In this previously broadcast webinar, Mayo Clinic heart experts Elizabeth H. Stephens, M.D., Ph.D. , pediatric and congenital heart surgeon, and M. Yasir Qureshi, M.B.B.S. , pediatric cardiologist, review the diagnosis of vascular rings based on anatomy and the treatment options available for each type. Vascular rings and slings are increasingly diagnosed with the advent of fetal screening and frequency of cross-sectional imaging. There are a wide variety of types, and management depends on anatomy, age and symptoms.
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Good afternoon. I'm Doctor Elizabeth Stevens, and I am a pediatric and congenital cardiac surgeon here at Mayo. I'm joined by Doctor Yasser Qureshi, who is a pediatric cardiologist with additional training in advanced imaging. We both have specialties in vascular rings, and we're excited to discuss this topic. Throughout this webinar, you can actually put in your question the QA portion. So we'll be looking for those questions and answering them most likely at the end, but please do put your questions there. Um, and yeah, so maybe we should start by covering the types of vassar rings, and I know you have some images to help explain them. Yeah, uh, thank you, Elizabeth, and, uh, welcome all to our webinar this afternoon. Uh, we're hoping to have a good session discussing about the most common vascular rings. Um, we'll, we'll talk about the anatomy, the management, um, and then don't forget to ask questions through the Q&A, um, um, portion of the webinar. So we'll start with this, uh, the first, um, one of the, the two more common vascular rings are double aortic arch and the right aortic arch with the barren left subclavian artery. So we'll start with this first one, which is the more typical, uh, one where you can actually see the blood vessels essentially all around. Around the uh trachea and esophagus, and that's the double aortic arch because we have the normal left-sided arch and the right sided arch, both of them joining together at the descending aorta, um, and then creating a, a true vascular ring around the trachea and esophagus. Um, as you can see, you know, the, the trachea and esophagus are really getting strangulated between these vessels, and, um, that's where all the clinical symptoms in these patients also arrive. Now just going over, uh, through the anatomy a little bit further, you can see that. Um, both arches basically give rise to the branches, um, their corresponding branches, so the right arch gives the right common carotid and the right subclavian artery, and the left arch gives rise to the left common carotid and the left subclavian artery. Most of the time there there is one arch which is going to be dominant and most commonly it's going to be the right arch, which will be dominant, and that's advantageous when one arch is dominant because the other one can be sacrificed during the repair. If they're both similar in size, that can actually be problematic. Um, so most commonly, like I said, the right arch tends to be more dominant, and, um, the ductus arteriosis in these patients with double aortic arc is typically on the opposite side of the dominant aorta. So if it's a dominant right arch, the ductus will be left sided, which is the more typical um configuration. But if there is a dominant left arch, then uh you have a right ductus arteriosis. The important thing uh over here is the recurring laryngeal nerve that goes right around the left arch, so you can see why this is important, um, as Doctor Steven will have to fix this, trying to avoid any damage to um this nerve. Here are some CT examples. Um, CT is the most definitive way for us to, uh, to assess the type of aortic arch and assess its, uh, effect on trachea and esophagus to some extent. The so in the top panels over here we're seeing the double aortic arch where you have the left arch and a right arch. The trachea goes right in the center and you can see all four brachocephalic vessels arising from their corresponding arches. This view we're basically looking from the head down through the barrel where the trachea and esophagus will lie right in the center. Um, and this one is from the side, from the left side, looking at the left arch and in the background, we can see the right arch similar to the picture we saw earlier. Over here, um, you can see the trachea right in the center of the right aortic arch and the left aortic arch, which are being cut, um, in the short axis over here. You can also notice that the right aortic arch in all of these pictures, you can see that the right aortic arch is typically slightly more superior than the left arch. That's not to be confused with a cervical arch. Because the right aortic arch is always going to be a little bit higher than the left aortic arch. A true cervical arch is when it goes all the way up into the cervix, meaning the neck. Um, so this is certainly not a cervical arch. Now, very commonly, one of the portions of the non-dominant arch can be atratic and that's what you can see in this picture. We have a dominant right arch. We're kind of looking at the CT from the uh from the back and uh slightly to the left side, um, as the descending aorta comes down here, you can see a little bit of bird beaking or this pointing uh thing right here. That's where the. Um, there is another ligament in between the left subclavian artery and the descending aorta. So that's where an atratic segment of the double aortic arch exists, and that's another ligament that. The surgeons will have to uh transect at the time of surgery. So even though it may look like it's just right arch and the normal kind of like a bifurcating uh first branch, it's actually a left arch with an atratic segment. And then here's