CLE Care of Aortic Disease: Arch, Descending and Congenital Aorta

Podcast Transcript

Announcer:

Welcome to Cleveland Clinic Cardiac Consult, brought to you by the Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute at Cleveland Clinic.

Kenneth Zahka, MD:

Our next speaker is Dr. Joanna Ghobrial. She is the co-director of the Adult Congenital Heart Disease Clinic. She will be talking to us about the preoperative phase, diagnosis, optimizing medical therapy and when to intervene.

Joanna Ghobrial, MD:

Thank you, Dr. Zahka. Thank you, Dr. Roselli. When we talk of congenital arch disease, we're going to be mostly talking about aortic coarctation. That's that posterior wall infolding into the aortic lumen. Was actually initially described by Giovanni Battista Morgagni back in the 1700s. It can range anything from simple coarctation, to aortic arch hypoplasia, to an interrupted aortic arch.

Joanna Ghobrial, MD:

It can be an isolated defect or it can be part of other intracardiac or extracardiac complex lesions. You can have it as part of Williams Syndrome, Noonan Syndrome, or Turner Syndrome. You can also have it as part of a complex lesion such as hypoplastic left heart syndrome and TGA and Shone's or it can be strongly associated with bicuspid aortic valve and VSDs. Prevalence is about three to four per 10,000 live births and it counts for about 5 to 9 percent of congenital cardiac anomalies. It is more common in men than women up to a ratio of two to one. It's the abnormal development of the embryologic left fourth and six aortic arches. And there's two theories of why coarctation develops. One is the ductal theory where you have this aberrant ductal remnant that goes to the aorta and causes that constriction and coarctation.

There's also the hemodynamic theory where we have this intrauterine alteration of blood flow that causes the coarctation, which can partly explain more the association with hypoplastic left heart syndrome and aortic stenosis. Now it can be sporadic or it can be genetic. And this is from a paper from the Journal of American Heart Association from this year demonstrating all the potential culprit genes in aortic coarctation and how it can overlap with aortic valve disease and HLHS as well. And the more commonly known one is the NOTCH1 mutation.

Joanna Ghobrial, MD:

But importantly from this slide what I want to emphasize is with aortic coarctation you have an associated aortopathy, you have abnormal aortic vascular tissue. Now that can present in infancy where you have severe heart failure, but you can have other forms that can be undetected until older adulthood. Symptoms that can come to you in clinic with angina, headaches, nose bleeds, dizziness. They can also have abdominal angina and claudication.

Joanna Ghobrial, MD:

In the adult population you will see left ventricular dysfunction, aortic rupture dissection and aneurysms, infective endocarditis or endarteritis. You can have intracranial hemorrhages obviously because they do have berry aneurysms and you can have premature coronary artery disease. Now, while we can encounter the native aortic coarctation in clinic, the most common thing is we will see the patients after they've had their surgical repair, with either recurring coarctation or a good repair.

And some of the repairs include A, here is an interposition graft. B is an end-to-end anastomosis. C is an patch aortoplasty. D is a subclavian flap repair and E is an ascending to descending jump graft. When you see them in clinic you can have upper body hypertension. It's important to actually check blood pressure in all four extremities.

Joanna Ghobrial, MD:

You can have a brachial ephemeral delay, a collateral murmurs, oftentimes heard on the back, allowed a two and an injection murmur. By ECG, you can have LVH. By chest X-ray, there's the classic double contour figure three and rib notching that we don't always quite see. Now importantly on echo, you do want to get a good echo assessment and that's the dopplering of the aortic arch. You'll have turbulence of flow. Let me go back one second here. You have the turbulence of flow and you'll have the classic diastolic tail. Your peak velocity can be more than three meters a second, and the diastolic velocity can actually exceed one meter a second and you can have a ratio greater than 0.5. But importantly for guideline indication, your mean grading will be greater than 20 millimeter mercury. And this here is just demonstrating that CW doppler of the aortic arch with that classic diastolic tail. And clearly, you have a velocity that's greater than four meters a second. Also, you can have a doppler of the abdominal aorta. You have continuous flow and you have a blunted systolic flow. This right here demonstrates that abdominal aortic flow that's continuous in addition to that very blunted systolic flow in here. With collaterals, obviously the grading by echo is no longer reliable. And if you do have concomitant aortic stenosis, you do want to use the expanded Bernoulli equation.

