largely depends on the anatomy and the medical complications resulting from that. The main issue, um, that brings most patients to attention, uh, with Mayer syndrome is, um, uh, with the stenosis of the, um, aorta and or the renals, um, the majority of the patients, uh, will present with hypertension. Um, and you can, the patients can show up in their Uh, pediatrician's office with just random unexplained hypertension or some of them maybe have had, um, exercise, um, uh, induced claudication symptoms, um, just poor exercise tolerance. Um, but blood pressure really is usually the primary, um, uh, presenting, uh, symptom. There's, we have, we've had an occasional, um, neonate or, um, young baby, um, who presents with heart failure, uh, from severe, severe disease, um, and those can be quite, uh, urgent, uh, cases. So, uh, today, uh, for, uh, we're gonna have, uh, Doctor Stein lead off with medical management of the patients, um, from the nephrology group, uh, and then Doctor Ferguson also from nephrology is gonna talk about, um, a genetic study that we've done, um, to try to figure out, uh, what, what the cause of, uh, Men's syndrome is. Um, Doctor Chaudhry from interventional radiology is gonna, uh, talk about, um, uh, renal artery stenosis, uh, and, uh, interventions for that. And then Doctor Porras from Interventional cardiology, um, will talk about, um, interventions for, um, in, uh, for, uh, aortic disease. Uh, and I'll finish off with, uh, some of the, uh, novel procedures that we've developed for this. So, Doctor Stein, Thank you. Um, so, again, I'm gonna review the medical management and our evaluation of mid-aortic syndrome. Both of these, um, topics have evolved over time, um, as our group has gained more experience and seen more and understood more, uh, where to focus our efforts. I have no conflicts of interest. Um, Doctor Kim already reviewed the definition, but typically we're looking at obstructive lesions of the aorta, um, often associated with osteo stenosis of one or, um, more of the major branches. And As he highlighted, we've taken a multidisciplinary approach to the care of these patients, um, in order to try to have the best outcomes and also develop individualized treatment plans. So, um, in thinking about patients that have been to other centers, we often hear from them that someone said, well, they can't have any procedure until they're this age or this weight, or they can't have this medicine. Um, and instead, we're really talking, um, with our entire group to try to develop the best treatment plan to have the best outcomes. So, um, our management is really, um, again, multidisciplinary, where every nearly every patient initially is approached with some sort of medical management. Um, this typically starts when the blood pressure is elevated, the patient has symptoms or there's end organ damage or a combination of those. Um, When we are either failing medical management or we're maximizing medical management and we're still not where we feel we need to be, we move towards an endovascular or a surgical intervention. It's important to note and you'll hear later that a lot of the patients who've undergone endovascular intervention, um, undergo that as a bridge. to surgical management, either to temporize them, to stabilize them, or to allow some of the end organ damage to improve before they undergo a definitive procedure. We do have some patients who undergo endovascular intervention and then are great um for a long time. But you know that on this slide, Uh, every pathway still, um, continues to involve optimizing medical management. Um, so our evaluation of these patients is fairly comprehensive. Um, nearly every patient will have a Doppler ultrasound. Many of them will have a mag 3. They all will have echocardiograms throughout their life. We employ CT angiograms, MR angiograms, DSA, um, or angiography, and head and neck imaging, and I'll go through each of those briefly. We also, um, do a fairly comprehensive laboratory evaluation, often including a genetics evaluation that Doctor Ferguson will review. Um, and lastly, a big part of our evaluation is meeting the team. So as Doctor Kim mentioned, we now have a full-time nurse, a full-time program coordinator, a part-time social worker, um, and all of the specialists, and we've taken, um, a fairly hardline approach to saying that we will not, uh, review a case and then have a patient come just for a procedure. They need to come and meet the team so we can get to know them and really do our own evaluation and ensure that we're doing the right thing. Um, because we often will get information that um has changed over time or, um, with, with our approach, we can sometimes buy more time before undergoing a procedure or we have different ideas about where to go. So we use Doppler ultrasound, um, both as a screening tool and as a monitoring tool. So, as Doctor Kim mentioned, um, very often the kidneys are involved in these lesions and they may not be growing well. Um, on the left-hand side, you can see a normal Doppler, uh, waveform, and on the right-hand side, um, on the bottom, this is a very abnormal Doppler waveform. This is the same patient who underwent a mag 3. And I highlight this because initially, um there would certainly be people who would say, why would you need a mag 3? A mag 3 is to look for urinary obstruction or urinary leak. Um, but what we found is that it's been very useful in looking at, um, at Uh, profusion of the kidney. Um, and so this is actually the same patient as the prior slide, and you can see that the, the left kidney, which is the blue line, has a very swift upstroke and excretion of tracer, whereas the right kidney, there's a delayed, um, uptake and delayed excretion. Um, which moves us to CT angiogram. Um, this has very high sensitivity and specificity. Typically, we don't need sedation even for fairly small children because it's very quick. There's excellent spatial resolution and as you will hear, our surgeons really prefer these for surgical planning. Um, unfortunately, we still, there's radiation. Um, there's contrast exposure which can be an issue if you already have compromised renal blood flow. Um, but the times that it's important, it's really important. So this is the same patient from the Doppler ultrasound, and this is her right renal artery. We could not see this on, um, any other imaging, um, but she had a fairly substantial renal artery aneurysm after her, uh, um, after the osteostenosis. And, um, so