an example of a dominant left arch and a smaller right arch. So this is less common, the most common one, like I mentioned, is a right dominant arch, um, in that setting. Moving on to the other most common um vascular ring, and this is probably the the most common uh vascular ring, that's the right aortic arch with the barren left subclavian artery. So here you can see the right arch again exists um and then on the left side, the first branch instead of having an ominate artery, basically only giving rise to the um left common carotid artery. And the left subclavian artery arises posteriorly from the descending aorta, usually through a diverticulum of Comoro, which gives rise to the left subclavian artery. So how does this constitute a vascular ring? Well, the, the thing that really makes it a is the presence of ductus arteriosis. Now, in most patients in the postnatal life, there's not going to be a ductus arteriosis, it will really be a ligamentum arteriosum in this area that actually completes the vascular ring that stragulates the trachea and esophagus in the center. So again, another form of a typical vascular ring where you have the, the, the right aortic arts on the right side, the left common carotid and uh ligamentum arteriosis on the left side, and posteriorly, you have the either diverticulum of coro or a portion of the descending aorta which causes the compression in these patients. Here's another model that kind of shows exactly how looking from the front it will look like you have a right side of the aorta cars. The first branch actually goes to the left side, so that's important when we're doing echo imaging that we look for the first branching pattern going to the left side. And this one does not bifurcate, as I mentioned earlier, into like an anoinate artery. Instead you have the left subclavian artery coming off all the way from the back, from the descending aorta via a diverticulum of Comoro, and that would give rise to the left subclavian artery. This diverticulum of Comoro is really just the dilated proximal portion of the left subclavian artery or the dorsal aorta on the left side that persists. Before the baby is born in utero, all the cardiac output is going through the palm. Pulmonary artery across the ductus arteriosis through this diverticulum of commoro down the descending aorta. That's the reason why this vessel is so big as compared to the rest of the subclavian artery. So that's the origin of the diverticulum of Comoro. Elizabeth, you brought some, uh, 3D models for us, so that was a great summer. Yes, sir. No, we do. I wonder if I should just start with this is the one we were just talking about. So this is the right arch. So if you concentrate on the kind of dark orange, that's the aorta that's swinging to the right, and here is trachea here that yellow. If I turn it around, you can see the aorta coming down the back and the subclavian right here. There's the coils of uh diverticulum, and that is the ligamentum right there. Here I'll use a pointer to try to right here. So if you look at on the side, you can appreciate trachea in yellow, very compressed esophagus in that tangerine color. Um, and then I'll show you, this is actually a patient who had right arch a left subclavian, had a repair in another institution but was still was having symptoms and you can appreciate, um, so she had a ligament to release. Let me see if I can angle this appropriately. So if you look right in there. You can see, here's the corals diverticulum here. Here's the subclavian, and you're sitting right on the spine there. There's the esophagus. Let me get it a little better, esophagus that's having trouble getting compressed. So even though the ligamentum was released, she still had compression of other structures and in here is the trachea blue. It's a little harder to see. Um, And then I wanna show you the double arch model. So this one, if you look um from, let me get you oriented. OK, so here we have the arch aortic arch coming up. We have a right dominant arch here and a left that's not dominant, and then this is the area where it connects. So that's the left arch connecting to the right. Coming down and there's the airway here, the esophagus. All right. Um, so many of these diagnoses are coming through the pediatrician or internal medicine, even emergency. Can you talk through imaging and how these come to light? Yeah, um, you know, that's, that's really important part because, um, the primary care providers or even the ER physicians are really at the front. Line to actually make the diagnosis of these patients and if they don't come to us, we can't really evaluate those any further, um, so it's really important to pay attention to details to, to things that can be helpful um in identifying these vascular rings because sometimes the, these uh symptoms can be pretty subtle. One of the things um that. You know, as well, actually, let's, let's look at it from the anatomic perspective. We have the trachea and we have the esophagus that both of them are getting compressed, uh, between these, uh, these vessels, so you can have symptoms from either one. You can have symptoms because of tracheal obstruction, which are usually more dramatic and will bring to attention um a lot more quickly. And you know, those symptoms could be simple things like, you know, noisy breathing, croup, coughing, repeated pneumonias, um, you know, um, some patients will actually get diagnosed with asthma, so that's what one of my pet peeves that I tell our residents and trainees that, you know, if you have a new toddler with a diagnosis of. Asthma with no family history of A to B, no asthma, no allergic rhinitis or aerobic dermatitis in the family, and only the toddler has a, has asthma, think a