Joanna Ghobrial, MD:

Now, MR and CT is obviously best in adults. It gives you far better anatomical delineation than by echo. And cardiac cath is obviously extremely important, it gives you hemodynamics, but it also gives you the chance to actually intervene on these patients should you need to. Now it is important to actually do ambulatory blood pressure monitoring in these patients. And if they are hypertensive, you treat them and you treat them aggressively. That is if they don't have significant re-coarctation, because that is a surgical or interventional therapy. And the mainstay of therapy is going to be your beta blockers, ACE inhibitors and ARBs, especially that they have this associated aortopathy as well. You do want to evaluate for exercise-induced hypertension, but importantly, if they have coarctation or re-coarctation, this is an interventional and surgical therapy.

Joanna Ghobrial, MD:

When do you intervene? By ACC/AHA guidelines from 2018, a significant coarctation or re-coarctation is if you have hypertension despite medications, whether resting or ambulatory. If you have upper to lower extremity gradient of more than 20 millimeter mercury or an echo mean gradient, more than 20 millimeter mercury. Now, if you have either collaterals or LV dysfunction or aortic regurgitation, that mean gradient now needs to be just greater than 10 millimeter mercury. And if you have clear anatomic evidence of coarctation, timing of intervention is a class one indication if you have significant coarctation or re-coarctation. The only time we ever balloon honestly is if we don't have any other options but ballooning. There's a lot of varying anatomy. This here is aortic arch hypoplasia. This is a patient that had both coarctation and a PD, and you see the pulmonary artery light up with contrast here. This here is a patient with an interposition graft that was heavily calcified. And here is a patient with severe near interrupted aortic coarctation.

Joanna Ghobrial, MD:

Now with catheter-based techniques, we first started with the angioplasty that was pioneered by Dr. Locke in 1983. However, we did realize that you have a lot of elastic recoil and you have re-coarctation in these patients. You also develop aneurysm formations, you can have tears and dissections. Then we moved to uncovered stenting. In the 1990s is when we started doing that and that was great. However, you fix the recoil recoarctation issue, but you still have the aneurysm formation risk with the uncovered stents.

Joanna Ghobrial, MD:

And then we did the post-trial with covered stenting, which is really meant to tackle the aneurysm issue. And that is created, does tackle aneurysm problems at the site of the covered stenting but not elsewhere. We do have good outcomes with the transcatheter techniques, but there are several limitations that should be mentioned. One, there's obviously complication issues when you put a stent in a stiff aorta, this is a patient that had a stent with a descending aortic dissection. The location of the head and neck vessels is extremely important. Sometimes it's extremely difficult to put a stent where you have a subclavian or carotid artery takeoff, especially if you want to use a cover stent if there's high risk of dissection or tear or aneurysm. If you have arch hypoplasia, that's a controversial issue whether you should be even treating that with stents or surgically. Preferably surgery for sure. And at the end of the day, I am leaving stiff metal in the aorta, which is supposed to be a compliant vessel. And that is the problem. And that's where we do need more advances to, for example, develop bioabsorbable stents for example. Or come up with something else that brings that aortic tissue closer to what it's supposed to be.

Joanna Ghobrial, MD:

And aneurysms and pseudoaneurysms can develop anywhere proximal or distal to that stent placement. Importantly, we do have a lacking in long-term outcome data in that patient population. There is some data that shows that LV remodeling is actually better in the surgical arm relative to the transcatheter arm at the five-year mark or so. But again, it's a smaller study and we still need longer term outcomes.

