Coarctation of the Aorta: Repair Strategies and Outcomes

Podcast Transcript

Announcer:

Welcome to Cardiac Consult, brought to you by the Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute at Cleveland Clinic. This podcast will explore the latest innovations, medical and surgical treatments, diagnostic testing, research, technology and practice improvements.

Eric Roselli, MD:

Hi everyone, I'm Eric Roselli, the chief of adult cardiac surgery and surgical director at the Aorta Center at the Cleveland Clinic.

Patrick Vargo, MD:

And my name is Patrick Vargo, I'm also a heart and aortic surgeon within the Aortic Center and the Heart Center here at the Cleveland Clinic.

Eric Roselli, MD:

Well, Dr. Vargo and I, actually with many members of our team, but you and I probably do the most aortic operations around this place, and as a referral center for the entire world, essentially, we have the unique positioning in Cleveland to see uncommon things commonly.

Patrick Vargo, MD:

Right.

Eric Roselli, MD:

Today, we're going to be talking about a condition, a congenital aortic condition called aortic coarctation. It's actually a fairly common congenital anomaly, and something that a lot of pediatricians and congenital surgeons take care of. But the really cool thing is, as we've gotten better and better about the way we take care of heart disease in general, and as imaging has gotten more widespread, we actually find this condition in adults a lot more than we ever had and before anybody ever appreciated before.

We have a growing practice over the last several decades taking care of this problem, and I think other folks elsewhere are also are going to find this, and it being a relatively uncommon thing, it's nice for us to share with patients and providers some of our insights, and that's why we're here today. Patrick, how about just for our audience, clarifying what coarctation is?

Patrick Vargo, MD:

Yeah. Aortic coarctation, as you mentioned, is one of the cardiovascular heart and blood vessel diseases that we often find in childhood, in young infants and children. It's a narrowing of the aorta just beyond the arch, or sometimes including part of the arch as well. The oblique blood vessel that comes out of the heart and goes through the chest and gives branches to the arms and the brain and continues down into the abdomen, that's your aorta. The part just up by the head vessels, just beyond the ones that go to your brain, into your arms, there's oftentimes an area there that's made up of tissue. The cells that make up that part can sometimes constrict after a baby is born, and it causes a narrowing which can causes disruption in blood pressure in the body.

So, the blood before that narrowing, the branches that come off that go to the head and the arms can sometimes have a very high blood pressure, and then beyond that could be a narrowing, beyond the narrowing could be a lower blood pressure. You get a discrepancy there, and that can cause problems with growth and can cause symptoms. It can also cause wear and tear on the heart and the blood vessels before that narrowing, as you can imagine, the pressures are going to be quite high there.

Eric Roselli, MD:

Yeah. It's interesting, we're still trying to figure out exactly why this happens. We are doing some research in this space, and we've been looking closely at some of the specimens of cells that are harvested from there. But it really is a miracle when we form, and all of our cardiovascular structures are formed. I say this all the time, I still think it's amazing. In the first trimester, pretty much all the cardiovascular structures are formed. The aorta comes from a bunch of different cell sources. This is in one of those areas where the different cell lines come together, and something goes awry.

Sometimes even, the aorta is incomplete. It doesn't form at all. In those situations, little babies have to have surgery pretty much right away. But in some people it's open just enough to get blood flow through there and the diagnosis isn't made until adulthood.

Patrick Vargo, MD:

There are some that present later in life, because it's very much a spectrum from a completely interrupted or separated aorta to more mild narrowings that become more significant as somebody gets older.

Eric Roselli, MD:

Yeah. As you get bigger and you need more blood flow, then it becomes more apparent. We've studied this in those series of patients and gained a lot of experience. The other thing that we see is not only these people that show up later in life, sometimes a teenager, sometimes the young adults, sometimes even middle-aged folks. I don't see a lot of 80-year-olds that have never been diagnosed with a coarc, but we see folks sometimes in their 50s, even.

But we also see folks show up after they had that surgery when they were a baby. They were told they were fine, they fixed the narrowing as an isolated thing, but sometimes they can show up late.

Patrick Vargo, MD:

So, I would say as predominantly adult heart surgeons, aortic surgeons, many of the patients we see with coarctation or these narrowing issues have had some kind of previous repair. Not all, but many of them. The issues that can happen down the road are that it can re-narrow. You can also get dilations in that area, where just beyond or as part of the connection that they've fixed in childhood can become enlarged and grow, and they’re at risk for rupturing or tearing. I don't know, why do some go one way or the other?

