Aortic Dissection: Your Questions Answered

Podcast Transcript

Announcer:
Welcome to Love Your Heart, brought to you by Cleveland Clinic's Sydell and Arnold Miller Family Heart, Vascular, and Thoracic Institute. These podcasts will help you learn more about your heart, thoracic and vascular systems, ways to stay healthy, and information about diseases and treatment options. Enjoy.

Eric Roselli, MD:
Hello everyone. I'm Eric Roselli, Surgical Director of the Aorta Center, and I'm here with my colleagues, who I'll have introduce in just a second. But, we want to have a discussion for patients who have the diagnosis of aortic dissection and allow an opportunity to share some of our thoughts, and answer some of your questions about this difficult diagnosis in this vlog, this video discussion. Scott, you want to introduce yourself?

Scott Cameron, MD, PhD:
Hi there. My name's Scott Cameron. I'm a vascular medicine physician and cardiologist, and I serve as Section Head of Vascular Medicine, so I'm interested in research. And Dr. Lyden and I have federal funding to study aortic disease, and we truly are a multidisciplinary team.

Eric Roselli, MD:
Sean?

Sean Lyden, MD:
So, Sean Lyden. I'm the Chairman of Vascular Surgery. Had been the Vascular and Endovascular Director of the Aorta Center. I just handed those reins over to one of our new colleagues, Frank Caputo, but I'm also happy to be here, and show how we work well together.

Eric Roselli, MD:
And we let Dr. Desai show up today too.

Milind Desai, MD, MBA:
I'm Milind Desai, I'm the Medical Director of the Aorta Center. I am a cardiologist and I'm also Director of Clinical Operations for the Department of Cardiovascular Medicine.

Eric Roselli, MD:
And somehow you found time to join us.

Milind Desai, MD, MBA:
And this is good, yes.

Eric Roselli, MD:
Yeah.

Milind Desai, MD, MBA:
Would not miss it for anything else.

Eric Roselli, MD:
Fantastic. I'm going to ask you, Dr. Desai, to address just the simple question about what is a dissection, and how do you differentiate it from an aneurysm, or what is the connection there? Those diagnoses often come together.

Milind Desai, MD, MBA:
Yes. Yes, they are. They come together. Thanks Eric. So, what is an aortic aneurysm? An aortic aneurysm is where the aorta, which is the big blood vessel that emerges out of the heart and supplies blood to the rest of the body, but the wall of the aorta, because of whatever reason, be it a genetic predisposition or some other attribution, it gets thinned out. It expands. It is not able to sustain the pressure of the blood coming out, and it basically balloons out. That is called an aortic aneurysm. Once it reaches a certain threshold, five centimeters, then that is when it is called an aortic aneurysm.

Milind Desai, MD, MBA:
Now, as a balloon, if you keep stretching the wall, at some point of time there will be ... it is not going to be able to sustain the pressure, and then portions of the wall may tear, and then the blood may enter from the lumen, or the central cavity, into the wall and essentially create two ... separate the layers of the aortic wall. That essentially defines dissection. A rupture is a continuum, as it sounds, as ominous as it is, where if all the layers are breached, and the blood exits outside the cavity to the chest cavity, then that's essentially aortic rupture.

Milind Desai, MD, MBA:
So, aneurysm, dissection, and rupture. That's sort of in a continuum, but aneurysm is where you have a dilation of the aorta. The other one is where there's a breach in the wall, and the third one is ... it is catastrophe.

Eric Roselli, MD:
Right. So, they clearly are tied together. So the aneurysm is when the vessel's stretched out, the layers can start to split, and that's the dissection. But, not all dissections are necessarily related to aneurysms, are they Scott? There's probably ... or certainly there is, another, or a whole bunch of series of different disease processes that are occurring in a microscopic level in the wall of the aorta. You and I both have research labs that are focusing on trying to understand this better. Can you tell us a little bit, what are the causes of these problems?

Scott Cameron, MD, PhD:
Yeah, so that's a great question Dr. Roselli. Sometimes there are syndromes, we call them acute aortic syndromes, so a patient for example may have hardening of the artery, arthrosclerosis, and they can have a little bit of erosion of one of those narrowed plaques in the aorta. And if that erodes deeply enough, sometimes the patients will have acute chest pain, and it can feel like an aortic dissection, but it's a slightly different process. As you said, it's disease of the inner layer of the blood vessel. And patients that have damage to the blood vessel, an ulcer, for want of a better word, sometimes have abnormally high cholesterol. It would be not unusual for us to see those patients in clinic and actually diagnose them with a genetic disorder, whereby they're making too much cholesterol, or they cannot clear that cholesterol.