if she had gone for an endovascular approach, there may, may have been trouble. Um, She had ended up having an auto transplant, uh, which made the most sense, but this was really crucial for surgical planning. In terms of CTA, it's most useful, um, again, for surgical planning and for really evaluating the length of lesions, um, and the extent of lesions. This is, uh, a patient who was picked up with high blood pressure at her 4 year old while child check. Her blood pressure was extremely high. Um, she, um, underwent an evaluation which showed a fairly discrete lesion. Um, she does not have an associated syndrome, and she has, uh, since had surgery and then Um, a catheterization for dilation of one of the, um, one of the areas. Um, there are some new avenues coming along. Um, we're working with the radiology group very actively, um, and they are developing new MRA sequences that get us better imaging and better spatial resolution. This is a post-op MRA of a patient who had, um, an aortic bypass here. Um, and then, um, digital subtraction angiography or angiography, um, remains the gold standard for the diagnosis of renal vascular lesions, um, but as we all know, it's invasive, um, requires, um, Anesthesia, um, and has potentially other risks. So, not all patients receive this, although, um, more frequently, um, um, Needing to move to this so that we have the best picture and so that we um aren't surprised because we don't like to be surprised. Um, and on DSA we can certainly see things that we can't see by other imaging modalities. So we can see, um, stenosis here, we can see aneurysms, and we can see parenchymal lesions, which is, um, which is also very important. Um, this comes up, oops, sorry, um, as part of our medical management, which I'll mention in a few slides. Now, the last thing I mentioned on the evaluation side was brain imaging or head and neck imaging, and People might say, why do you get that? We said mid-aortic syndrome, it's mid-aorta, it's in the middle. Um, but we, um, this is a, an example of a patient with neurofibromatosis, and this is her, and I have permission to use her photo. Um, she had fairly severe, uh, mid-aortic syndrome and renal artery stenosis, um, at the origins of the renals. Um, we had recommended head and neck imaging for her. She has neurofibromatosis. Um, on head and neck imaging, she was found to have severe moyamoa disease bilaterally. Um, had we proceeded with an aortic bypass or other intervention that dropped her blood pressure substantially, she would have likely had a catastrophic stroke. Um, as it is, she underwent bilateral, um, Palsyningiosis for the moya moa, um, nearly a year in advance of aortic bypass. Um, And it was felt that her brain was fairly well protected. She still, unfortunately, had a stroke but has recovered very well. Um, and again, the feeling from neurology and neurosurgery was that if, um, we had proceeded with surgery a year before and not picked up the Moya Moya disease, that stroke would have been catastrophic. This is her a few months after her stroke with her new dog. So, um, moving on to why patients develop hypertension, we know that stenosis, um, leads to increased circulating renin levels, which activates the, the angiotensin system and leads to complex effects within the body, um, all of which end in, um, hypertension and can lead to end organ damage. In terms of medical management, it's critical for us to determine the goal because the goal is not always normal intensive blood pressures. And this patient um here is having surgery tomorrow, so whoever is um covering 7 South today, we should speak after this cause he's being admitted later today. Um, this patient, um, is currently used to blood pressures that are 160s to 170s over 110s and he is 4 years old. So, um, oops. Um, so again, the goal is not always normal blood pressures. Sometimes the goal is just safe blood pressure, sometimes, that's the best we can do. We also wanna protect the brain, which is why we have adopted fairly universal screening of all patients with brain imaging to ensure that we're not missing, um, cerebrovascular lesions, which would be, which would result in, um, uh, cerebrovascular injury if we lower the blood pressure too much. Um, we're always monitoring for side effects and we're monitoring for claudication as Doctor Kim said. So often these patients are requiring 3 to 5 medications. Fairly often, the treatment has started before the patient reaches Boston Children's. Um, this is an example of a patient who underwent an aortic, um, uh, a venous patch, sorry, as a baby. He presented to us as a teenager after someone heard an abdominal bruitit and said we should probably get repeat imaging and he had a large abdominal aneurysm. Um, so this patient, you came to us like this. It's not how we would have approached him as an infant, but it's what was done back then at many centers, um, and so, Um, so we're not always starting at the beginning. Sometimes we're starting in the middle. Um, for medications, we'll typically start with a low dose and titrate to effect. We always want to try to maximize one agent before we add a second agent. Um, and the medications can include calcium channel blockers, beta blockers, alpha blockers, ACE inhibitors, and ARBs, diuretics, and vasodilators. We have a fair, um, a fair number of patients who require minoxidil, which is the most potent vasodilator. Um, and in terms of blood pressure assessment, we're always looking at what our goals are. Again, this is from, uh, the newest, uh, clinical practice guideline for screening and management of high blood pressure in children. And more and more frequently, we're employing 24 hour ambulatory blood pressure monitoring, both to uh determine patterns of hypertension as well as monitor the effect of our therapies. Um, finally, medical management does not end with surgery. Many patients will still require medications and we counsel all families that the reason to undergo surgery is not, uh, to be medication-free, but to be either more manageable, um, or to preserve renal health. Um, and some patients have progressive disease, so nobody is done. Um, everybody requires long-term follow-up. That's all I have. I think Doctor Ferguson is next. Let me just start with the disclosure that I, I am, I am not a geneticist. Um, so, uh, but I would, um, take this time to describe one of the, uh, recent studies that we've done to try and tease out the genetics of, uh, midi-ortic