CardiOhio Podcast
CardiOhio Podcast
All about FMD and SCAD, with Dr. Heather Gornik
Join our host, Ohio-ACC President Dr. Kanny Grewal, and guests Drs. Ellen Sabik and Heather Gornik, both from University Hospitals of Cleveland, as they discuss the diagnosis and practical management of fibromuscular dysplasia (FMD) - including clinical assessment, classification, screening and the role of imaging. They then review spontaneous coronary artery dissection (SCAD), including clinical assessment, diagnosis, and therapy.
For more information, see:
Spontaneous Coronary Artery Dissection: JACC State-of-the-Art Review from the ACC.
2019 FMD International Consensus: https://journals.sagepub.com/doi/10.1177/1358863X18821816?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
AHA SCAD Scientific Statement
https://www.ahajournals.org/doi/10.1161/CIR.0000000000000564
Interesting case of identical twins with FMD
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So welcome back to the cardio podcast. Thanks for joining us today. We are going to have a very exciting discussion today about vascular medicine as a specialty, but also specifically about fibromuscular dysplasia. And spontaneous coronary dissection and as we often get to do on our podcast, we have a couple of experts from right here in Ohio joining us. I want to start at you by introducing Ellen Savick. She's currently a senior staff cardiologist at University Hospital in Cleveland. As many of you know. But we're also excited that she's the president elect for the Ohio chapter of the ACC. And in fact, she'll be starting her term, I believe, around early April, April 4th now. And not that I'm counting down the days specifically, but so first of all, Ellen, welcome. And can you just take a few minutes to let those who maybe don't already know you a little bit about your clinical interests up at and kind of the path that led you there?
Heather:Wonderful. Thank
Ellen:you, Kenny, for the introduction. So I'm a noninvasive cardiologist here at University Hospitals, Cleveland Medical Center. I do spend some time clinically at Ahuja Medical Center as well, and I, I enjoy doing a lot of teaching as well as clinical work in the echocardiography lab. as well as taking care of general cardiology patients, as well as patients who specifically have valvular heart disease. So I'm actually really looking forward to my time as president upcoming in the future years and, and looking to build on a lot of what you have accomplished over your, your three year tenure. So I'm very excited to introduce Dr. Heather Gornick. Heather and I have known each other for years, first at Cleveland Clinic, but now at University
Heather:Hospitals.
Ellen:Heather is a one of the co directors of our vascular center here at the Harrington Heart and Vascular Institute, as well as a professor of medicine at Case Western Reserve University. In addition to that, she is one of the past presidents of the Society for Vascular Medicine and is a world leading expert on fibromuscular dysplasia. So, welcome, Heather. We're so looking forward to speaking
Heather:with you tonight. Well, thank you, Ellen and Kani, and hello, Ohio ACC.
Kanny:Yeah, hello, Heather. Thanks again for taking time with us. You know, I think many of us do know you from your time here in Ohio as a clinician. I know there's some excellent vascular specialists here in Columbus, actually, that trained under you and other leaders in the field here as well. Can you just say a little bit about, you know, your path? Because I know you were a bit of a trendsetter into the field of vascular medicine with a background in cardiology and how that led to your clinical, focus and then ultimately what led to your interest in FMD as a subspecialty.