Three decades ago, these patients who were untreated would only live to their mid-30s. Now they live a lot longer. However, this is crucial, is there is a high incidence of late complications requiring very careful surveillance. If they've had surgery when they're young, does not mean they're fixed forever. You need very close monitoring of that patient population. And post-repair, you want to monitor for arterial hypertension and treat it well. They can have coronary artery disease, systolic and diastolic LV dysfunction, cerebral aneurysms such as this and endarteritis. You can have aneurysm formation and you can have re-coarctation. Or like the case that Tiffany presented, you can have both re-coarctation and aneurysm formation. And that's usually when we want Dr. Rosselli to come to the rescue. Thank you.

Kenneth Zahka, MD:

Thank you. Our next speaker is Eric Rosselli, cardiac surgery director, Aortic Center Chief of Adult Cardiac Surgery, intraoperative surgical interventional perspective, open versus endo versus hybrid, Dr. Rosselli.

Eric Roselli, MD:

Thanks. Joanna, I like that lead in. That was pretty great. Congenital aortic disease and area where we're seeing continued growth. Disclosures and some off-label and investigation devices may be discussed here. I think when we look at older patients with congenital aortic disease, we need to appreciate that they present differently.

Eric Roselli, MD:

And I'm going to share with you several very interesting cases during my talk. One of which was taken care of recently, a 34-year-old woman from Afghanistan, who was basically born with looks like no thoracic descending aorta at all. But as I've told my patients with inheritable aortic diseases, you are not your genetic code. As we heard from many of the speakers. I think Adam's point was clear that every person is an individual. And so we have to look at these patients with congenital aortic disease as individuals and tailor our therapy to every single patient that we see. And as our treatment therapies broaden, we can become more precise in delivering precision care for our patients. This lady underwent an open repair. We did an inner position reconstruction and gave her that descending aorta that she wasn't born with off the shelf. And she's done great with this. You can see a picture intraoperatively on the left. We're able to do this through rather two rather small incisions considering how much we had to do. And she recovered beautifully.

Eric Roselli, MD:

As we just heard from Dr. Ghobrial, the mean age of death after repair co-arcs used to be 38 years old. This was data from Dr. Magoon's series out of the Mayo Clinic. But the cause of death were often related. Hypertension, early coronary artery disease, a significant portion of those patients required reoperations. I think the number's probably larger now as we understand how to monitor these patients longer. And we went back and looked at our experience in adults and adolescents undergoing various operations for aortic coarctations or associated late complications. I published this a few years back. And we had a series of 110 patients. Interestingly, the mean age was 38 years. Pretty wild. Twenty percent of them had previous cardiac surgery. Almost half of them had a bicuspid aortic valve. And we've done about 100 cases since then. We'll be updating this series soon.

Eric Roselli, MD:

One of the things that we saw was that there was various indications for the operation. Surprisingly, a large proportion of them had newly diagnosed coarctations. Like that lady I showed you, it's pretty amazing how someone can survive with a severely coarcted aorta well into their adulthood. They look normal and they survived normally, but they have those late complications that need to be treated. Many of them have recurring coarctations and a lot of them had aneurysm or pseudoaneurysm associated with, like some of the cases that we saw before.

Eric Roselli, MD:

Our technical success was excellent. Mortality was low. We had only a couple of minor complications. The respiratory failure recovered and then dialysis was temporary in those couple of situations. Those are complex cases. And we were able to reduce the gradient to single digits in all those patients. And so far, we've had no late ruptures and we were able to take those aneurysms and reduce the diameter.

Some of these were treated endovascularly. And you can see survival's pretty good in that initial series where we have follow-up up to about eight years. Let me show you a couple of examples of some interesting cases. Some of these patients, we just treat them with a TEVAR. We get percutaneous access. I prefer to use stent grafts in a lot of these adult patients. You do have actually a compliant device. You've got some fabric in there. So when you dilate it and you stretch it or you potentially fracture part of that aorta, it's covered and it's protected and you can do it in one setting.