vascular ring. That's really an important thing that, uh, because a lot of those symptoms may be uh pretty subtle and you know, anybody who's having a little noisy breathing and. Uh, we'll get diagnosed with asthma and try to inhale it and things like that, and they don't get better, so that should again be another reason for you to actually assess further. Now, coming from the um esophageal compression standpoint, those are a little bit more subtle, especially in children uh where adults can actually, you know, uh tell that they're having dysphagia or they need, uh, they can't really swallow more easily. Um, children are sneaky in the sense that they can, um, they can actually look at, um, you know, they, they, they learn how to swallow with those rings, so they know exactly what they have to do to actually make it easy for them to swallow. They take smaller bites, they flush down with water, and those are the kind of things that you'll actually have to ask the parents that, you know, they're asymptomatic, but when we ask them directly, uh, you. You know, what are the kind of things that the child really likes to eat, um, they will never pick anything that is sticky and they will actually say, oh, my child avoids peanut butter because it kind of gets sticky and they can't really flush it down with water. Are they, every time they take a bite of steak, they have to drink half a glass of water, you know, things like that are, um, should be actually a clue that there is some dysphagia that is happening. In in babies, sometime, another important thing to remember is that because of the esophageal obstruction, they can have pooling of saliva or secretions in the upper part of the esophagus. And then that can, you know, when they're lying down, or they can, that can continue to trickle down into the trachea and that can actually increase, uh, you know, can present as coughing spells or, um, uh, you know, even uh repeated chest infections from that reason also, um, those are more dramatic, uh, presentations, but a lot of these patients are nowadays even asymptomatic. But let's say, you know, we have some symptom, whether it's a tracheal obstruction symptom or esophageal obstruction symptoms, how do we evaluate? Well, one of the simplest things that you can do is a chest X-ray, so if I. Go to here. So a simple chest X-ray, the important things to um to look for is where the aortic knuckle is on a chest film. So you can see the air in the trachea. And the normal trachea will be slightly to the right side, and then you'll have this uh aortic knob or indentation into the column air column in the trachea on the left side. So that tells you that this is the left aortic arch. As opposed to the right aortic arch where you can see the air column in the trachea being indented uh to the uh to the left side, being pushed to the left side and the indentation is from this right-sided aortic arch. So a simple chest X-ray can kind of give you a clue that there is some arch anomaly. It doesn't give you 100% of what's happening, but it will raise your suspicion about, uh, you know, you need to look further. So, here again, you can uh look at the comparison that the normal left aortic arch is a little bit inferior, whereas the right aortic arch is a little superior, but it's still not going all the way into the neck, hence it's not a cervical arch. On the lateral view, you can see that the inferior part of the trachea is usually pretty straight. You know, you can see there's really no angle here in this portion. But if you look over in this patient with a double aortic arch, as the trachea, as the, uh sorry, the one of the arches is traveling behind the trachean esophagus, it causes an indentation and it's almost a booing of the inferior part of the trachea, uh, over here. So that's again another clue that there might be some arch anomaly does not necessarily tell you exactly what is going on, uh, but it is important to recognize that. And if you um if you are more suspicious, another thing that is commonly available is the barium esophogram, um, in the barium esophogram, you know, you're allowed, you, you let the patient swallow uh barium and. You see the esophagus, and if you have a right side a aortic arch, you'll see a rightward indentation. If you have double aortic arch, you see an indentation on both sides. Again, you can see the right arch being a little bit superior than the left arch. And then in any patient, and this, this is a patient with double aortic arch, you see a big uh indentation on the lateral view posterior to the esophagus. Similarly, in a um right aortic arter, the barren left subclavian artery patient, you see a uh a uh sorry, an indentation on the right side, but also an oblique indentation that goes leftward, uh, and superior. That's the aberran left subclavian artery as it's traveling right behind the esophagus and then goes to the left arm. Um, contrast this with the double aortic arch. The leftward indentation was inferior. This one is superior to the right aortic arch. Um, once again, if it's really just a barn less subclavian artery, you'll see a smaller posterior indentation on the lateral view, but that's not always true because, you know, if you have a large diverticular comal, it may look something like this as well. Now, you know, a lot of uh the new diagnoses are actually a fetal diagnosis, um, the asymptomatic patients, um, you know, because of the change in how the obstetric ultrasound is now being done, we're picking a lot more prenatally diagnosed, uh, vascular rings, and most of these patients actually tend to be asymptomatic. And that's where it, you know, becomes a question of when to operate on those patients, what is a good timing, um, so Elizabeth, I'll ask