syndrome. Uh, and these pictures, uh, on the left, this is a patient with neurofibromatosis who, uh, you can see has some obstruction of the aorta predominantly infrarenal. In the middle is a patient with ageal syndrome who has um an obstruction right up about at the level of the renals and has previously gone, um, a right renal artery stent, and then the patient all the way to the right is a patient with Williams syndrome with long segments uh stenosis of the aorta. I have nothing to disclose. So, um, back in 2013, we published, uh, our initial experience with mid-aortic syndrome. It described 53 patients, a, uh, majority of which had some sort of associated syn uh syndrome or diagnosis. And in the literature at the time, uh, these percentages were pretty similar to what had been reported by other groups. There was a, uh, the group from Toronto. Children who in 2015, pulled together all of the different studies in the literature that included 640 cases about of meteortic syndrome, of which they reported uh 64% were idiopathic. So 64%, we didn't know what was causing it. Obviously, our numbers were a little bit different than that, but, um, um, obviously, all the center's experiences were relatively small, so it's not surprising that there's some, uh, some variability there. Uh, right around the time that we published this paper is when, uh, uh, our division hired, uh, Doctor Friedhelm Hildebrand to, as, uh, our new, uh, division chair, and he is a geneticist who's done lots of impressive work to tease out the underpinnings of the genetics of chronic kidney disease and, um, and kidney abnormalities. So, uh with that relationship, we approached him, uh, to collaborate to see if we could do similar work with mid-aortic syndrome. And we hypothesized that a substantial fraction of idiopathic cases of meteortic syndrome may in fact be caused by mutations in vasculopathy candidate genes. And we've, we, we, we uh also hypothesized that uh mutations in some of these genes were probably already known to be associated with syndromic mid-aortic syndrome, whereas other mutations, uh, would not have, uh, previously implicated mid-aortic syndrome, but would have other known, um, uh, would be known to cause other vasculopathies. Um, as a result, from 2016 to 2018, we recruited 36 patients from 35 families with meteortic syndrome and performed Hall exome sequencing to try and, uh, uh, tease out, uh, the genetic underpinnings here. Um, and these were the results of the 35 families, there was no causative mutation detected in 57% of patients, but, uh, in 43%, there were mutations. Uh, the majority had mutations in the NF1 gene, so a gene implicated in neurofibromatosis. There were 6 patients from 5 families. Uh, 4 patients had JAG-1 mutations that's associated with Aliel syndrome. Uh, 3 patients with elastin mutations that's associated with Williams syndrome. And the, the two, others were GAA 6 and RNF 213, which are, were mutations that had never been reported in midiortic syndrome before, and I'll talk about those a little bit more. Um, so, of the 16 individuals found, uh, to have a monogenic cause of disease, there were 10 who had already known clinical diagnoses or genetic diagnosis. So these were not new, and we were recruited them almost as sort of a positive controls. We wanted to ensure that the testing we were doing picked up what we knew to be there. The other 6 were new diagnoses. So there were 2 patients with neurofibromatosis of mutations in neurofibromatosis and one with JAG-1, who did not have diagnosis of the NF or alligals. And the likely, uh, the, the likely reason is because these patients had what were, what were considered minor mutations. So rather than truncating mutations that typically would cause a full-blown syndrome, they had missense mutations that caused an isolated, uh, vascular phenotype, which, uh, was what we presumed we would find, uh, going into the study. Uh, there was one patient with an Elastin mutation, um, and there are, it's known that some patients with elastin mutations do not develop full-blown Williams syndrome, so this was not an entire surprise. And then the other mutations, as I said before, were a bit of a surprise. What the first was an RNF 213, which is a gene that's implicated in the development of Moya-Mya disease. And as I said before, there's no, there were no reports in the literature of anybody with a mutation in this gene that had, uh, caused midi-ortic syndrome. And then the other was in data 6, which, which has been implicated in the development of cardiac outflow obstruction, but never been implicated in mid-aortic syndrome previously. So we found some novel mutations as well. So, what implications does this have? Well, um, well, I think what we've, we've established is that whole exome sequencing and, and other genetic testing can provide a conclusive molecular diagnosis in a good fraction of patients with mid-ortic syndrome. And it's our, our hope that as we uh identify more, um, uh, uh, people with definitive diagnoses that we will have the, uh, the potential to provide important genotypic, uh, and phenotypic correlations as well as optimized clinical management. And I'll give you an example of that. So those patients with, uh, who are identified as having mutations in NF1 genes, this could impact how we manage them moving forward. We know that patients who have NF do not do as well with endovascular interventions. They have a high rate of developing aneurysmal change in vessels that have been, uh, either ballooned or stented, and they do much better with surgical approaches. So, we do think that this has the potential to impact how we will manage patients, uh, moving forward. Thank you. Thank you. Good morning. I'll briefly go through some of the interventional radiology procedures, uh, that we use in mediortic syndrome, but also in renal vascular hypertension. I have no financial conflicts of interest. Um, these are the most common procedures I would say we perform in this, in this patient subset. So this is, uh, been presented already. This is a radiologist's understanding of the renal angiotensin system. Yeah, so I'll skip through a lot of the middle stuff, but it goes that the diminished perfusion pressure leads to increased renin and eventually you get to increased hypertension. So, however, measuring serum renin levels can fluctuate uh based on intravascular volume and salt consumption and therefore, it's not really a sensitive or a specific