Heather:Sure. Thank you. Well, it was a real journey. When I started my internal medicine residency, I trained at Brigham and Women's Hospital in Boston. I thought I was going to be an oncologist. And actually then I fell in love with critical care. I loved physiology. I was doing a, a cardiology rotation and I met Dr. Mark Krieger, who's a past president of the American Heart Association and was the head of the vascular medicine group at the Brigham. And he really just inspired me. He was a, he is an amazing bedside clinician. He is a translational and physiology researcher. So I had this great sense of physiology and he introduced me to the field of vascular medicine where there is lots of. Physiology and a real chance to use clinical skills coupled with physiology, imaging to diagnose and treat patients. So I became interested in cardio, cardiovascular medicine. I did training in cardiology, and then I did two additional years of training with Mark Krieger's group with people I think well known to ACC members like Josh Beckman and Marie Gerhard Hermann and Sam Goldhaber. and I trained in vascular medicine. I finished my training in 2005 and was looking for my first job and I had a sense I wanted to, to leave the Brigham. I had been there for eight years at that point and was looking around the country and Came upon the program at Cleveland Clinic, and they had one of the largest vascular medicine programs in the United States, actually the second program in the United States. It was founded after the Mayo Clinic program, and I was very impressed with their clinical program, their training program, their academic program, and I joined the staff of the Cleveland Clinic, and I guess the rest is history. I was there for 13 years. I had a chance to run the vascular lab there for, oh, about 11 or 12 of them, and it was there that I really developed an interest in FMD, and I saw a few patients who were actually A Patients previously cared for by Dr. Jeff Olin, who, for those who know the field of FMD, knows he's another person who's very active in the field, and he had been at the Cleveland Clinic and left a few years prior to my arrival, and some of his former patients were put on my schedule because people knew I was In addition to being a vascular medicine specialist, I'm a cardiologist and was very interested in arterial disease. So I started following these patients and a couple patients made comments to me with things like, gosh, it's so nice to meet a doctor who. has seen one of me before. Or, you know, Dr. Gornick, you didn't have to pull out a textbook to know what to do about me. And I just realized that the bar was so low in this disease state. You know, I'm used to a cardiology background where we have randomized trials of thousands of patients. And here patients with a serious condition are talking about doctors relying on a A textbook. So I realized that it was a chance to have a real impact. So in 2008, after I had come back from maternity leave from my second child, my son, who's now going to be 16, I started the first dedicated FMD clinic, kind of hung out a shingle and said I was going to see patients with FMD once a month. And eventually it became a couple times a month and then once a week and really grew from there. Subsequently, as we'll talk about, I became very involved in FMD related research and national collaborations, including with the FMD Society of America, a wonderful patient advocacy organization. And then in 2018, I had the opportunity to really grow professionally and cross town to University Hospitals, Harrington Heart and Vascular Institute to continue my work in FMD and also to take on some new roles. I help run the vascular service line across all of the hospitals. And so I'm able to do some different things, but my passion for FMD has continued. We now have FMD. It's gone from once a month in 2008 to now up to three or four times a week in 2023 and have also, as the years have gone by taken on an interest in spontaneous coronary artery dissection, SCAD, other arterial dissections aortopathies and the like. So it's been, it's been a real journey. And like, All of us who have careers in cardiology the path is really interesting and continues to evolve.
Kanny:It sure does. That sounds like a very interesting path so thanks for sharing that with us. So just to kind of frame a discussion about FMD before we eventually, get into more about coronary dissection for our general cardiology audience, I think back to when I was a trainee in the 1990s and we had such a, cursory exposure to Non coronary arterial diseases mainly just an occasional case of vasculitis or suspected, vasculitis, for example. how, how do you frame our current perspective on FMD now that there's been, a little bit more research and understanding about the epidemiology? how do you approach it in relation to other Very, very common atherosclerotic cardiovascular disease in terms of your, how you classify it, and then eventually when, what clinical scenarios should we as clinicians suspected.
Heather:Well, I think it starts with the fundamentals and recognizing that all of us as cardiovascular physicians and APPS are attentive to the vascular system as well as the heart. So I think it just starts with the fundamentals and being in practice and recognizing that our patients with heart disease may well have. peripheral vascular disease as well, so it's including that review of systems for symptoms like claudication, limb swelling, for FMD, we think about pulsatile tinnitus and headaches. It's doing a really thorough physical exam, blood pressures in both arms, auscultating for breweries throughout the body. palpating the pulses. So first it's like recognizing the peripheral vascular disease beyond the heart disease. And then once we determine this patient has vascular disease and that it seems to be arterial disease, it's figuring out if it's the horse or the zebra. And I would say the horse is atherosclerosis. As you said, Kenny, I mean, that's like 90 percent of arterial disease that we see throughout the body is atherosclerotic. But then we have the zebras. And I think among the zebra conditions, fibromuscular dysplasia is probably one of the more common. And just for the, for the listeners, as a reminder, FMD is a disease that affects the arteries. It's generally the medium sized or branch arteries. It can affect arteries throughout the body, most commonly the renal arteries, the carotid and vertebral arteries, but can also affect the intracranial arteries. the coronary arteries manifest with SCAD the intestinal visceral arteries, and the iliac and brachial arteries. So it's really a total body process. About 90 percent of patients, perhaps more in some series, are women, generally diagnosed at midlife. Say our average patient is in the early fifties, but can present in children. Then it's generally a type of FMD called the focal type. In the adult type it's generally called the multifocal type. It looks like a string of beads, and again, more commonly in more of a middle aged adult population. But we do have patients who are diagnosed in their sixties, seventies. I even have a few patients who are in, in their late eighties with FMD. So I think the general approach is to recognize there is peripheral vascular disease as well as heart disease. Determine that it seems to be an arterial problem, and then try to address whether it's the horse, the atherosclerosis, or the zebra. And of course, if you have an older patient with diabetes, hyperlipidemia, all of those risk factors more likely to be athero. But think of FMD when you have younger patients who don't have a lot of atherosclerotic risk factors and who have disease in the, in the typical locations. So, Heather,
Ellen:I guess just to, to focus that a little bit further is what patient population would you say, or what features would you say you need to start thinking about FMD, and what is the role of imaging in making that diagnosis once you have the question of is there possibly FMD or not?