you, you know, about the general management of, um, vascular ring first, and then maybe you can talk about something about how to deal with patients who are asymptomatic, truly. Yeah, so that's a great point. So we're meeting these families during the pregnancy. We have an idea of them. Anatomy that is finalized, you know, after the baby is born, but we have a pretty good idea of what the type of astro ring that they have. And of course then once the baby is born, the parents are watching them and seeing how they're doing and they're able to do normal baby things, but um over time some symptoms can develop the parents are watching for. So let's maybe start with double arches since this is the one that presents the earliest with symptoms. So this again, you'll recognize this. Um, diagram from previously, but it has, um, shown here again in this case a right arch that's dominant and then a left arch and these tend to be tighter, they tend to have more symptoms earlier respiratory and esophageal symptoms. So what do we do for these patients? We're actually gonna be dividing in this area where the red line is dotted line is also dividing the ligamentum and I'll show you that on the next slide. So this is showing the surgical repair. So the ligamentum is already divided and now we're putting clamps here and we're dividing this part of the um distal left aortic arch. So this is the accessory arch and again, the baby has enough flow going the right way, the right arch to um provide it to the descending aorta. And so it showed here you can see that it provides blood, it goes up to the head and um arm on the left side and then similarly on the right side, the blood comes around here to the descending. So these patients are virtually everybody's getting repaired, usually less than a year, and we expect symptoms quite early on those patients. Um, so I'll move on to the, the next one. So we looked at this anatomy previously and this anatomy can be a little more tricky in terms of when they present us with symptoms and when they should be repaired. So again, this is the right arch. The last subclavian, the Corose diverticulum is jelly right here. And so, um, generally speaking, if you have a large cars diverticul with this less subclavian over time we see the vast majority of these patients developing symptoms. Um, often it's not until they're having the solid foods and a little bit older. And so we've, um, as a specialty been um considering, you know, when to repair these. Like we would rather not have a child have chronic, you know, tracheal and esophageal compression. We think the longer you have this, the more likely you have things, longer term consequences. And so at least in our hands, and we do do a lot of these, I think the optimal timing of repair in terms of the, you know, pain, the child's tolerance of the operation, recovery, and also the technical ability of the operation. I would say it's about 1.5 to 3 years. Again, um, this is, you know, it's easier to repair when their age is at that age, it's easier to spread the ribs and do what we need to do. The children. Remember anything about the surgery or the hospital when they're older, they heal very quickly at that age, so that's um you know a discussion obviously with the families, but um the vast majority of families are electing for repair in that uh time period and many of them by then are already having, you know, recurrent uh viral illnesses can have um slower eating and preferences to more um softer foods and things like that. But again, of course, this is a discussion with the family. It's interesting that even for patients who the parents say we're really not noticing respiratory wise when we look down with a bronchoscope during the um repair, we see about 30% compression externally. So as Yasser said, these kiddos compensate. They're used to their anatomy. They've never known anything different, so many of them are having significant compression and we just don't see clinical signs of it. Um, and then I just wanted to show this is also how the repair is done. So again, this is a right arch and left you can see the corals. So we move the subclavi to the carotid. This is an operation we do quite frequently. You are working on the carotid. we've never had any neural events or anything like that, and this makes sure that that area behind the um esophagus and behind the trachea is widely pain. Um, for older, larger, um, patients and adults, the operative repair is more complex. So to get to the carotid in the chest in an adult, so like if we go back to this model and we can zoom in on this, um. They're, uh, getting to the carotid up here. Let me see if I can get you back in the center here. All right, great. Thank you so much. So to get to the carotid up here, if you're going through the chest in an adult, that's a very deep way and a long. a way to get all the way up there. So that's very challenging. So we frequently have our vascular surgeons help us, and what they can do is put the subclavian onto the carotid in this, um, working in this area, so just above the clavicle, use my pointer. Just in here and that takes care of the subclavian um and then in older adults we're actually replacing the aorta so this part in here that's involved in the master ring because by that time you've got atherosclerotic disease, you get um your coils diverticulum is not as good tissue and so again repaired adult is is um. More challenging and it does require the heart lung machine so um that's another reason that we do feel like if they're going to have a surgery, can we do it when it's easy and um the kids won't remember it and that's relatively low risk. Surgical ease is obviously, you know, important, but from a, from a pediatric cardiologist