assessment of renal vascular hypertension. So the rationale behind, uh, selective renal renal sampling is that you, if you directly catheterize the, the renal vein, you can reflect the physiological response in that portion of the kidney to decrease renal renal blood flow and profusion. Many different ratios calculated. The simplest ones are the venous to arterial ones. If you take, uh, the venous, uh, levels within different parts of the kidney and compare it to the arterial level. And in most cases, we just use the intravenal IVC which correlates well to the arterial red. And the other way to calculate it is to look at the affected side and non-affected side. It obviously this is in cases of unilateral uh renovascular disease. Um, this is a child, I think he was about, uh, a couple of years old, with, with hypertension. And this is a caster within the right renal vein. And as you can see in the subsequent images, we have passed microcaster both into each individual branch of the renal vein to get samples and then obtain samples in both the suprarenal IVC as well as the infrarenal IVC. As mentioned before, arteriography, we tend not to perform, um, routinely anymore just because of significant improvement in cross-sectional imaging. However, there are a few still residual indications, particularly if you have a equivocal cross-sectional study, if you require dynamic pressure measurements, um, and also prior to any therapeutic endovascular procedures. So this is a 17 month old boy with really quite marked hypertension. This is a CTA he had performed prior to the, uh, to, prior to the arteriography. As you can see, it certainly suggests that, well, there are two, arteries on the right side. And certainly the suggestion of a stenosis, at least in one of the arteries and possibly in the, uh, the other artery as well within distal to the osteon. However, we performed the, in addition to the redin sampling, we also performed arteriography and selective, um, arteriography of both arteries, demonstrated, well, these are small, um, there really is no focal stenosis identified. Moving on to angioplasty, we, we tend to, uh, as much as possible, not perform stenting, just obviously for, um, due to reasons that, um, eventually you get instant, um, stenosis, as well as the, there's lack of growth in small children. So, tend to really concentrate on angioplasty and indications would be a focal stenosis and failed optimal therapy or the fact that you're adding multiple anti-hypertensive agents that record to control blood pressure. There are many uh potential, you know, uh, absolute and non-absolute contraindications, but technically, the ones that are contraindications are really long segment stenosis as well as intrarenal stenosis. This is an 11 year old boy with hypertension. You can see there's a discrepancy in size of the kidneys, and as well as the g 3 demonstrated a decreased, um, um, uptake within the upper port of upper pole of the right kidney. And you may be able to see here initial on the initial arteriography and better seen on the, excuse me. Be seen on the subsequent, uh, selective imaging, there is a focal stenosis just distal to the proximal branch of the, uh, super pole, suprapolar artery. So, we use a combination of small, um, 3 to 4 millimeter balloons going up to 5 millimeter balloons as required. And, and this is the post angioplasty appearance. And you can see there's significant improvement, and I believe he's now down to 1 medication to control his blood pressure. In some patients, we actually discover um something like more like fibroid muscular dysplasia. You can see the a very typical beaded appearance. Of this kidney. This is a 14 year old girl with hypertension, and these respond very well to angioplasty, again with uh cut cutting or non-cutting balloons, and as seen on the image on the right-hand side. Moving on to caster directed thrombolysis, um. The indications would be an acute thrombus, um, multiple, again, multiple contraindications. The one you really would be concerned about most would be recent GI bleed. Recent, uh, surgery is a relatively contraindication, active hemorrhage or recent intracranial hemorrhage or stroke. This is, uh, this young boy, I believe he was about 3 at the time, and I think he's now 7, had, uh, media aortic stenosis and And then subsequently had a, an aortic graft placed. In addition to the, the graft, he had PTFE grafts going from the graft itself to the arteries that were not initially mobilized. And he did very well for the first year. Then he presented into his local hospital in Kansas with vomiting, nausea, and initially had a CTA performed, which was read as unremarkable, sent home. But within the course of the next 7 days, he became progressively auric and as well as worsening with worsening hypertension. Went to a different hospital locally, which actually identified the fact that He actually has bilateral renal artery thrombosis with the from the graft itself. And the arteriogram on the right hand side shows that there is actually no pacification of either of the renal arteries from the graft. So this was about 7 days post that he was actually transferred here, and we went on to perform gastro-directed thrombolysis. This is a selective arteriogram on the left-hand side, and this is the initial arteriogram. You can see that very little perfusion in any part of the kidney. Uh, we initially administered some TPA, some heparin, and then left it infusing overnight, came back and performed, uh, further, um, thrombolysis and angioplasty, and this is what the kidney looks like the day after. And on the left-hand side, similar sort of appearance, very little perfusion initially, and this is what the kidney looked like on the uh subsequent day following a combination of thrombolysis and angioplasty. Now, this child did very well despite having Um, basically 7 days of no flow coming through the main renal arteries. And in, in some ways, he actually benefited from the fact that he had me aortic stenosis. He had actually very well developed capsular flow, which managed to, um, maintain his kidneys long enough that we, um, by the time he was revascularized, he did very well and recover became, you know, his hypertension resolved eventually. And also his renal function returned. So these are just a brief overview of the kind of procedures we perform in I in IR. I, there are a couple of procedures I mentioned at the end in parentheses because we don't really