Heather:Yeah, I, I think patients with FMD have three broad classic clinical presentations. One is the hypertensive patient. So early onset hypertension, drug resistant hypertension, hypertension in a, a. thin middle aged woman who really you're surprised has hypertension. So that's the hypertensive phenotype we'll say. The other bucket is people who manifest with symptoms related to the cerebrovascular disease. So these are patients who have carotid bruise. have migraine headaches, have pulsatile tinnitus. I never thought I'd be spending my career when I started asking people about ear noises, but it's a classic symptom of FMD, a pulsatile sound that times with the heartbeat, and most patients describe it as a swooshing, like a whoosh, whoosh, whoosh. So that's another group of patients. And then the third category is the manifestations of dissection and aneurysm. So these are patients who have. coronary dissection, carotid or vertebral dissection, renal dissection with infarct, and that's how they're diagnosed with FMD. So those are the three general clinical categories where we most commonly see patients present. I think Absolutely, as you alluded to, we have to move quickly to imaging. I think duplex is a good start to evaluate the carotid and renal arteries and just screen for FMD, but to definitively rule out or in FMD and definitely to screen all of the vasculature for aneurysms and dissections, we generally need CTA or MRA. And these patients with FMD, we recommend they get a head to pelvis cross sectional imaging.
Kanny:Yeah, thanks, Heather. As Ellen was mentioning and obviously we're both imagers, so we have a big interest in this as well. For for us, general cardiologists who do a lot of consoles many times, the entry point is, you know, a patient was suspected or confirmed, you know, coronary dissection I assume in those patients, we do want to screen for other manifestations of F. M. D. With the image and you alluded to
Heather:absolutely. And the story of of recognizing the link between coronary dissection and FMD is just so interesting. And it really starts in Western Canada. And there was an interventional cardiologist, Chris Buehler, and his subsequent and his partner Jacqueline saw. who did a lot of the early work. This was really in the 1990s and early 2000s. What they did was they had catheter angiograms on their patients who had acute coronary syndrome and they would do kind of drive by renal angiography as I, as, as some may remember, was kind of in vogue in the 1990s. And they reported on this series of patients, younger women with acute coronary syndromes and quote atypical coronaries who when they did these renal angiograms had string of beads multifocal FMD and they sort of postulated could this be coronary dissection and that initial publication I remember reading it as someone taking care of patients with FMD and being very skeptical I didn't know what this was about what is coronary FMD. But now we really know that this is SCAD, and Jacqueline Saw at Vancouver General Hospital, the group at Mayo Sharon Hayes, Marisha Tweet, many other investigators have now done a lot of work that has really linked these atypical coronaries, determining that they're actually coronary dissections, and then with imaging of the coronaries. Extracoronary vasculature, identifying that probably about half, it's more or less depending on the case series and how the patients are imaged of patients with SCAD have FMD, if you image them comprehensively. So there was a really nice consensus statement from the American Heart Association that I love. I was not a part of it. I love, I love reading in it. It's very informative that came out in 2019. If any of the listeners want to download it and it recommends that patients who have a coronary artery dissection have comprehensive imaging, which is defined as head to pelvis CT angiography or contrast enhanced MR angiography to look for FMD as well as a colt. Aneurysms or dissections. Wow, that's
Ellen:wonderful. Heather, I have a question for you regarding when should we be referring patients to specialists like yourself? You know, whether, like I had a patient who we found out that she had FMD because with hypertension that was uncontrolled, I was looking for renal artery stenosis and we found the classic beating pattern. That person was referred. if they had a spontaneous dissection, that's another person. But when do we send them to the specialist and we let you all pick up the head to pelvis CTA or, you know, at what point is it appropriate to send?