Eric Roselli, MD:

Yeah, it's interesting. There's been this growing appreciation over the last generation, like a couple of decades now, when they do those kids and the babies, and the kids are pretty malleable, they now know the best way to do it is just cut that whole section out, stretch the aorta, and bring more normal aorta together. But sometimes that's not as easy as it sounds. Sometimes, or for a lot of times, there was a whole bunch of different ways to do it, where they would say, well, just split the narrowed area and patch it open. Or swing the neighboring vessel to the left arm, down on top of it, and use that native tissue to reconstruct it. What we've learned is that that whole ring of tissue is bad.

So, even though they can fix the narrowing by opening up one way or another, that bad tissue continues to be less than ideal. Over a lifetime, when you're 30, you've now had a billion heartbeats against it, we'll see that bad tissue can stretch into an aneurysm, or what they call a pseudo aneurysm, where maybe they sewed things together kind of starts to break down, and then that can be at risk for rupture.

Patrick Vargo, MD:

Yeah. In general, in young children or infants, there really isn't and wasn't stenting, because a stent or a synthetic tube to put in doesn't grow with the child. It would form a coarct as a kid then, or narrowing forever. When they did these repairs, they were open surgical repairs where they actually cut and remove things. As we discussed how different the anatomy can be, whether the arch is interrupted or partially narrowed, there were different solution depending on that. But they all involve cutting and sewing back together these narrowed blood vessels.

Eric Roselli, MD:

And now, as you mentioned, the stents, they also are stenting them in the kids. Sometimes they just need to get just open enough to allow the kid to get blood flow to the organs downstream, and let them grow. But as you said, those stents are kind of rigid, and so we'll see sometimes the stenting, they stretch it and balloon it, will injure the wall of the blood vessel, so they can develop again that pseudo aneurysm. I always tell patients it's like a bubble on the side of a tire, an old worn tire.

Patrick Vargo, MD:

Or a blood blister.

Eric Roselli, MD:

Or a basketball or something like that, with a blister on the side. But it can become dangerous and rupture. Anybody that might be listening to this, whether it's a physician with a patient who recognizes they had this remote history, or the patient themselves know that they had this history, they potentially have a lifelong risk, don't they?

Patrick Vargo, MD:

Yes. Just because you were diagnosed with this at one time and decided that you didn't need anything done, or maybe you did need treatment and they did treat it, doesn't mean that you can stop looking at it. I think it's important that these kinds of aortic issues are followed throughout your life. If you reach a period of stability where it looks like there's not a lot changing and blood is getting where it's supposed to go and there's no issues, then sometimes that imaging and those checkups get spread apart further.

But I don't think we can ever say, no, it's done with, and we're not going to look at it anymore. Because some of the biggest problems with these aortic issues are that when we stop looking, some of these issues develop without somebody knowing about it. Many times they're asymptomatic. These narrowings later in life for these aneurysms and blisters that can form, often are asymptomatic for a long time.

Eric Roselli, MD:

Yeah. Yeah, that's the thing about an aneurysm, you won't know it, or certainly won't feel it until it's killing you.

Patrick Vargo, MD:

Yeah, it's rupturing.

Eric Roselli, MD:

So, it's interesting, we've studied this in a couple of different series that we've looked at. The average period of time from when a patient who had had a previous coarctation to when they needed something redone was around 30 years. One study, it was 32, the other one we did, it was 29, right around 30 years. Which I always found fascinating because I went back to when I first started gathering data around these patients and trying to study it further. I went back and looked at some of the oldest series from the 60s. They had great success as they learned how to fix this problem, but the average lifespan of those patients was around 32 years.

Now you can have a normal lifespan. But I thought it was really interesting. They didn't have CAT scan or anything like this. These patients were dying when they were around 32 years old, and now we find we're re-operating on them when they're 32 years later.

So, I wonder if some of those deaths could have been attributed to things that we know now how to handle very well. Certainly, open surgery is always an option, but you mentioned the endovascular. Can you talk a little more about that?

Patrick Vargo, MD:

Yeah, so as the years have progressed since the 60s and things, and they first started fixing these, so has technology and the way we treat these. One big area of growth in how we treat aortic and cardiovascular disease in general is stenting and what we call endovascular or minimally invasive therapies, where we can avoid a big incision oftentimes and access the blood vessels from the groin with a puncture. Over that, and over a wire we advance stents in. Stents, I always describe them as kind of a new inner tube or a relining of a damaged blood vessel.