standpoint for for for me, one of the most important thing is that um many patients who are asymptomatic early on. They develop symptoms in their teenage years and by that time the tracheal ring, you know, and uh those things that the trachea has developed under this external compression. So even after release of that external compression, their trachea is still not completely normal and that's uh and. The same thing can happen with esophagus also, um, so that's, that's the other thing that, you know, doing it early on while the child is still growing can actually have certain benefit from the uh from the developmental standpoint as well. Well, we, uh, maybe we'll pause here. We have a question. um, can you clarify what symptoms an infant might present with and what would happen if untreated? Yeah, so you know the. The in infancy, most of the patients, if they have symptoms, they're going to, um, you know, especially in the neonatal time frame, as soon as they start breathing, you'll start having noisy breathing or you can have stride or you can have, um, you know, cough and, um, you know, repeated spells where they act. Aspirated or choked and had to be seen in the emergency rooms because of these frequent choking spells because they just couldn't swallow properly. So that's, that's one of the things that they can uh they can present with but you know, from the esophageal dysmatility standpoint also, um, like I said, one of those um problems. Could be just pooling of secretions and that's one of the situations where we can encounter in in NICU where a child is repeatedly getting, you know, suction because had pulling a secretion in the upper part of the esophagus that aspirate and cough and, you know, becoming hypoxic and uh things like that are uh are developing. Although like I mentioned early on, many of those patients who are we now. Diagnosing prenatally before uh they're born, they actually don't have any symptoms and you actually have to um check for those symptoms periodically um as the child starts eating and, you know, especially solid foods and uh and table food because those are the ones that are actually going to elicit symptoms of esophageal motility if they didn't have any respiratory infections. The respiratory in you know, respiratory symptoms are. Going to bring them to attention a lot more easily than esophageal symptoms in general. Yeah, I think I also feel like I've seen, especially in the teenagers and young adults, uh, that diagnosis of GERD, which tends to go away after surgery, so they've been put on reflux, um, medication and like you said, very frequently to be diagnosed with asthma, exercise-induced asthma, and then, you know, it goes away after we have, you know, do a repair to your point. Uh, a lot of teenagers will present with just exertional dysia because again there's, you know, there is at least, you know, 30% of, um, narrowing of the trachea which at baseline. Nobody has any symptoms, but when they're pushed in high school sports and things like that, then they manifest that, oh, you know, I can't keep up with my peers. So that's another um thing that can actually present, um, in, in teenage years but again at that point, you know. It may or may not get better with the surgical repair and the same thing in the in the adults also we always counsel them about that that, you know, yes, the surgery will relieve that external compression. But it may not return to like 100% caliber and things like that. Yeah, yeah, um, maybe it'd be good to talk about some more complex rings. We've talked about kind of the most um basic and most common, but, um, something like a circumflex aorta or something like that, um, some of these more complex ones. Yeah, so circumflex aorta can occur in in combination of other arches, um, and so what the the problem in that is that. Essentially, you have a, a, a transfers the aortic arch that is more horizontal and it's going behind the uh the esophagus because the dorsal aorta, the descending aorta is on the opposite side of their dominant arch. So if you have a right aortic arch, you have a left sided descending aorta, how does it connect? It connects through an arch that's actually traveling behind the esophagus. So that's a bigger deal because even if you relieve the uh vascular ring, you still have a big vessel that's traveling behind the esophagus, so you can continue to have symptoms because of that. So in those situations, um, you, you have two options. One is, you know, you do some sort ofexi where you attach, you know, trachea or um esophagus in the opposite direction, or you do a very complex, uh, repair what we refer to as the aortic uncrossing procedure, and Elizabeth, you know better about those things than I do. Yeah, well, that's a good point. Um, aortic crossing is very uncommonly done and it's for severe symptoms and for circumflex aorta. But to just explain what, um, Yasser was describing, um, one of the important things is how if we can, sorry, if we could also zoom in on this guy. So how the spine relates to the aorta. I'll move you back in. Thank you so much. If you look here, you've got the spinal column right here. And so what Yasser is talking about is the aorta is starting on the right and on this case it ends on the right, but a circumflex aorta starts on the right and as it goes in this area behind the esophagus, behind the trachea, it then comes and ends where my finger is here. I'll put a pointer. So it that crossing point where it crosses is often very close to the coina. Right there, so you end up even if you repair the ring, you have aorta behind the carina that's going from one side to the other. So that's what defines the circumflex aorta, that's what can be tricky because even if you