perform them uh commonly, if at all. Renal ablation really is the, the last resort. If you can find a focal area of the kidney that does not respond to any other sort of intervention, then you can inject uh alcohol directly into that kidney portion of the kidney itself. And renal generation is a controversial, uh, procedure. Uh, it goes in and out of vogue where you're effectively, um, Administering radiofrequency ablation to portions of the renal arteries and we can potentially decrease the requirement for medical uh therapy. At the moment, uh, the, the jury is very much still out on those. Thank you. Good morning. Um, so where does the cath lab fit into all of this, um, In general, for diffused mid-ortic syndrome, which is more common, uh, the cath level usually play a supportive role and give you some examples there. Um, for isolated lesions, which are not that common, uh, or in the case of supraceliacmiortic syndrome or thoracic lesions, uh, uh, sometimes we can play a more central role. So we'll talk about a couple of examples, uh, here. So starting with palliation of the unstable patient. Um, this is a CT, uh, a reconstruction of a patient that had, um, Atrisia of the abdominal aorta. So you see on the right hand panel there, the aorta kind of stops right after the celiac artery, then reconstitutes distally uh from collaterals. Um, so this patient was 2 years old, presented in outside hospital with severe hypertension and just multi-organ dysfunction, uh, was transferred here on dialysis and event, uh, LV ejection fraction was 10%, uh, so quite ill, not the best surgical candidate. This thing is jumping. Um, so this is the initial angiograms in the cath lab, uh, that's kind of a composite figure. So we had access from the axillary artery and from the uh femoral artery, and, uh, you can see the injections where the aorta, uh, is a retic, and on each end you can see kind of how it tapers, um, which allowed us to try to Um, Hm. Um, recananalyze it. So this is, there's a, a snare here coming from above and a, uh, hydrophilic wire coming from below, uh, getting through this atretic segment, which allowed us to make a, a loop of wire from the femoral artery to the axillary artery. Um, Then after some uh angioplasty, you start to see a lumen, which allows us to get the sheath from above uh across this atritic segment and uh do more dilation, stenting. Um, and this is at the end. Uh, so you see that there's flow now through the aorta that is stented. Uh, you see flow to the left kidney, still don't see flow to the right kidney, and, uh, this is CT following the procedure, um, uh, showing the same thing. It's a little confusing cause it's looking from the back. So that larger kidney is the left kidney, uh, it has two arteries that, with pretty good perfusion after interventions. The right kidney is atrophic. Um, and so just doing this part of it, uh, allowed us to get her out of the ICU, de-intensified off dialysis, uh, and able to just get her in better condition. She had a, a, right nephrectomy laparoscopically, uh, which allowed us to even wean the meds more, get her home. Mm, I don't know what's happening with this thing. Um, and then recently she had a more definitive surgery a couple of years after initial presentation and is doing well. So that's just an example of like supportive management, uh, trying to get the patient to surgery in a better state. Uh, going back to the supraceliac meteortic syndrome or, or thoracic lesions, uh, this is one example of a patient with a neurofibromatosis type one. Doctor Ferguson mentioned. Um, that we've learned over time that, uh, interventions in these patients with neurofibromatosis can, uh, are associated with a, a lot higher incidence of aneurysms and, uh, restenosis, uh, and you see that here, um, this thing is just jumping on its own. Uh, so, uh, I initially presented with mid thoracic aorta lesion there, um, which I don't know, this is just Moving without me doing anything. Um, and then initially looked really good after stenting and dilation, uh, but then 6 months later I developed aneurysms. So this was kind of like the index case for what Doctor Ferguson was referring to, a big learning experience for us. Uh, we were able to put covered stents and exclude the aneurysms, but it was still not the kind of thing that we're looking to, to do. Uh, initially our response was more, we'll just do surgery for a neurofibromatosis type one, but in mid thoracic lesions like this, uh, the surgery is quite involved and it's a big procedure requiring cardiac surgery and vascular surgery and usually done on bypass. Uh, so there was always incentive to try to figure this out. Um, so just looking at what we normally do or traditional approach of angioplasty, you're trying to get a, a therapeutic tear. Uh, by doing balloon angioplasty, sometimes, uh, stenting after that, and this was resulting in a lot of instant stenosis, aneurysms, uh, just more kind of, uh, activating of the disease. Uh, so looking in the literature, there was an animal model of NF1, uh, in which they used angioplasty actually to induce these lesions, so you can see there a big aneurysm in the, in the heterozygote. Um, and on the histology, you see how much there's like this exaggerated intial medial response to angioplasty. So clearly our traditional approach was not gonna be ideal, uh, but we thought maybe if we try to avoid intial injury by just primarily stenting it, uh, and doing this in a more staged fashion, maybe we can avoid this kind of process. So we did that in this patient, uh, again, you see the thoracic lesion there, it's a long segment lesion. Kind of big gradient. Um, so, essentially, instead of dilating and then stenting, we just primarily stent, uh, leave, intentionally leave this in a kind of an hourglass configuration with residual stenosis, and then we just come back three months later and dilate it a little bit more, and 3 months later, a little bit more and you can see the end result there, um, and we haven't had trouble in that patient with aneurysms or, or re-stenosis or instant stenosis. Uh, she's probably 3 or 4 years old. Um, so that seemed to be working for NF1, uh, which, but that's only a small, uh, portion of the patients with meteortic syndrome. There's all kinds of other causes which you've heard about before, so it made us wonder whether we should be doing this kind of approach, whether there were common mechanisms and we should be taking this approach for other