Care of Patients with Aortic Disease

Podcast Transcript

Announcer: Welcome to Cleveland Clinic cardiac consult, brought to you by the Sydell and Arnold Miller Family Heart and Vascular Institute at Cleveland Clinic. In each podcast we aim to provide relevant and helpful information for healthcare professionals involved in cardiac, vascular and thoracic specialties. Enjoy.

Dr. Steve Nissen: I'm Dr. Steve Nissen, chairman of the department of cardiovascular medicine. We're here today with Dr. Sagar Kalahasti ...

Dr. Sagar Kalahasti: Yeah.

Dr. Steve Nissen: ... who's in the department of cardiovascular medicine.

Dr. Sagar Kalahasti: That's correct.

Dr. Steve Nissen: And Dr. Eric Roselli, cardiac surgery. And we're gonna talk with these two experts about aortic disease. Both of you are very focused on this, this disorder. So, when we talk about aortic disease, what are we talking about, Dr. Kalahasti?

Dr. Sagar Kalahasti: Yeah, one of the main things when we talk about aortic disease is aneurysms are one of the most important things. Aneurysm is such a dangerous term. When patients Google aneurysm all kinds of dangerous things come up on Google. It's a very scary term, but at the same time there are different grades with regards to aneurysm. And patients most often come here, mainly because of worries of what could happen when they have aneurysm.

Dr. Steve Nissen: Yeah. And of course many physicians are gonna see these patients in their practices and they've gotta understand when should these patients be referred, what testing should be done. So, let me ask you a few, both of you a few questions about this. First of all, are there heritable forms of this disorder? Do you see, does this run in families? What do you know about the genetics and the inheritability?

Dr. Sagar Kalahasti: Yeah, so ...

Dr. Eric Roselli: Can I handle this one?

Dr. Sagar Kalahasti: Sure.

Dr. Eric Roselli: 'Cause I'd like to actually go back and expand a little on the first question about what are we talking about when we talk about aortic disease.

Dr. Steve Nissen: Yes.

Dr. Eric Roselli: I think that we're gaining an appreciation that we're actually talking about a whole bunch of different diseases. A bunch of degenerative processes that come to a funnel at a similar kind of presentation that is aneurysm or dissection. And that's why a lot of times people will lump together dissection and aneurysm, 'cause they often happen in concert, although they are different processes. If we step back, and as we're starting to understand really what those fundamental processes are that are leading to it, some are certainly genetically triggered, causes for this degenerative process. Some are probably something that acquired from exposure from various things. For example even some of the giant cell aortitis or etiologies we see, we suspect maybe even acquired from something in the community or some infectious disease exposure or something.

Dr. Steve Nissen: Atherosclerosis.

Dr. Eric Roselli: Atherosclerosis, of course. And so all of those are a bunch of different diseases that come together and cause it.

Dr. Sagar Kalahasti: Absolutely. Yeah, so, going back to the second question about genetic diseases, one of the most common ones that we do here is Marfan syndrome. The cardinal feature of the disease is aortic aneurysm. And we have a specialized center that takes care of patients with Marfan syndrome as one. But there are many other forms of genetic diseases such as Loeys-Dietz syndrome. Bicuspid aortic valve is one of the very common congenital heart disease, I would say. The most common adult congenital form of heart disease, which has high association with aortic disease and aortic aneurysms.

Dr. Eric Roselli: But not all of them are syndromic.

Dr. Sagar Kalahasti: Sure.

Dr. Eric Roselli: So a lot of people come to us and maybe even have been diagnosed with Marfan syndrome in the 90s and we've found that now they actually have what we now Loeys-Dietz.

Dr. Sagar Kalahasti: Loeys-Dietz syndrome.

Dr. Steve Nissen: Yeah. So, we've gotten smarter about this.

Dr. Eric Roselli: We've gotten smarter. Currently we're screening for about 23 genetic abnormalities that we know of. Some of which do have other syndromic-like features. And many of whom don't.

Dr. Steve Nissen: So, let me ask you. When these patients first present, do you always get genetic screening in these patients?

Dr. Eric Roselli: I get my genetics counselor to see all my patients who are under the age of 16. And that gives us the benefit of trying to gather some data about a family tree and exposure, as you know, doing that for other cardiovascular diseases, most of us don't know how anyone beyond one generation really died. But it's still helpful. And now insurance companies are more often actually allowing us to pay for that screening panel that we get. That 23 gene screening panel.