So, the outside of the blood vessel may be either blistering or narrowed, but then you feed inside of it a new tube that expands and relines the inside of it. It offers protection and sealing over those blistered damaged areas. And if it's narrowed, it affords us the opportunity to put a balloon and stretch it open with protection, that if there is some tearing or some blistering while you balloon it, the stent has relined it.

I think that's the way that we're tackling a lot of these late issues now. Or even sometimes those patients that present for the first time in young adulthood or middle age, that haven't had previous repair, this often becomes the first line therapy then.

Eric Roselli, MD:

Yeah, absolutely. We now have a couple more devices available to us to do that safer. There are these, what they call balloon-expandable or rigid stents, that allow us to really push more force on it. Those are covered now. They protect from that focal damage to the blood vessels. With the stent grafts, which are a little bit longer, we can get to healthier vessels, even getting into the arch where the branch vessels come on.

Patrick Vargo, MD:

Right. A big limitation early on was that you couldn't cover those branch vessels without having to reroute blood flow. But some of these new stent grafts have branches built into them. They offer us a little more flexibility and allow us to treat a wider range of those anatomical variants, those differences between one person's aorta and another's, the variations where the branches come off.

Eric Roselli, MD:

Yeah, it's pretty cool. I've been doing that for this disease for quite some time, and I always have my patients come back for a couple of follow-up CAT scans. When it looks great, I say, "Well, come back and see me for a five-year anniversary." Well, now I've had a couple come back for 10-year and 15-year anniversaries.

And it's the coolest, they still look great. I think the principles of getting the healthy tissue, whether that's doing an open surgery in an infant, where you stretch that aorta up and get healthy to healthy, or if it's with these endovascular techniques in an older patient, who's had maybe even a couple of operations, and we get a branch device in and we get to healthy tissue on either end, seems to have demonstrated some durability. It's been really great.

I guess there's one other thing we should talk about if we're talking about aortic coarctation, in our young adult population, particularly, and that is bicuspid aortic valve. Those things go hand-in-hand, don't they?

Patrick Vargo, MD:

Right. I think it's half the people with a coarct will oftentimes have a bicuspid valve, and that's important for different reasons. One is that bicuspid valves in general are associated with a higher incidence, a greater percentage of people with bicuspid valve have aneurysms than do people without bicuspid valves.

Eric Roselli, MD:

So, just real quick. Bicuspid aortic valve, it's a malformed aortic valve.

Patrick Vargo, MD:

Right. The most common aortic valve that most people have is a three-leaflet valve, flapper valve, there's three leaflets. In one to two percent of people, they're born with only two flapper valves, and that's called a bicuspid valve, meaning two cusps. It oftentimes functions fine for a very long time, but we know they can wear out a little bit earlier. They can become leaky, or they can become narrowed a little sooner than a three-leaflet valve. Then also, about a third, or maybe even a half of them have a larger than normal aorta, usually in the part that's by the heart, the ascending aorta. Patients with coarctation often have this bicuspid variant. That carries with it for an aneurysm or aortic dilation in that part by the heart, the ascending aorta.

Eric Roselli, MD:

Yeah, it's critical because if you have either or both of these conditions, you have a dedicated team to take care of it. Although, yeah, it's sweet when we can just do a puncture in the groin and pop something open with a stent, some patients have a constellation of these things going on. We also now have several different treatment options. We can treat it all at the same time. Sometimes it's a bypass in one portion of the aorta. It's another to treat the coarctation while we're repairing a valve and reconstructing an aneurysm, what we call a hybrid operation, where maybe we'll put a stent in and do open surgery at the same time. The great thing is we can do a lot of that really safely, and I think give people a much longer life.

Patrick Vargo, MD:

Yeah, I think it's very important. There's a lot of variability among the way these things present, what they go along with, and you got to have a very expert heart team. I think that's what we have here between the different surgeons and cardiology team members to really follow patients over their entire life and monitor stable situations, but also if things change, what's the safest and least invasive way to address them?

Eric Roselli, MD:

I like that. I guess I'll wrap it up because I like what you said about monitoring for life. I think that should be the take home message to people with congenital heart disease, cardiovascular diseases is sometimes you get a diagnosis like that, that you hadn't thought about, or maybe you hear about something you hadn't really thought about for decades, you don't have to be afraid of it. Reach out to teams that can help you with the knowledge that you need, and there are many great treatment options. Lifelong care is what we need to provide. We can take care of your heart and make it last as long as possible, and still, especially with newer techniques, do this in a way that we maintain the quality of a patient's life over a long period of time as well.