thoracic lesions. Then this patient came along. Uh, which is, uh, an interesting one. This patient had presented an outside hospital with that lesion that you see there in the mid-thoracic aorta. Um, and they had actually treated it in a similar way to what I was proposing. It's just primary stenting and not completely resolving everything, and this is what it looked like afterwards. Um, the patient came back 6 months later and had a big gradient again. Um, they got an MRI. They couldn't see much inside the stent because of the MRI. Uh, or the modality, um, and they assumed that there was instant stenosis and, uh, re-obstruction there, so they sent the patient here for, uh, intervention, and we found what they had done looked really good. Uh, however, there was a new lesion, uh, in the, right below the renal arteries. Um, at this point, we were still trying to figure all of this out, so we did our traditional approach of angioplasty, stenting. Uh, and then again 6 months later, big gradient, so we came back and this is just the original lesions for comparison, and this is the, the, the lesion that had been stented primarily without, or, you know, without causing an intimate tear, uh, still looking good, and the part that we had treated with dilation, tear, and stent, uh, had severe, uh, instant stenosis and progression of the disease, um. We did an intravascular ultrasound, uh, to just look at the, the, the wall itself, and you can see here, uh, those bright spots, uh, the red part is the lumen, the bright spots are the stent struts in the thoracic lesion, uh, and the lumen is wide open. There's, you can barely see any entema there, uh, it's, which is the way it should be. Uh, and the lesion we had treated below, uh, you see a lot of, uh, intima medial hyperplasia, a lot of thickening. Uh, uh, inside of the stent, uh, uh, with, which is encroaching concentrically on the lumen and causing a lot of re-obstruction. So this was a kind of like, uh, presumably the same process at two different sites treated in different ways with a different result and went along with what we had learned from the other patient and looking back at our experience, went along with other experiences that we had had, um, so. That kind of encourages us to try to, if we were gonna do something in the calf lab, especially for thoracic lesions, um, we would try to do this in a staged, uh, primary stenting way. So, uh, these are some examples, and, uh, we've done a handful of patients, and it has gone pretty well so far, but it's only a few patients, uh, follow-up, it's only about 3 or 4 years at the most. Uh, and so far we haven't seen a lot of reobstruction or aneurysms or anything like that, but obviously, we still have to get more data. Um, so just to summarize, obviously management of midiortic syndrome is all about teamwork. Uh, our role in the cath lab or in the IR, um, is mostly in diffused disease, uh, will be a supportive role if it's discrete lesions or superciliac MAS, it may play a more central role, uh, and then we obviously learned that adjusting our approach to the underlying mechanism, uh, may help improve our outcomes. So now HBO. All right. So, We often, um, uh, have repeated meetings about patients, and many of these patients eventually come to surgery for a definitive repair. Um, the best example for why it might be useful to wait is, you know, if you have a 3 year old kid and you wanna do something like this, a graft, um, You're probably gonna have to upsize that graft at some point in the kid's life and you wanna try to avoid having to repeat the aortic surgery. It's, um, first time it's difficult, the second time is very difficult. And so, uh, we, we try to grow these kids and if you can temporize um with a lot of the um procedures that you saw, um, previously, um, it's good, you know, get them to maybe like 8 to 10, um, range. Um, uh, it's, it's much less likely that they're gonna need a repeat procedure. So, um, uh, Doctor Fakili and I have been, um, working on the surgical aspects of this, uh, for, uh, the last 7 years or so. And, uh, one of our goals is to try to eliminate the need for prosthetics because prosthetics have problems. They don't grow. Um, they can get infected. Uh, we have seen patients, um, come in who've had grafts for, um, a long time, who, um, have, um, graft, uh, problems. And so, uh, graft problems are difficult to deal with. And, uh, so one of our goals is to try to avoid grafts if possible. And so, um, we have developed some methods, um, uh, to avoid, um, the, the, uh, the use of grafts. Crafts are standard. Uh, they've been used for decades. Um, they're typically used in adults, um, where your life expectancy, um, is much less than when you're, um, you know, 5 years old. And, um, so, Uh, we, we do know that grafts, vortex, sacron can last for decades because there are patients who have had them for decades. Um, but again, you know, we are, we, we're still concerned despite that fact, um, that, uh, grafts, um, aren't the best solution long term. So, um, one of the procedures that we've developed is uh called tissue expander stimulating lengthening of arteries or Tesla. And, um, this, um, the series of panels, um, shows you, um, sort of what this is. Um, What we basically do is we take a patient with meteortic syndrome and we put a tissue expander um behind uh the normal aorta distally in front of the spine, and then we slowly inflate this. And what happens is the aorta has no choice but to lengthen, uh, because it's, um, uh, the spine is not gonna move. Um, and you can get, um, increased length of the normal aorta, and you can resect this and put it together. This is 3 years and this is 6 years, um, in our first patient. Um. Uh, who had, had an excellent outcome. As you learned in the last video, meteortic syndrome is a very rare but serious. I'll shut that off. Um, The these, these animations are being made actually for our website. So, um, uh, uh, I just got hot off the presses, um, and, uh, it's gonna be a part of a video series to teach patients about this. Um, We got the idea from Russ from Rusty at the Eat Center, so yeah, we have to copy it. It's a good idea. Um, so we, um, Uh, this, this is sort of the typical anatomy, um, that we would consider, um, sort of optimal for, um, doing the Tesla procedure. Um, a relatively short segment stenosis, maybe, um, not quite up to the SMA here, um, not quite down to the IMA, uh, you know, about 3.5 centimeters or so, uh, involving the