You can get it all way expanded to the point where you reduce the gradient down to less than 10 millimeters, which is what our target should be. But some of these patients are more complex, but we can use some of these newer techniques. This patient was able to be treated with a TEVAR into her arch where there was a decent landing zone.

Eric Roselli, MD:

We were able to use a big enough device, a 22 millimeter device. And then we went from the left arm. And instead of doing a crowd subclavian bypass, we put a stent in and we were able to laser fenestrate that and create a branched endograft repair for her and we eliminated the gradient across her coarctation. Some of these post-coarctation aneurysms are more complex. We did see very interesting case to start off the talk and several examples from Dr. Ghobrial. This is one that was repaired with open surgery. The one in the middle, she was in labor for almost 20 hours with that aneurysm and nobody knew about it. And I don't know how it didn't rupture in her, but it didn't. And thank God, she came to us afterward and we were able to fix it. There's a lot of different options though, not just endo and open. But we prefer to use the endo procedures if we think that it meets the criteria for endo procedures. That is having good landing zones, treating the coarctation, sealing off the aneurysm. And some of the techniques that we learn and some of the special considerations in this patient population is that they might have small access from their groins. A lot of times, their lower aorta is underdeveloped because it hasn't been exposed to the usual flow. If there's difficulty crossing a really tight lesion, getting access from above can be really helpful. Getting through and through wire access to help cross also helps. Sometimes you can pre-dilate that to get the device through there. Most of the time, we have to cover the left subclavian artery. And be prepared to do so. I had one case where we stopped a little short and we ran into trouble in that patient and then we had to extend that repair. But remember that our goal with the coarcts is to really achieve that gradient less than 10. And we shouldn't compromise that if we're going to choose an endo solution because you can certainly get that every time with an open repair. And these are young people who can tolerate an open repair most of the time.

Eric Roselli, MD:

We want to choose our devices carefully. We want to limit the amount of over-sizing. We are landing a normal aorta above and below these things. Usually 10 or 15 centimeters is enough. And so, the risk of spinal cord injury is very low. And we do want to again, post-dilate generously. You got to be careful though, if it's a stenosis in an old graft, you're not going to be able to dilate graft material very much. Those patients might need another solution. Some patients, and we heard this from Dr. Ghobrial, especially those with a hypoplastic aortic arch, we need to think of something else because putting a stent graft into a tiny arch is still going to leave them with a narrow aorta. Here's one patient. He had a coarct repair when he was three months old, a BSD repair when he is three years old. Another coarct repair when he was seven years old. And then, forgot about it for a while because he didn't have regular follow up. But got in a car accident and they found this big pseudoaneurysm in the aorta. Whether it was there or not or was related to the accident, nobody knows, but his arch was 15 millimeters.

Fortunately, his ascending aorta and aortic valve were normal, but he had these huge intercostal collateral arteries. Going into that left chest again would've been a pretty bloody mess and we probably wouldn't have been very nice to that left lung. We went through sternotomy with the hybrid strategy. We opened the aorta, we used circulatory arrest. And we did a frozen elephant trunk repair through this. Even though the arch was hypoplastic, I was able to patch that hypoplastic arch. You can see the green arrow's pointing to the patch. We did an augmentation of the aortic arch. And then the left subclavian artery was addressed with a supraclavicular bypass just above the sternoclavicular joint. And this is what that repair looks like. And this has been durable. This is an old case of mine.

Eric Roselli, MD:

Some patients present with recurrent coarctation, like this 50-year-old. And some of these can be really complicated. This patient had a patch repair at the age of eight, left subclavian to descending bypass at the age of 18. And now presented with severe aortic insufficiency combined with aortic stenosis. The patient had a bicuspid aortic valve and there was a 20 millimeter gradient across their aorta.