Dr. Steve Nissen: So let me try to understand this a little bit better. Does the genetics drive how you manage these patients?

Dr. Sagar Kalahasti: Yeah, if you have a family history where they had had dissection at a young age and genetic mutations are found in those, we are more aggressive with their treatments. Not just medical, but surgical treatments. Where we intervene on is much higher.

Dr. Steve Nissen: So you operate earlier in ...

Dr. Sagar Kalahasti: Yeah.

Dr. Steve Nissen: ... some of these syndromes.

Dr. Eric Roselli: If someone has a TGF beta receptor abnormality, the guidelines tell us to operate on aortic root when it's in the four to four and a half centimeter range. And so yeah that's a very clear example of it. On the other hand, also this genetic information and familial information drives the way that we plan diagnostic followup or imaging of family members. So I think it very much guides our treatment, more than it ever has. Hopefully as we learn more we'll be able to tailor that even better. We'll be able to have personalized medicine kind of approach.

Dr. Steve Nissen: So let's talk about this. And tell me, sees a patient and typically how are these patients come to medical attention? What's the ... how are we finding these things?

Dr. Sagar Kalahasti: That's a great question. With the number of imaging tests that we do these days and the screening evaluation we do, perhaps for a long nodule for lung cancer screening, or we do calcium scoring for cardiovascular disease screening. We are actually detecting more and more patients come to us ...

Dr. Steve Nissen: Asymptomatic aneurysms.

Dr. Sagar Kalahasti: ... asymptomatic aneurysm, much smaller sizes. Right? About the threshold about what we would call as aneurysm. So that's probably the most common.

Dr. Steve Nissen: And what's that threshold? Where do you ... ?

Dr. Sagar Kalahasti: I think anything greater than four centimeters, would you agree?

Dr. Steve Nissen: I agree.

Dr. Sagar Kalahasti: More than four centimeters I think is ascending aorta or the aortic root.

Dr. Steve Nissen: How do you address those thresholds for the patient's size in general?

Dr. Sagar Kalahasti: Yeah, that's a good question. It used to be adjusted for primarily body surface are and one of the biggest caveats is body surface area incorporates patient's body weight, which can fluctuate. And sometimes adjusting to body surface area may not be ...

Dr. Steve Nissen: I thought...

Dr. Eric Roselli: Yeah well if weight goes up, your index goes down.

Dr. Sagar Kalahasti: Goes down.

Dr. Eric Roselli: And using that as a way to index means your risk goes down if you get fatter.

Dr. Sagar Kalahasti: That doesn't make any sense.

Dr. Steve Nissen: No, it doesn't make any sense.

Dr. Eric Roselli: So we have good data from our group that demonstrates height as an important tool for indexing. And it's been validated, even by the folks who were pushing the BSA, have now come full circle and everyone's appreciating height as helpful. But, still, I think in a overall way of screening patients for ... certainly for primary care folks or anybody else that sees someone, it makes sense to raise a flag if it's more than four centimeters. I think that's a good ...

Dr. Sagar Kalahasti: Threshold.

Dr. Eric Roselli: ... threshold for aortopathy. And then it doesn't really get serious or necessarily warrant a surgical intervention til it gets closer to five.

Dr. Steve Nissen: Now let me ask you this. So, somebody picks up this. And I'm assuming that the majority of these people ar asymptomatic, is that ... ?

Dr. Sagar Kalahasti: Yeah, the majority of them that we see I would say so. Unfortunately ...

Dr. Steve Nissen: Even the ones you operate on.

Dr. Eric Roselli: By far. And even when people have symptoms, it's usually not related to their aorta. It may bring it to attention, but it's probably not what's causing the symptom.

Dr. Sagar Kalahasti: Yeah.

Dr. Steve Nissen: So, what kind of surveillance do you then do with those people? How often do you see them? What tests do you get? Is there a preferred approach?

Dr. Sagar Kalahasti: Yeah, I think depending on the initial size, I think that's the primary thing. Depending on the initial size you can determine the interval of followup and as far as the imaging, echoes, CT, MRI are prominently the modalities that we use. In general ...

Dr. Steve Nissen: Are they equivalent?

Dr. Sagar Kalahasti: They are fairly equivalent to certain segments of the aorta. And in center where they have special expertise and complicatedly looking at particular segments, I think they would be comparable. I would say aortic root and ascending aorta are fairly well visualized in echo. But if you come to the aortic arch or descending aorta, you would have to rely on tomographic imaging like CT and MRI.