renal arteries. And, um, uh, so what you do, we, uh, stage one, is when you basically put the tissue expander in. So you slide the tissue expander in a surgical procedure. Um, and then stage two is really doing serial injections. Um, and we do one or two a week or so, and you slowly increase the size of this expander. And again, the spine doesn't move, so the aorta does have to move, um, and slowly gets stretched out. Um, and what we see is when you, when you initially, um, uh, with each expansion, you see a little compression of the aorta. But as, uh, by the time you get to the next, um, injection, uh, the aorta is rounded up again. And so it's sort of, you can almost visualize, um, the growth that's happening, uh, as you're doing this. And then stage 3, is another operation. We actually remove the tissue expander, we resect, uh, the damaged portion, and then with this redundant aorta, uh, you can then slide it up, um, do an anastomosis with no grafts. And then you, if there's kidney involvement, you move the kidneys, um, down south and reastomos them either to the aorta, um, or down to the iliacs in a full renal autotransplant. So that's the first procedure. And then the second one, is, um, uh, something we call magic, uh, which, uh, was just published. And this, this is the first sort of index case that, um, showed us that this approach would be possible. And this, um, operation, we actually take a native vessel, and I'll describe which one it is. And we, and we use it as a bypass. Now, you can see, uh, in the acute phase, the bypass is just not, it doesn't look like it's big enough and it's not big enough. Um. But we've learned that if you put this, um, graft in, in a, an appropriate way over time, uh, 6 months and 18 months, um, the graft itself, because it's your own tissue, has the ability to grow. Um, and, um, by the time it gets to this size, it's basically, um, in addition to what's going through the native aorta, uh, it's basically, uh, delivering normal, uh, blood flow distally. So these patients, um, uh, tend to have some, uh, uh, this vessel, and this is a mesenteric artery. It's called the meandering mesenteric artery. Uh, some of you, uh, might, if you're paying attention in, in anatomy class, uh, it's archiverielin. Um, and, um, what we noticed is that a lot of these patients have an enlarged, um, MMA, meandering mesenteric artery. The reason, um, is that when they have their meteoric syndrome and their stenosis, this acts either as a forward shunt um from the SMA to the IMA to bypass the, uh, the narrowed aorta or a reverse shunt, um, to, um, if they have a severe SMA stenosis, um, their SMA blood flow actually can come predominantly, um, in retrograde fashion through that, uh, bypass. Now, the interesting thing is that this artery does grow in situ. In, in other words, a lot of our patients turns out to have this artery, and it's big, a lot bigger than it should be. And so that was sort of the reason why we thought it would actually continue to grow when used as a bypass. And so, In the magic procedure, what you do is you take this artery out as a free graft. Then, um, in cases where it flows retrograde, we actually reastomose it so that retrograde flow is maintained to supply the SMA. Um, and then, um, you, this, this is, uh, becomes a free graft, and we basically do a bypass with that graft. One of the tricks that we've, uh, figured out is that you need to make a wide-open anastomosis. We actually, uh, do a long spatulation of the ends of the artery on both ends, um, to make the anastomosis much bigger than the diameter of the artery initially. But what we don't wanna do is create a, a small anastomosis and have that become a sort of flow restricting anastomosis as the artery grows. So we sort of anticipate future growth. We make this sort of as big as it should be at the end. And then, um, we hope that this thing will grow into, into the anastomosis on each end. And uh it grows like magic over time, as you saw. We um We also, um, usually at the same procedure, we'll move both kidneys, um, and, uh, uh, often we'll do a full auto transplant. So you take out each kidney individually, um, you sort of, um, um, put it on ice, uh, for a few, uh, temporarily while you work on the, um, renal artery, uh, to trim it up. Um, we've also done aneurysm resections of renal artery aneurysms, um, on the back table as well. So this allows you to sort of work on the kidney in a relaxed fashion. Uh, and then you put it back in sort of where we'd normally put kidney transplants, um, down in the iliac vessels. So with those two procedures, uh, from a surgical perspective, we've been able to avoid using grafts in a number of, uh, patients. Um, and, um, uh, I think overall, I think the magic procedure is probably gonna end up being more applicable to more patients than the Tesla, um, just based on anatomy. Um, but, um, obviously, we're still early in this experience. So, uh, you know, I just wanna thank all my colleagues, um, who presented, um, and the rest of the, uh, group. Um, particularly thanks, uh, for, from, from a surgical perspective to Kash uh Vikili, who has been a partner in all of this, um, in all of this work, um, over the years. And, um, I think, you know, uh, what you heard today is really a good example of what this hospital is really good at, it's just interdisciplinary, uh, program development. Um, I think it's really good to get a lot of, uh, interested, smart people in the room together, think about a very difficult problem together. And come up with uh, uh, unique, unique solutions, uh, for these difficult problems. Um, so thank you, uh, very much. Um, I think, uh, all of us would be happy to entertain any questions at this point. I'll see what can sound this morning so I'll, I'll just start you. I'd like to thank you. I think it's great to see what. Thank you. That Other questions I can go without a microphone if I don't have one. I, I just wanna a, a, a comment, um, more of a question, and that I think, you know, home base last comment is really, really crucial. Um, these are really rare complicated cases and really rare complicated problems generally require smart people from just different discipline. Of color race sort of gave the for the non-nephrologist view of the rental independent system, right? It's like. Narrow, lots of red blood pressure goes up, you know, I, I do that, I've been very, very peripherally sort of, um, cheerleader for this, and I, I do, how do you measure