Well, nice thing about the sternotomy and having all of the sort of techniques at hand is we can address both the proximal disease, the valve, and the ascending aorta. And also address that that residual coarctation distally. There's no aneurysm associated with that by doing ascending to descending bypass. And that can be done quite readily. And those ascending-descending bypasses are very durable.

Eric Roselli, MD:

Some patients require a little more thought. This patient who showed up, 56 years old, had a repair at the age of six with an interposition graft that was small. And then a redo coarct repair at the age of 25 that was based off of the left subclavian artery. That left subclavian artery bypass leaked both at the proximal and distal anastomosis. And left with this huge pseudoaneurysm and yet, he also had some aortic stenosis across his bicuspid valve. Not horrible but bad enough with a main gradient of 35. We didn't want to ignore that. We came up with a solution. Again, we were able to fix this in a hybrid way, addressing it all at the same time. We did an aortic valve replacement, we did an ascending to descending bypass. We used an EVAR iliac limb to bypass in the left subclavian artery via the ascending aorta to seal off that pseudoaneurysm where it was being fed from the left subclavian artery. And then we did a TEVAR from the groin. Did all this in the hybrid operating room to seal off the area where it was leaking from the bottom of that bypass. And you can see the post-op image on the right looks great. This was done several years ago. What I don't have is an updated photo because he just came back in about a month or two ago. And there was, the device in the left subclavian artery had migrated a little bit. The cool thing is Patrick Vargo was able to address that by adding an additional plug delivered from the patient's arm, sealed everything off and he's doing great. We just got a follow-up CT scan on a few weeks ago.

Eric Roselli, MD:

Even super complicated things like this where you come up with a creative solution, they need regular follow up, we got to keep track of it. He's doing great and his aortic valve's still working well. Not sure how we're going to do a TAVR valve and valve in this guy, but we'd probably go transapical or left carotid or something like that when his bioprosthesis wears out.

Eric Roselli, MD:

This is a 45-year-old lady who had something a little different, not a routine coarct. We've been talking a lot about coarctations, but had a right-sided arch with mirror image vessels. She had more of an interrupted aortic arch that was repaired as a child. And then she came to us with a dissection and a pseudo coarctation in her descending aorta. And a right-sided descending aorta and some moderate coarctation. Again, based on the last case I told you, we spent some time here scratched our head and looked at all of our tools in our box. And we came up with a hybrid redo repair. Again, she underwent an ascending to descending bypass to address that distal coarctation. We plugged the old bypass inner arch that was feeding the pseudo aneurysm. We ligated her interrupted arch to make it completely interrupted because it was also feeding that dissected segment, but just beyond the right vertebral artery so we didn't compromise that at all. And again, I've seen her recently and she's got a really nice reconstruction. We also bypassed the right subclavian artery off of that bypass in her chest. And I don't know what you call her operation, we just call it by her name.

Eric Roselli, MD:

But we also treat some of these other vascular arch anomalies. Something we see quite frequently is chumorals diverticulum. Which can either be related to a right arch with an aberrant left subclavian artery, like you see in this patient, where we did a hybrid reconstruction through a sternotomy. We transected the ligamentum arteriosum and delivered a frozen elephant trunk to seal off that very aneurysmal chumorals for her, as well as a bypass to that left subclavian artery. But more recently when we've seen several of the patients with this, we've been doing a small thoracotomy in the right axilla.

And after a carotid transposition, Dr. Rouse and Dr. Tong and I have a couple patients like this now. We've been able to just transpose that aberrant artery and completely resect that diverticulum quite satisfactorily without leaving any prosthesis in this patient. Again, we got to tailor our approach and think about all the different tools that we have at hand and our multidisciplinary team to each patient that we see.