repair the vascular ring in a normal way, you still have that mass effect because again you have the spinal column right here and you have limited space. So, um, just to explain that part further. And in the Pixi, what exactly do you do. So if you're, um, if you look again, thank you so much, um, at where this spine is right here. So a pexi means that the aorta, we're gonna move it a little to one side or the other. So often the right arch is coming around and it's coming a little too, too far to the left and in our way. So we'll put little sutures and move theor a little bit to the right to keep it out of the way. And then they'll put um with our peach surgery team, they also put sutures of the coina or the um. Main stem bronchus and they put sutures to pull that back. So we mobilize the esophagus out of the way and then we put sutures to on the back wall of the trachea to open that up so these tracheas and the bronchus can get very narrow slit like, and those little sutures open it up more like a circle. So those would be called an aoropexy or bronchopexy. So those are like adjuncts. And for the aortic and uh uncrossing procedure, that's obviously a, a bigger procedure and you really have to weigh in the risk versus benefit in those situations, so. Uh, while the, the simpler repairs obviously are favored because, you know, they're less complex and even for moderate symptoms, it's easily justifiable, whereas an aortic and crossing procedure you have a higher risk, a bigger operation, you know, how do you decide about uh doing one versus the other? Um, there is a number of risks related to the uncrossing and um it basically brings the aorta, um. Uh, let me get you back in the view, there we go. So it brings the aorta from the right side and it brings it over to the left side, so you have to dissect the whole aorta and bring it from in the back in here, bring it around so it's like a natural left aortic arch. The problem or the challenges with that are both frontic nerves are at risk, risk, recurrent laryngeal nerve is at risk and it can be very challenging to get that aligned. Right, so you don't also have actually the esophagus taken on do turn and have actually esophageal symptoms after. So even though conceptually it sounds great, like, oh, I'm gonna recreate a normal aorta, um, the results of the risks related to are higher, so we usually are working to look at them and be really carefully, um, to see. If they're um a candidate for a regular vascular ring like plus or mus a pexi because you could put those sutures in and move the aorta some, um, and then if they're really struggling with symptoms and their anatomy is really circumplex where you see that the anatomy is not going to be amenable to a fashion ring repair with the adject, that's kind of when you have to discuss with the family on crossing I see. All right, um. It looks like um there was one other uh type of kind of sister raster ring we call it a sling. Um, do you want to go over what that anatomy is? Yeah, so you know. One of the probably the most common arch anomaly is uh where the arch is actually on the left side, but only the left subclavian artery is arising, um, you know, apparently from the descending aorta. So I have some pictures of that. So here On the CT you can see the aorta is on the left side, but then you have the left subclavian artery coming off um from which is crossing behind the trachea and esophagus. Now this is not a true vascular ring because this side, the, the left side is actually free. Um, sorry, the right side is actually free, so the arch is on the left side, the ligamentum arteriosum is also on the left side. So there's really nothing closing this complete, um, it doesn't really make it a complete ring, and this is what, you know, sometimes people refer to as incomplete ring, but you know, a sling is probably a better term to kind of describe that. Most of these are actually found incidentally for a CT done for some other reason, but you know when um when we do discover that and very uh rarely there is um there is a symptoms, it's uh. You know, it's really because of the uh these are esophageal symptoms just because of the subclavian artery crossing behind the esophagus, as you can see in the esophagram over here that the posterior indentation is um from that. That's a great description. We actually have a model of um of this vascular sling. Um, so in this case, if you look, the aorta is coming up, it's going leftward, so it's just like normal, and then if you flip it over, you can see that the subclavians, so there was subclaviants coming to the right side from the left here. And it occasionally it can cause mostly esophageal symptoms. We don't really see tracheal symptoms and that's what that light pink colors esophagus it's getting really smooshed. So as Yasser said, unusual to need an operation, you really have. to show on a barium esophogram that they're having functional dysphagia so they can't swallow um bulkier things. It's very common that they have um a globus sensation, meaning they can kind of feel food pass but it still passes fine. And then if we do um have that, then again we move the subclavian up to the carotid and then we um resect this com so they have nothing behind the esophagus. So it's a similar idea to other types of vascular rings. Well, great, I'll check if we have any questions, um. You know, while we're looking for more questions, I'll, um, I'll ask other, uh, you know, about the surgical approach. Is it always done through a lateral thoracotomy or sometimes to sternotomy? Because obviously, you know, sternotomy seems like a a bigger operation as opposed to thoracotomy. Right, um, so the bulk of these are done through thoracotomy, so like the right arch or