Dr. Eric Roselli: Yeah I think the nice thing about echo is is totally non invasive. You don't even need an IV. And it's easy to get that coordinated and paid for.

Dr. Sagar Kalahasti: In addition, you can also look at the aortic valve. If you have valvular abnormalities, is it causing aortic valve regurgitation, echo is an excellent tool.

Dr. Eric Roselli: But if you have a high suspicion.

Dr. Sagar Kalahasti: Sure.

Dr. Eric Roselli: Remember that you only see the first part of the ascending aorta, which often misses the maximum diameter of the thoracic aorta in this echo in a large majority of patients. So, if you're even suspicious on the echo, I think you have to get tomographic imaging with CT or MRI to scan the whole aorta. I think that's critical. Echo might be a nice first step but they're definitely complementary, they're not competitive, different methods of imaging.

Dr. Steve Nissen: But how often should we?

Dr. Sagar Kalahasti: Again, going back to the initial size. If it is only four centimeters and if you have previous imaging to compare to and if we can establish that it's been stable for a few years, then you can see them annually. If the size is more than four and a half centimeters, then you would want to be seeing them more soon. Perhaps every six months. Again, if it's a family history, where they had dissection of smaller sizes, then you want to be more aggressive. So it all depends on the initial presentation, family history, if they had a prior dissection in the family of smaller size. So that's what determines the interval of follow up.

Dr. Steve Nissen: Now

Dr. Eric Roselli: Typically and we, I think, share the same ideas, right?

Dr. Sagar Kalahasti: Yeah.

Dr. Eric Roselli: Many of us in the cardio aortic sub specialty in our group, typically what we do is, let's say ... and sometimes it's even just to put the patient's ...

Dr. Sagar Kalahasti: Ease.

Dr. Eric Roselli: ... mind at ease a little bit, is to make sure we get two imaging studies that are comparable that are within six months of each other.

Dr. Steve Nissen: And then you know what your trajectory is?

Dr. Eric Roselli: Then you need to establish some sorts of stability ...

Dr. Sagar Kalahasti: Right.

Dr. Eric Roselli: ... and then you come up with a plan based on all those other features.

Dr. Sagar Kalahasti: Absolutely. In fact, patients prefer that we do that. And they mind is a lot more at ease ...

Dr. Eric Roselli: Well, they'll even-

Dr. Sagar Kalahasti: ... if you're taking it seriously.

Dr. Eric Roselli: Yeah, they'll even ask you, "Six months? Too long."

Dr. Sagar Kalahasti: They would want to be there every month.

Dr. Steve Nissen: Yeah, in a lot of diseases surgery has come to be done earlier and earlier because as you've improved surgical technique and you've lowered morbidity and mortality, then it's done earlier. Is that true in aortic disease as well?

Dr. Eric Roselli: Yeah, absolutely. So, the one thing that's important to understand is certainly the recognizing the limitations of our ability to predict the natural history of any one patient's aortic aneurysm disease has to be balanced against an individual center's experience with surgical outcomes.

Dr. Steve Nissen: You do a lot of these surgeries.

Dr. Eric Roselli: And we do a ton. We ...

Dr. Steve Nissen: How many do you do a year?

Dr. Eric Roselli: So, in the Aorta Center at the Cleveland Clinic Cardiovascular Institute, we operate in over 1,200 aortas a year and over 800 of them are thoracic aortas.

Dr. Sagar Kalahasti: Thoracic, yeah.

Dr. Steve Nissen: Is that the biggest center in the world?

Dr. Eric Roselli: Pretty sure.

Dr. Sagar Kalahasti: Yeah.

Dr. Eric Roselli: And there's a couple centers in China that operate on a lot.

Dr. Eric Roselli: So, obviously, having that level of experience has driven down the complications of surgery and so you can be reasonably aggressive. Obviously the cost of waiting too long can be death.

Dr. Sagar Kalahasti: Yeah. Serious complications and performing were emergency surgery, which has much higher complication rate than doing an elective surgery.

Dr. Steve Nissen: Now, I'd like to turn to the issue of the approach. The surgical approach. Obviously these things can vary from simple to complex. What are some of the innovations that are really driving how surgery's being done for these people?

Dr. Eric Roselli: Well, that's something I could talk about for the entire time and ...

Dr. Steve Nissen: I'm sure you do all the time. I'm sure you do.