Eric Roselli, MD:

And then, one other area, which be curious to hear from Doctors Najm and Karamlou, their thoughts about this, but one other area where we're seeing more and more complex patients are the patients with hypoplastic left heart and other congenital diseases where their aorta is part of the problem. This particular patient in 1989 was born with the hypoplastic heart. On day three underwent a Norwood stage one. And then at six months of age had a stage two. And then six years had a stage three.

And then, when he was 15 years old, he had a heart transplant. And then nine years later, his arch ruptured, he developed an aneurysm in his aortic arch. And a surgeon in Arkansas did an unbelievably heroic operation to save this young man's life and his heart. Protected his transplanted heart, but did it with a patch reconstruction. And then he came to us with that arch still aneurysmal and growing. And it grew a centimeter in a six-month period of time. Fortunately, although he ran out of finances, the Cleveland Clinic it was pretty easy for us to go through the processes to get him here and help this young man. And we did the be-safer operation. Our branch sent anastomosis frozen elephant trunk on this guy, through a six time sternotomy. And he's quite a remarkable young guy. I saw him recently with a virtual visit. And he's doing really well and his aorta looks great. And again, a pretty cool save. And I suspect we'll see more and more of those complex problems.

Eric Roselli, MD:

To summarize, I would say that congenital aortic disease, and especially in adults and adolescents, is something that we are going to see more of. The indications and presentations certainly are diverse and we need to be creative about how to handle it. We need to tailor those approaches and understand things about the anatomy like the hypoplastic arch. And appreciate that some branch aneurysms can be quite common as well. And understand that they have valve disease and cardiac disease as well.

But the multi-modality approach, where we use endo, hybrid and open surgery in combination is safe and effective. And all of these patients require lifelong surveillance. Thank you very much.

Kenneth Zahka, MD:

Thank you, Roselli. Please tell me those patients came from across the world and not from Cleveland as a remnant of surgery back in the old days.

Eric Roselli, MD:

The world is long. The world is long. Yeah.

Kenneth Zahka, MD:

All right. Our next speaker is Dr. Margaret Fuchs, who is also one of our adult congenital heart specialists in the HVTI. And she'll be speaking about post-operative phase lifestyle recommendations and providing lifelong care and surveillance. Dr. Fuchs.

Margaret Fuchs, MD:

Thanks Dr. Zahka. Hi, everyone. We're going to be talking about these complicated patients that we've been hearing about from Dr. Ghobrial and Dr. Roselli. And talking about how to pick up the pieces after we manage all of their complex aortic anatomy and how to follow them lifelong. We'll be talking about the following management principles. Imaging surveillance over time, managing their ongoing medical comorbidities, discussing lifestyle concerns. And finally importantly, exercise and sports participation. Imaging surveillance for these patients, of course, depends on what management strategy we opted to pursue. If the patient has a TEVAR, such as Dr. Roselli demonstrated for us, the vascular surgery guidelines tell us that a CT scan at one month and 12 months is indicated. And sometimes at six months as well, depending on how the one-month scan did. And of course, the clinical specificities of that case.

If the patient has either a stent or surgical repair, we get cross-sectional imaging, often CT and/or MR at the one-year mark. And then depending on how they're doing, sometimes we can just space that out to every three to five years. Again, we've seen a whole gamut of the complexity of these patients. Really depends specifically on how they were managed.

Margaret Fuchs, MD:

The role of echocardiography is an important one. Of course, we know that in most adult patients, the descending aorta is not going to be seen real well due to patient size and some challenges with imaging. But as we've seen, these patients have a number of other structural congenital conditions that oftentimes go along with their coarctation. And so, there's a lot of other things for us to keep an eye on, even if we can't see their descending aorta real wall with echo. We're following valve disease. Again, 50 percent of these patients will have a bicuspid aortic valve. Some of them will have Shone's complex, a lot of the other congenital conditions that Dr. Ghobrial elaborated on. We're following things like bicuspid aortic valve function, ascending aortic size, et cetera. Detecting re-coarctation using hemodynamic assessment at the time of the echo. And finally, following both LV hypertrophy, as well as diastolic dysfunction, which can functionally become a big issue for these patients as they go along in age throughout their life.