this stuff works? Well you just surgeons do to count how many antipertensive medications they're on. They were on 5 before and then 1 now something was good, right? That's the surgeon's view of nephrology of hypertension. But not only is this complicated, this is really rare. So think about what happens to patients. In the world when there isn't a place to come where people work together and we have an experience. So every one of these patients come from someplace else where without this kind of effort, They would have a doc, a specialist who read some paper, who had some experience, and they would do what they know how to do. They don't have to put a stent in. They don't anything about defense. They didn't hurt him when a mo, the kids throats out when they, you know. These patients basically were trashed, right? And we see some of them come in. That is what this hospital should be good at. That is the sweet spot, the special sauce of this they should be good at, and we have sort of the history of these silos of all these departments of excellence. And the only way you solve problems like this is by getting smart people together in a room and getting to work together. And one of the problems, one of my passions for strategic institution has been to support them because you get a bunch of docs who went to medical school, did all. Highly intensive training and specialties, and you put them together, no really smart. They, they're not, they didn't go to school with project managers. They didn't go to school to develop databases. They didn't go to school to be marketers. They didn't go to school to raise money. They, they didn't go to school to, um, to, to be coordinators. I can tell you, right, I think the most gratified statement of this whole day is when Deb Stein said, and now I'm a full-time nurse, and now we have a full-time coordinator. Now we have part-time social worker, and I can tell you that she was all of those things for the first several years of this program. The database was her, you know, figuring out how to write one on her laptop and her taking all the phones from, from the patients and her secretary complaining, I don't have time to do this. So she was the program in terms of the coordination effort and with the sort of center of excellence, the silk centers, and they applied and this is an obvious application for this kind of thing. And you see this in the ORs every day. Um, the agen treatment program is the one that's sort of obvious to everybody every day that's, that's burdened in terms of volume. These patients are all trash and have horrible lives unless a place like this can support them to work together across discipline within a a specific department. All departments have lots of resources and you can build programs that only take one specialty. But these rare and complex problems take multiple disciplines, and who hires a nurse. Right. And so a program might not develop because you can't hire. That's absurd. So we've developed a mechanism which isn't perfect within the institution to support people who, who wanna do this, and it's a lot of uphill climb of these guys and others who are in the room and not in the room to build this program, um, but the message is to follow their example. When you have good ideas, find smart people down the hall, and then ask for help. There's a mechanism now to get help to do it. You don't have to go yourself, you know. The patients come because of Facebook. It's not because the literature search is necessarily one of the doctors finding, right? The website, you look at the website, you see these videos like it's incredible, and these patients refer to each other and they come. So, um, kudos to, to this team, not only for the innovation and the smarts and the genes and, and all that, but the persistence to, to work together and the egos aside. Um, this is how, how you change the world from a strategic perspective. This is what, this is what's making the national International Hospital happen is these little programs. Um, now accounts for an enormous success of case this possible and an even bigger percent of the margin at all. Just to to you all. Now there's no, not that no cross. Let's start with. Thank you. The comments Tom, so, uh, HP, uh, that was an amazing present. Magic procedure, uh, it's that, uh, artery actually continues to. Uh, yeah, it's also equally amazing that the aorta doesn't. Uh If anyone's taking a look and trying to find what, Just stays there and it remains stenotic still having the pressure gradient from above while the normal artery. Yeah, um, you know, the, the abnormal aortas, um, histologically, I mean, they're not normal, right? And they're, uh, and it's unclear, you know, some of these patients are born like this, and we don't know if it's a progressive disease in those cases or if it was just a sort of a malformation. Um, Uh, in utero. Uh, we do know that some patients, um, for instance, with inflammatory diseases like Takayasu's, um, do have progression of disease. And we have seen some kids with idiopathic meteoric syndrome also have a progressive disease in front of our eyes. Um, but yeah, we, we, we don't fully understand, um, what the pathophyysiology of the actual vessel is, um, that, that causes this. Um, but, um, it's clear that these mesenteric arteries are, uh, that, that, that, you know, meandering mesenteric artery is almost designed to grow, um, when you have, um, SMA stenosis, um, that you see in adults too, uh, with the aneurysms, um, and, and inclusive disease. So, um, uh, that extra vessel was put there for a reason, and, um, uh, you know, we've, we've just taken advantage of that. Chris, So on some of your other kids that go looking at the, the older kid you tried Tesla. genetic Uh, yeah, we haven't. So the oldest one we did, the aortic test on was 7. we tried an iliac one on a teenager, and that's the one that failed. Um, and that may have been a combination of age and we just pushed too hard. We, we stretched too much. Um, and the gap was just too long. But, um, I mean, I, I sort of, I, I guess most of us sort of feel like, uh, age does matter, you know, little kids are more flexible than adults. Um, and more adaptable, but, uh, we haven't actually tested that, um, in the lab or anything like that. Like, I don't think you could stretch an 80 year old's arteries real well, you know. Any other questions? All right. If not, thank you very much.
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