Dr. Eric Roselli: But I would say simply it really depends on the segment of the aorta. And we are getting better in every way. So, in the aortic root, which is the most complex section of the aorta, we are now offering valve preserving kinds of operation. So, commonly doing over 100 of them a year in our center. And we'll even offer it to patients who are in there seventh decade and beyond, 'cause we can do that really safely.

Dr. Steve Nissen: What do you mean by valve preserving?

Dr. Eric Roselli: A root replacement with a re-implantation of the valve. So we replace that whole aorta right to the inside of the left ventricular outflow tract, we re-implant the coronary arteries, but instead of giving them a prosthetic valve, we save their native valve and put it inside a, as I say, a happier home. So it's in a new graft. That's a complex operation that's not offered everywhere but is very common here and we now do it consistently with excellent expertise and predictability about our ability to save the valve, which changes the planning of when to operate. Because now we're not worried about giving them ...

Dr. Sagar Kalahasti: A prosthetic valve.

Dr. Eric Roselli: ... the issues that are associated with a prosthetic. So that's a great innovation. That's not really minimally invasive. On the other hand, when we're talking about the downstream aorta, the descending aorta, we almost are predominantly treating those aortas with endovascular therapies. We're delivering devices percutaneously. We've got a whole bunch of devices that are actively in trial, that have branches built into them for the aortic arch and the thorical abdominal segment. And so I think there's a lot of exciting technology on that. And we're about to enroll our first patient with a novel device even for the ascending aorta, a dissection. So I think that by bringing all these new technologies and innovations from the way we do open surgery better to the way we do endosurgery better, we can treat this disease safer and really change the natural history of it.

Dr. Sagar Kalahasti: Yeah. And a nother innovation that we're also doing is in connective tissue diseases, where most people have always thought surgery is the only option. We are using endovascular therapies in those patients too.

Dr. Eric Roselli: That's a good point, yeah. So, we've gained this ... we've taken a different look at it. We're not just looking at aortic disease like, "Yeah, there's this one thing that's gonna kill you tomorrow, we're gonna fix it and then disappear." We're giving patients a lifelong approach to the disease understanding that it is this chronic degenerative process.

Dr. Steve Nissen: And it may refer somewhere else.

Dr. Eric Roselli: Absolutely. And so by using innovations in our imaging suite, so we minimize radiation exposure, we optimize the contrast timing in our interpretation skills and everything else. We can offer combinations of therapies or hybrid therapies. Even our connective tissue patients will get an open surgery for one part of their operation but during that time we're thinking about what the next one might look like, so we set something up so that we have a place to land a stent graft in the event that they ...

Dr. Sagar Kalahasti: On the second stage.

Dr. Eric Roselli: ... go on to need to need a second or third or fourth stage down the road.

Dr. Steve Nissen: What are the outcomes?

Dr. Eric Roselli: It's great.

Dr. Steve Nissen: So, how well do these patients do with this disorder? Obviously I'm sure it depends on what the ... a lot of factors, but overall, so ...

Dr. Sagar Kalahasti: For an elective ascending aortic root replacement our mortality is almost less than one percent.

Dr. Steve Nissen: Oh, it's, yeah ...

Dr. Eric Roselli: It's definitely less. In several big series we've published, for example, in patients with bicuspid aortopathy mortality has been 0.25% with a stroke rank less than one percent as well. So combined stroke and death of less than one percent in these patients.

Dr. Steve Nissen: Well, so with all these innovations in diagnosis and in therapy, the prognosis has really gotten better for these people over the last few years.

Dr. Sagar Kalahasti: That is excellent. In Marfan syndrome patients you could almost tell them that they have a normal life expectancy because we are recognizing the disease early on, we are treating them early on with excellent outcomes and with continued, long-term followup. Their outcomes are excellent.

Dr. Eric Roselli: Yeah, we're actually ... again, with that broad eye to this disease, I'm very optimistic about where we've come. We're even doing research now that's focusing on lifestyle. Equality of life. 'Cause we not only wanna make them live longer, we wanna make them live better. We're trying to understand specifically how much exercise can someone do when they have this diagnosis. We don't have hard, objective answers but we're working on ways to figure it out.

Dr. Steve Nissen: Fantastic. Well thank you both for bringing us up to speed on this really important disease.

Dr. Sagar Kalahasti: Thank you so much.

Dr. Steve Nissen: And thank you all for joining us.

Dr. Eric Roselli: Thanks.

Announcer: Thank you for listening. We hope you enjoyed the podcast. We welcome your comments and feedback. Please contact us at heart@ccf.org. Like what you heard? Please subscribe and share the link on iTunes.