Margaret Fuchs, MD:

Managing comorbidities, as everyone has alluded to, is a huge part of taking care of adult congenital heart disease patients. And in particular coarctation patients, blood pressure management is really the key issue. And I think is sometimes underappreciated by some of the folks that refer to us. And so, really picking up the pieces and making sure that all of the other medical things going on with the patient are optimal is really important to give them the best quality of life lifelong. We know, again, that these patients have increased incidence of hypertension. And that is even if their obstruction and their coarctation is relieved. They can have a wide open aorta and still develop hypertension overall lifelong, that needs to be detected and managed. And so again, as Dr. Ghobrial said, we use ambulatory blood pressure monitoring as well as exercise testing to detect hypertension that might not be appreciable just on a resting echo and a resting exam.

Margaret Fuchs, MD:

And a reminder to everyone that there are peak blood pressures that have been devised for men and for women stratified by age that we can use when send our patients to exercise testing. Keeping an eye on how old is our patient, what do we expect their top blood pressure to be, and what's outside of the norm is really important to detect in these patients. And again, also coronary artery disease happens in these patients at a higher rate than the general population. Premature CAD happens a lot of times in coarctation patients. Traditionally, it's been understood to be perhaps just part of the coarctation, part of the vascular disease. More recent studies have also suggested that perhaps a greater player is just the fact that coarctation patients also tend to have a lot of these other coincident conditions. Again, such as hypertension, hyperlipidemia, and the male predominance of coarctation all probably play a role in the reasons that these patients develop CAD at earlier ages in the general population. And so, things like lipids and diet and exercise and all the general cardiology stuff are actually really important in this patient population. And again, can preserve their quality of life lifelong.

Margaret Fuchs, MD:

The other important feature that Dr. Ghobrial mentioned is intracranial aneurysm screening. There's been a longstanding demonstration of the association between aortic coarctation and intercerebral aneurysm. Prior studies have suggested that the incidence in coarctation patients is upwards as 10%, which is a fivefold increased risk above the general population. Some more recent studies suggest that perhaps that incidence is decreasing over time. And my hope is that it's because we're more aggressively targeting our patients' risk factors, but we also know that this happens over time. If you scan a bunch of 20-year-olds with coarctation, you're not going to find too many aneurysms. But if you're sampling an age group of 40s or 50-year-old patients, likely you'll find more. And so, probably we need to keep this on our radar and look periodically to be sure that we're not missing a intracerebral disease.

The current ACHD guidelines give a class two B indication to screen with either an MRA or a CTA. And again, there's not a lot of data for whether or not serial screening is appropriate. I think a lot of us tend to do it about every 10 years or so. Again, especially as our patients age throughout their lifetime.

Margaret Fuchs, MD:

Lifestyle is a huge question for patients when they come to visit with us and is therefore, a really important thing for us to discuss with our patients. The goal is to help them form good health habits to support a healthy vascular system. And so again, habits that promote blood pressure control are really important. Low fat diet, low salt diet, achieving and maintaining ideal body weight. All of these standard things that we take for granted are again, really important in this group of patients. Avoiding tobacco products, importance of medication compliance. Why am I taking this blood pressure medicine? I feel the same with or without it. Well, let's talk about that. You have coarctation. These are the risks lifelong. And so those things, I think the more that the patient understands, the more that they're able to advocate for themselves and take good care of themselves.

Margaret Fuchs, MD:

Regular follow-up with physicians is critical. As Dr. Roselli showed, there's a number of patients who have an intervention as childhood and then fall off the radar. And that's part and parcel with what ACHD does is help keep these patients with us, bring them back to the fold, make sure that we're doing all the other things that need to be done to take care of this population. The ACHD guidelines in 2018, spell out the optimal follow-up for patients with coarctation. This stage A group is the best of the best coarctation patients who have no residual aneurysm, who do not have any residual obstruction, who are feeling excellent. Those patients were recommended to see an ACHD cardiologist every two years. And again, patients who are not that optimal, who have a residual lesion, who have an aneurysm that we're following, have other comorbidities probably should be seen at least once a year.