Scott Cameron, MD, PhD:
So a patient may come to the Cleveland Clinic and have pain, and they may have an imaging finding, but what we often find is that by comprehensive care, and by communicating back and forth very effectively, we can often find two or three medical reasons that actually got them into that situation in the first place. And arthrosclerosis is one of them, but certainly, as you alluded to, genetic disorders of blood vessels, particularly those that affect the aorta, is certainly another consideration. I think generally it's a lot of undue anxiety for patients as well as their families.

Eric Roselli, MD:
So, the atherosclerotic disease process is where we get these penetrating ulcers. I think ulcer is actually a good description of what we see in the operating room, right Sean? The ulceration process is one thing. That typically happens in older patients, but not necessarily, but mostly in older patients. But, our younger population, or middle aged population, it's often some ... what we're learning is more and more now are genetically triggered, an error in the ability of the aorta to maintain its integrity, and sometimes we'll see dissections at even smaller sizes. Sean, we see a lot of those, and we get called on in the middle of the night and we have to make a plan together about how to take care of them. Can you tell us a little bit about what's the treatment for aortic dissection?

Sean Lyden, MD:
So, I think we sort of look at it where the location is. And so if it's close to the heart, or if it comes off before the artery to the left arm, the risk is that tear can propagate towards the heart and leak into the cavity that the heart sits in, or the pericardium, that bag, or it can tear down to where the heart arteries are and lead to lack of flow to the heart, and so the heart can die. And so those are called type A dissections, and those really need to be repaired as soon as found. The second type is where it happens distal to the arm artery, and those are called Type B. Those are-

Eric Roselli, MD:
Just so ... you mean like downstream?

Sean Lyden, MD:
Downstream, or down towards the legs. So, coming from left arm artery, down where the aorta ends at the belly button. So, those are originally helped by lowering the blood pressure, and so just taking some of the pressure off the wall. But, we found that many of those patients can be relined from the inside, to sort of put an inner tube within the aorta to cover over that entry tear or hole is, to try and reline it and get it back to normal size. And so we look at it, whether it's close to the heart or farther away from the heart. We treat both, but the timing of when we do it is a little bit different, whereas it's much, much sooner when it's closer to the heart, and it may be a little bit more delayed if it's further away from the heart, and that's really because we've continued to study how best to treat these patients, and who best to treat.

Sean Lyden, MD:
And then there's patients where it involves the whole thing, where it may have a little bit of both and early repair, and then a late repair down the road.

Eric Roselli, MD:
Yeah, and one of the things I think, message I often will tell my patients is even if the dissection, the layers coming apart, is involving the downstream part of the aorta, and we've either opted to treat it with really strict blood pressure control, medical therapy, or as you mentioned, now we're more often putting the devices in to reline things, because we've had real good success with that. The substrate of that aorta, even the parts that aren't dissected, are probably vulnerable, because we were talking about, there's something going on at a microscopic level. So, we've got to follow all these patients. I think one of the things that's hard for patients to grasp when they're in the sort of the chaos of everything that's happening with acute aortic syndrome process you were talking about Dr. Cameron, is kind of understanding what's next. And so I think an important message that we have to send, and it's a question that's come through from Betsy's office, through our patient and nurse directed patient education office, is if I've had something taken care of in one part of my aorta, is the rest of my aorta at risk?

Eric Roselli, MD:
The answer is pretty much yeah. I think we all have to sort of feel that yes, the answer's yes. Specifically, how much at risk and when at risk really depends on some of the very specific things that we're seeing, based on imaging studies, cross sectional, what we call cross sectional imaging studies, which are CAT scans or MRI. Milind, can you talk a little bit about that, about kind of how we manage a patient after we've gotten them through the acute phase of either an open surgery for the upstream aorta, what we call type A repair with one of the cardiac surgery teams, or a downstream repair with the vascular surgery or cardiac surgery team? We work together in unison to manage all that, then what do we do? What's next?