So, this has been great talking about aortic coarctation I think, again, it's one of these things we're going to continue to see more of. Fortunately, in Cleveland we see these less common things quite often, or often enough to have a sense about it. We recently pulled together about a 25-year series of 145 patients. What do you think is an important message for healthcare providers about taking care of those folks?

Patrick Vargo, MD:

I think one of the important things that we've seen over those patients is that many of them were re-narrowings of the coarct, about 60% of them or so were folks that had a narrowing of the aorta, and many of them were re-narrowings of the aorta. I think an important message in all of them, as well as the aneurysms and pseudo-aneurysms that form after repair, is that these patients need to continue to be followed. We see a lot of them here, but still over 25 years, we treated 145 adult coarcts, still a relatively, I don't know if rare is the right word, but uncommon patient group. So, I think it's important that they have contact with specialty centers that see a lot of these because there's a great variability from patient to patient.

I think another interesting thing that we saw in the study that you led was that there's great variation in how these patients sometimes get treated again. Meaning that there wasn't a one-size-fits-all approach if they did have an issue, I don't know if you want to talk a little bit about what we found.

Eric Roselli, MD:

Yeah, I talk about tailoring the therapy to the disease, and that's the cool thing about having a lot of different treatment options. I think over half of the patients were still treated endovascularly in that series, which is great. I remember early on, there was a lot of concern, are we going to be able to get that relatively bulky device across the tight, narrowing?

It responds pretty well. It's very rare that we can't get an endovascular device across a coarctation and get to some healthy aorta, but sometimes that's not the right thing to do. If they have concomitant aortic valve disease or an ascending aneurysm, we can do an ascending to descending bypass through the same kind of incision, and that's really well tolerated. We demonstrated some good durability of those. Then every once in a while there's someone with really wild anatomy, that's had maybe a couple of different reconstructions, and the imaging that we have available to us, whether we print it in 3D or we have the readily accessible software that allows us to really manipulate those 3D images, it's been cool and fun to get creative with the plan, and know that you can trust it.

Patrick Vargo, MD:

Right.

Eric Roselli, MD:

So, we've seen many of these where we stage it out, we do some bypass, sometimes they have a subclavian aneurysm associated with it, and we'll have our vascular colleagues help us with some vertebral artery reconstruction or something. I think that the disease, even though coarctation itself sounds like a very focal, isolated issue, it's not, especially when you see it in adults. It's important to get the input of an entire aortic team's expertise to develop a plan.

Patrick Vargo, MD:

Yeah, and I think all this planning with the different team members, the better imaging for really complicated cases, the 3D printing and modeling, it's part of what led to the excellent outcomes we saw as well. Even though it's a very complicated treatment algorithm sometimes, we were able to do it safely. The risk for death and for complications thereafter was all very, very low.

Eric Roselli, MD:

Yeah. Less than 2%.

Patrick Vargo, MD:

Less than 2%. I think that was very encouraging, that even though these weren't straightforward all the time to fix and to treat, and that they were often re-repairs, we could do it with safety and good outcomes for the patients.

Eric Roselli, MD:

Yeah. I think one other thing that’s important to talk about too, especially to our physician audience, is late complications of aortic coarctation are related to hypertension, atherosclerosis and cardiovascular disease, and although the dedicated balloon expandable stents are nice, it was interesting to look over time. Sometimes the treatment goals went from completely eliminating gradients to tolerating something in the teens. In our series, we saw the median gradient go from high 20s to, I think it was three.

Patrick Vargo, MD:

Most people did it when it was three.

Eric Roselli, MD:

Yeah, I think if we embrace all the different treatment options, we can not only do it safely, but we can achieve something that's really effective for folks.

Eric Roselli, MD:

Well, this was great. Thanks for listening to Cardiac Consult. Patrick is always, it's good to chat about disease. Usually, it's us sharing one patient at a time, but it's fun when we pull together a series, and we can parse it out and see if there's something we can learn by looking at the data in different ways as well.

Patrick Vargo, MD:

Yeah, it's terrific. Yeah, I think this is excellent, it's always great to share these conversations.

Eric Roselli, MD:

Right on. Thanks.

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