Margaret Fuchs, MD:

And again, at each of those two year visits, we're getting an EKG and an echo. And then three to five years repeating that exercise test, looking for onset of hypertension with exercise, keeping an eye on their descending aorta as well. And finally, management of mental health concerns is important. There is an increased risk of anxiety and depression that occurs in all heart patients, but especially in congenital heart disease patients. And if that's going on and we're not detecting it, our patients are not going to be able to live their best lives and take the best care of themselves that they can. And so, detecting that at the visits and helping them identify optimal care is really important.

Margaret Fuchs, MD:

And finally, exercise and sports participation, which is a big question, especially in our younger coarctation patients who come to us and played high school or college sports. And can I still play? And how are we going to handle this situation? We know, of course, that in all patients there's a great benefit to physical activity, helping that blood pressure that we keep talking about. Maintaining their overall health, supporting their mental health. But there are risks, even in a patient with repaired coarctation or other aortic disease. We worry about provoking hypertension with the exercise. We worry about those aneurysms forming and us not knowing about it. We worry about aortic dissections, especially if they have an injury to the chest. And so, all of these things are factors we have to consider when we approve or disprove a patient to play. Aerobic exercise only results in a modest increase in mean arterial pressure. And so, a lot of times again, as long as our patient doesn't have hypertensive response to exercise, that's likely an acceptable and a good thing to encourage our patients to participate in. But isometric exercise, where we see a large increase in mean arterial pressure is more worrisome. Valsalva maneuver has been demonstrated to result in a systolic blood pressure of greater than 300 millimeters of mercury. And imagine all of that stress on that fragile aorta. That's something that we definitely want to avoid. Our patients who are interested in a weightlifting routine, we recommend low weight, high rep activity. Please don't be a professional mover. Avoid helping your friends move the refrigerator, that sort of stuff to avoid undue injury.

Margaret Fuchs, MD:

Many of you will recognize this chart. This is a 2015 eligible and disqualification for competitive athletes from the ACC. And the exercises here are spread out by increasing static or isometric intensity and increasing dynamic or aerobic intensity. And the guidelines recommend that our patients with coarctation of any sort, repaired, unrepaired, no residual gradient, residual gradient, regardless should avoid the high intensity static exercises. Here at the top. Unfortunately, all of these activities are things that really our coarctation patients should avoid in most cases. Additionally, things that, again, we're worried about, bodily injuries, chest collisions with the asterisk. There are some competitive athletics here in the middle column with the asterisk that we also probably should have our patients avoid in most cases.

Margaret Fuchs, MD:

If our patients are greater than three months after intervention, if they're doing well and if they meet the following criteria, they can likely participate in everything else. If they have, again, no hypertensive response to exercise, no residual aneurysm that we're worried about, no other significant heart valve disease, there's a lot of activities that our patients can and should be involved in. And we encourage them to do that. And I think helping our patients think through what's a great sport for me and what's perhaps something that's a little bit more risky is an under-recognized part of the follow-up of these patients. But can really help them feel confident living their daily lives. And then finally, if there's a patient that we're worried about with residual aortic dilation, other complications that we're following, those are patients who probably should stick with the lowest intensity exercise. 

Margaret Fuchs, MD:

In conclusion, our patients with aortic coarctation can be quite complicated. They require lifelong subspecialty follow up at least every two years with an adult congenital heart disease specialist, as well as serial imaging. Associated medical comorbidities should be actively sought, aggressively managed to minimize comorbidities developing over time. Lifestyle modification is an important focus for follow-up visits. Many physical activities remain accessible to these patients, but a few probably should be avoided. Thanks very much.

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