Milind Desai, MD, MBA:
So thanks. So like Eric said, it is ... the initial insult and the initial operation, initial procedure, to fix this insult is just the beginning of a long-term relationship with your aortic expert. Essentially, at that time, depending upon where the insult was and what kind of an operation or a procedure you had, that helps dictate long-term follow-up plan in terms of imaging. If you have an endovascular stent graph, that requires a different type of imaging at different time intervals. If you have a surgical graft, it requires different type of imaging at different time intervals.

Milind Desai, MD, MBA:
Now, as Dr. Roselli alluded to, it is absolutely crucial to have precise tomographic imaging, so either contrast enhanced CT scan, or MRI, off your aortic vasculature to help not only establish a baseline, but have serial follow-up at various time points to make sure everything is stable, make sure things are regressing appropriately, and sure as heck make sure things are not progressing at an alarming rate, to a point where we need to do something. Additionally, especially the early part of disease manifestation, we are to make sure there's no post-procedural complication at the operation site. It could be infection. It could be a weakness at the junction, which results in something called a pseudo aneurysm, various things.

Milind Desai, MD, MBA:
So, bottom line is, imaging. Having a plan, having a follow-up plan and having an imaging plan are absolutely crucial. And as Dr. Roselli alluded to, this is essentially a lifelong disease that requires a lifelong plan. This is sort of 401A, like you have your 401K for retirement. This is your 401A. You need a long-term plan.

Eric Roselli, MD:
401 aorta?

Milind Desai, MD, MBA:
401 aorta.

Eric Roselli, MD:
I like that. That's good.

Milind Desai, MD, MBA:
There you go.

Eric Roselli, MD:
That's good. And it really requires a multidisciplinary team, like the one we have sitting here, doesn't it? So Sean, what do you think after we treat someone? How often do you think we have to go back and touch things up, or add a repair? I mean, it gets-

Sean Lyden, MD:
So, it depends. If you were the abdominal aorta, we know that at five years about 10-15% of people will develop an aneurysm in their chest, and so the current guidelines suggest if you've had the aorta in the belly fixed that you go and look here at least every three to five years. If it starts in the chest, it's actually much, much more likely that you develop in other places. And well as, you might have it in other places, like your brain or other things. And so it's really the coordination of the multidisciplinary team to sit there and say, "Where was your original problem? Do you have a problem with your tissues, the connective tissue that holds us together, that makes you more likely to have it? And then what pattern we might look for it to happen?" And so, if it's closer to the heart, the closer to the heart it gets, the more likely you'll have it in more places. The further away from the heart, the less likely it is.

Sean Lyden, MD:
But, the key is it really needs to be lifelong care, coordinating your blood pressure control and how often you get imaging and having someone who can reassess is your repair okay and are the areas that haven't been repaired degenerating? And so I think that's why you really want to be with an expert who feels very comfortable in seeing these types of patients and works as a team, because we don't all know each part of it as well as we do as a group.

Eric Roselli, MD:
Yeah, I like the way you described that, the location sort of coordinates with the association of risk. Someone we don't have sitting with us today, but part of our team, that's really critical is our clinical genetics team. And so we very commonly have, especially aortic dissection in patients with thoracic aortic disease, where there tends to be a stronger association with genetics as part of the cause, if not the very clear cause. We have them evaluate most of our patients. And what's been interesting is we're learning so fast that even if we've had someone screened for genetics, five or more years ago, they often need an additional screening study to update the genetics, right? Because, now we're screening for panels of up to 35 genes from the latest invitae panel.

Milind Desai, MD, MBA:
Yeah, and we are expanding that with our newer upcoming initiative into a lot more, or full spectrum of cardiovascular disease. So, you are absolutely right. I think the bottom line is, it is not a one-time deal. This is a start of a long-term relationship. And you have to stay true to yourself. Every time that patient comes in, have you looked at today's images? Have you compared them to previous images? Is it just slowly growing just enough that if you compare the image from today to three years from now, of three years prior, is there a substantial increase? And like Eric mentioned, genetics, it is rapidly evolving. So, what you heard five years ago, I mean if you have a definitive mutation it's one thing, but if you were told five years ago you were negative, or you have a variant of uncertain significance, then it is absolutely worth your while to have a revisit.

Eric Roselli, MD:
Yeah, I just had a patient who had a very well-known significant change just in the last year. They just called him back. He's really cool. Scott, what do you tell your patients about getting family members tested or family members evaluated?