left, the double arches are each done during uh through. it's pretty unusual actually to do sternotomy to get to these, um, in part because those structures are really posterior, so they're, you know, that you're to be in the front, but the subclavian, the KD, all the ligamentum, um, you could actually get to, but those structures are pretty far posterior, so mostly thoracotomy, which in a baby and even like, you know, a 1 year 1.5 year old, they, they have very pliable ribs. They really don't have nearly the pain either, even of an older kid, like it's so much easier on a kid when they're kind of in that age range. Yeah, yeah, yeah, what about if somebody is going to the OR um for VST repair and has to have a stenotomy, are you still able to do a vascular repair in that setting, or? That's a great question. So tetrarology of below. About a quarter of those patients have right aortic arches, so they have naturally the arch going to the right side, and a proportion of those will have a vascular ring. So it's very easy to take our ligamentum. So that does open it some, but we're really not able to get to the subclavian if they have an aorrate subclavian to address that. But you certainly can, and it doesn't really add a significant risk, divide that ligamentum to try to open up the area. Yeah, yeah, uh, you know, right, uh, right aortic archs and tetra is a good example of a, you know, because most of those will actually not constitute vascular ring with a, with a few exception, obviously, the, um, and part of that is that even though you have a right aortic arch and a barnless subclavian artery, the ductus arteriosis is actually. More anterior either coming from inominate artery or you know, typically nominate artery, you know, we have a more mostly a mirror image branching pattern and that's why most of the patients who have tetralogiafolo and a right aortic arch don't constitute a vascular ring, but a small subset does, um, so yeah, that's a great example. Similarly, you know, actually in, um, thinking of other congenital heart defects, you know, cronchus arteriosis will not have a ductus arteriosis, and you know, that can also, even though you can have right aortic ar, you can still have, you know, circumflex aorta in these uh situations that can have a mass effect in the, in the posterior esophageal area but not necessarily, uh, a true vascular ring, um, to repair. Obviously, you know, when you have a congenital heart defect. The, the repair is also more complicated. It's not just a a vascular ring, uh, where you're approaching it in the normal uh pathway. You've also seen a whole variety of things that we haven't even discussed. I feel like we've seen there's, it feels like there's almost like an infinite type. I mean, yes, these are the standard ones, these are the most common, but often you and I will be reviewing a scam that is not any of these specific bread and butter bass rings. Yeah, um, you know, actually, um, one of my, um, my interest is actually just looking at the development of how these vascular rings. Um, actually, uh, form, uh, from the embryologic standpoint and the work that Doctor Jesse Edwards, who was a pathologist at Mayo Clinic, um, is really a, um, of great interest. Um, I recently inherited his original book from his son, so I was super excited about it and, um, I was actually gonna bring it in here, but I, uh, figured, um, but I, uh, so. Doctor Jesse Edwards, like I said, he was a pathologist who actually described all the possible vascular ring that can potentially exist, um, and what he described was this model of double, you know, aortic arch and a hypothetical, um, arch that, uh, that can explain all sorts of vascular rings and he even predicted. Vascular rings that he never saw. So and we're actually seeing this now and we can explain it based on his original diagram, and that's, that's the brilliance of his um his his original diagram where he uh he explained that. And just to clear or to um some people might not be as familiar with that, but um if you could explain a little further, that's relates to the fetal development and then uh we'll start with arches on both sides if you could just so you know it's uh um it's hard to show it without the picture, but you know obviously we have um. We all kind of develop start off with two arches and the right side regresses and the left one persists and which portions of the arches, which arches uh regress, uh, is going to dictate and which one persists is going to dictate what kind of vascular, uh, anomaly, the aortic arch anomaly you're going to be, uh, be seen and that's the, that's a brilliant, and a lot of those structures that he described, um, as a primitive, you know, double arch, uh, model, um, a lot of those. Those are, uh, many of those vast destructions actually don't exist in one point in time. That's the other part of that. So you know, you, you have development or regression of persistence or regression of portions of that, but it could be different times and that's why you can have, you know, 5th persistent 5th arch, which is, you know, another interesting one where you have two arches, but both on the same side, the left or the right, um, so it's not the double arch, uh, where you have one arch on the right, one on the left. You know, persistent 5th arches usually, you know, both on the left side, and they usually don't have any implication. It's just an interesting imaging finding that we see. Sometimes you can develop a coreation where they both join together, but again, that's rare instance as well. um, another, uh, kind of vascular sling, um, that we didn't, uh, talk about is the, um, LPA sling, where the, um. Whereas the left pulmonary artery is actually taking off distal RPA instead of