Scott Cameron, MD, PhD:
So, that's a great question, and part of it depends on if the patient has a mutation that I know has predictable heritability. And so certain mutations and genes that go into the proteins in the blood vessel wall, some of those you have a 50% chance of inheriting them. In fact, I had a patient like that just a few months ago, and based on this particular genetic test, I told him, "You have a one in two chance of passing this on. Do you have any children?" And this patient did, and asked me to see their child and it turned out, as adolescent, also had that gene.

Scott Cameron, MD, PhD:
And what was a good example for this particular patient is this patient came to us already having known "I've got a little tear in a blood vessel, and I probably need surgery," but then the person's child on the other hand had absolutely no symptoms, but in fact did have an enlarged aorta. And that presents an opportunity to try and correct something, and so my surgical colleagues of course I'm sure they would prefer to perform surgeries not always in an emergency, where the environment is maybe a little bit less controlled, and I think the difficulty is of the genes of unknown significance. And certainly we're learning more everyday and also they can overlap. Sometimes we find blood vessel disorders in the head, just based on noticing a blood vessel disorder in the aorta. So, personalizing it to the patient is key.

Eric Roselli, MD:
Yeah, that's a good point. The more we understand, the more precise we can be in our plans for patients. Sean, one of the questions that we really commonly get from our patients after they've survived an aortic dissection is what can they do? Can they go back to work? Can they exercise? What kind of lifestyle modifications do they have to implement into their now survival, chronic phase of life?

Sean Lyden, MD:
So, I think they can get back to activities, but really the key is what part of the aorta was damaged? What part of the aorta is repaired? What part is at risk? And really, working with your care team to define what that risk is, and then participating in supervised exercise to make sure that you're maintaining a safe blood pressure, a safe heart rate and that your team of physicians is helping coordinate when it's safe to do those activities. So, if you still have untreated dissection or aneurysm that is small or not causing problems with flow downstream, and your blood pressure is low and your heart rate is low, then it may be safe to do more than if your blood pressure's very high. So it really is important that you work with your doctor to sort of understand that.

Sean Lyden, MD:
But, it is possible for patients to get back to activity. I think it's within reason, and so I don't think really, really, really strenuous activity makes sense, so I always tell people they're not going to be weightlifting. They're not going to be maybe doing a triathlon with some of these processes, but there are, depending on what their disease was, we do have a lot of professional athletes we've treated here at the Clinic that have had ascending aortic aneurysm repairs that get back to playing professional sports. So, I think it's really understanding where your problem was, and then working with the team to make sure you're doing it safely and that there's ongoing checking that everything remains safe.

Eric Roselli, MD:
Yeah, I like that answer. That's also a very much personalized thing. So many patients come in, really scared to death. Someone told them, "Don't lift more than 10 pounds. Don't get constipated." Honestly, poor patients, sometimes they're walking on eggshells. They don't have to be once we know, as you said, blood pressure's key and getting to a point where we're happy with anatomy and what the aorta looks like. That's really critical. And I think it's an important message to send to patients. And if you're having fear about what you've gone through, it's normal. So, we studied this. We did a survey of a whole bunch of patients who survived type A aortic dissections.

Eric Roselli, MD:
So they came in on a helicopter pad and went straight to having open surgery, and we've done a subsequent survey, and those results are being analyzed right now. We tied them into the patient records, which is really cool. But, in both of those studies, where we did the primary care physician PTSD or post-traumatic stress disorder screening study, where we asked them the four questions that are asked in that standard screening process, it was pretty remarkable to see that nearly 50%, at least 45% of patients, answered one of those questions positive. And those questions are like serious things that most people would say no to in a normal situation.

Eric Roselli, MD:
And nearly a quarter of the patients answered three out of four of those questions positive, which means they screened positive for PTSD and should have been referred to someone in the mental health field to help them deal with those PTSD issues. And a lot of these patients answered the questionnaire five, six, seven years after the event. So, the fears that patients have about this are common, and it's okay that there are certain things that you're feeling if you're a patient who survived this, you're a family member who kind of dealt with the long-term issues of it, but know that there are dedicated teams that understand all aspects of your care, from complex imaging to basic science research, to genetics, and to complex open endovascular surgical repair that can work together, and also understand the emotional issues that are attached to all this, to help you live a normal life and get back to it. I think we probably should wrap things up. I really appreciate the opportunity to sit down with you guys, as always. We'll be talking probably in 10 minutes about a patient together, all of us, in no time. And thank you to the audience. Please, contact us if you have any questions. We have lots of people here to help.

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