the main pulmonary artery. And just take a scenic route um around the trachea and it runs, that's the only anomaly or ar anomaly or vascular anomaly where it um where a vessel is actually crossing um or traveling between the trachea and esophagus. Everything else is either in the front or the back, but this is the only one that will actually travel in between trachea and esophagus. So now. If you have a barium esophogram, you can see an anterior indentation. That's the only thing that can happen, you know. I, I remember how I showing you in the barium esophogram, you see posterior indentation. It could be a multiple things, uh, it's just showing you there's a vessel crossing in the back, but if there's any vessel crossing in the front of the esophagus, it's a pulmonary vat, a pulmonary artery sling or LPA sling. And we can kind of show that for the audience, and this is not a PA sling, but if you, we do have the PAs here and so basically, you know, this is the left PA here and it would be going around, so coming off starting on the right side and coming around to then feed the lung. If that makes sense, yeah, so it's kind of takes it, like I said, uh, a scenic route goes around the trachea and that's what causes the pull on the trachea. So you have tracheal, you know, um, tracheal obstruction, stenosis, um, that's also very commonly associated with other, uh, extra cardiac, uh, cardiovascular abnormalities like complete tracheal rings and tracheal esophageal fistula. So many a time, um. More important than the fixing the LP sling is actually fixing the tracheal uh bronchi tracheal bronchial problems. You wanna comment on that? Yeah, so the tracheal rings, complete tracheal rings means instead of having um a hard part in the front of your trachea and a mousseptum in the back, they have hard cartilage all the way around and that causes stenosis. So when you do repair it, you actually have to cut out that part and reconstruct the trachea, and that's the highest risk of the operation, not the PA sling itself. So whenever you have a PA sling, you have to check for the trachea rings and that you repair that on bypass. Um, generally they're presenting quite young, um, and so usually these are repairs less than a year of age, usually in the infant. Now again, you know, that's one of those things. It kind of depends on how good our OB team is and if they uh pick it up and we can actually diagnose this on the fetal echocardiogram as well. So, um, not very common because this is again one of those ring uh lesions that can easily get mixed uh missed on the fetal lacho, uh, not because. We didn't do uh um we didn't see it, but because we didn't do a fetal echo because it really depends on the people at the front line, which is the OB um sonographers and, um, specialists who can actually refer the patient for further assessment. Um, well, I did include a couple extra slides. It looks like we have time. I don't see any more questions about adult master rings. So these are generally gone through, um, like a right doconomy because they go is on the right and so you wanna, um, replace that area and so this is showing, um, the. Excuse me, let me get that. Um, the coral's diverticulum, this is the right subclavian, um, and then um as it gets here and then um. The less of clay and carotid has already been done in the neck, um, and, uh, basically I, I didn't show it well back there, but that was, um, basically replacing the portion of the right aorta that has the corals. So that's how it's done in adults. In the um in the adult, there is also a risk for aortic dissection from the divertic right, very good point. Uh, we don't have a good sense of the, the level of risk because we don't have a denominator on these patients, but there is, um, it's thought that a certain, you know, KD size, like a, um, similar to an aorta that dilates over time that that as that. Uh, while stress gets higher than they are at risk for um dissection, so some people are coming for repair to decrease their dissection risk. That's pretty uncommon and again we don't have the denominator of that, um, to know like what percentage. Extremely rare to see, but you know, many time you also see some calcification in the diverticularlycomma or perhaps those patients are also at higher risk. Um, I just to go back to this image, so, um, you basically are gonna be replacing the portion of the aorta that includes the coral, so the ligamentum is gonna fall away. You've already taken care of your subclavian, so it ends up being an aortic, uh, replacement, and it is done with the heart lung machine. It does have a higher rate of recurrent laryngeal nerve injury, but that's, that's it, you know, if you're. 50 years old, then the tissue is not gonna be as good and you just have to replace it. In the adults, when you actually do the, you know, repair of the right aortic arch with the barnless subclavian, do you also translocate the um subclavian artery or you just do the oh I see, so but it has to be a separate procedure, OK, yeah, so it's two procedures, yeah, yeah, so that's another reason why you want to do an earlier infant surgery because it could be just one step and be done, yeah, yeah. Well, I hope we've had some good discussion here. Yes, anything else that you could think of to close? Um, is there any, I don't have any more questions. I know that we had, um, one which was great, but we don't have any more questions here, so. Well, thank you all for listening. It's been great to spend some time. Um, thank you so much, Yasser, for all your expertise and thank you for all who joined. Thank you for our crew who helped with all the 3D models of that difficult imaging. Um, we hope you have a great day and cut to us if we can be of any help.