Heather:Well, I think I would say really at, at any time, I think for patients with a rare disease to see a physician who, for them, it's just another day in clinic versus for. For some specialists, it may be pulling out the textbook, I think makes a big difference in the patient's experience. I think the opportunity to see a specialist who's participating in research. There's a national FMD actually now it's North American registry for FMD. That other 20 centers in the United States which are participating for patients to have a chance to see an expert who sees FMD routinely, who has a multi specialty care team they work with to help out with things like brain aneurysms, visceral aneurysms, pulsatile tinnitus, severe headaches, and to participate in research I think has value. I think that may not always be available, but I think if you have a specialist In your area who's interested in FMD and I'm just up the road in in Cleveland. But there's others throughout the country. I think, I think there's value for patients with a rare disease like FMD. Yeah, thanks,
Kanny:Heather. One quick question is obviously I know the image, it can have a very classic appearance, especially like in the renal bed, for example. Is that plus a clinical event enough to make a diagnosis? Do we have any, have there been any advances, for example, in genetic testing? Or are there any times you really have to rely on histology to really understand that a patient actually has FMD?
Heather:Well, in the early days of this disease state, it was described in the 1930s, actually in a pediatric patient at Johns Hopkins who had an aphrectomy. It was an early days of diagnosis was by histopathology. In the modern era, however, it's Almost exclusively by imaging. So even in my center, which is a high volume FMD center It's maybe once a year that we actually get histopathology. So we are really relying on imaging Similarly as of this time, there's no biomarkers for FMD And although there have been advances in terms of understanding the genetics and genetic markers for FMD, and there's a large international consortium of investigators working on this, we do not yet have a genetic test or a blood test. So we're really relying on imaging. I will say as we talk about imaging, FMD overlaps with many other conditions on imaging studies. So Every woman who has a carotid dissection is not a patient with FMD. I mean, you can just have dissections without FMD. Dissection is on the differential. Atherosclerosis sometimes can mimic FMD. Vasculitis can mimic FMD. There's other Arterial syndromes like Loeys Dietz syndrome, vascular Ehlers Danlos that can mimic FMD. There's some imaging artifacts actually with reformatting or or beam hearting artifact from dental work that can mimic beating of FMD. So it's imaging and then a knowledgeable person reviewing the imaging to confirm the diagnosis and avoiding some of the pitfalls of things that overlap with FMD on imaging. So Heather, one other
Ellen:question is, is once you make that diagnosis in that individual patient, you said we don't really have great genetic markers or genetic testing. What kind of screening do family members, if any, need? And, and at what point do you start screening
Heather:people for this? It's a great question. It's one that, I think we're is evolving. There was an international consensus. Actually, it's if anyone's interested. There's an international consensus on FMD led by myself and Alexander pursue of Belgium, which was published in 2019 and is available for free download on the journal vascular medicine and At the time of publication, that group looked at the literature and really felt like we cannot broadly recommend screening of family members for patients with FMD. Because if you look at the data, such as in the United States, or now I should say North American FMD registry, among the people in the registry with FMD, less than 10 percent have an affected family member with FMD. What's interesting is a quarter of patients with FMD in the registry have a family member with an aneurysm. So there may be, there clearly are genetic mechanisms and familial mechanisms. I actually published a few years ago a set of ladies I just saw. Within the past week who are identical twins who have FMD and they actually have FMD in the same spots, carotid and renal, although one has brain aneurysms and one has splenic aneurysms. So clearly there are familial FMD cases and families. But it's complicated and in, in most cases, family members are not affected. So what this consensus recommends is not screening all family members for FMD, but focusing on history and physical exam really. So if the family members have symptoms, they have pulsatile tinnitus, they have migraine headaches, they have hypertension at an early age, those folks should be screened. I think the one caveat I'll throw in there is if I meet a patient with FMD who has aneurysms, and especially if they have a family member with aneurysms, I would recommend other family members be screened. That's
Kanny:fascinating. Fascinating case you mentioned. Just 1 very quick question before we spend the last 5 minutes talking about scads specifically is about serial imaging. You know, like a lot of cardiologists, I have a panel of patients who have gone through a coronary dissection had a good recovery and we see them and they. You know, as you know, do very well clinically over time. Generally if they've had a negative initial screen, I assume at some point it's worth re imaging them as well as your established FMD patients as well. And would you basically stick to C. T. A. Combined with occasional duplex studies for that?
Heather:Yeah, I say, Kenny, I would say the guidelines really do not address this or the scientific statements. But I like you do for my patients with scad. Where there's no other explanation and they have initial imaging that's negative for a number of those patients. I've obtained repeat imaging at the 5 to 7 year mark or so. And similarly, for patients with FMD, let's say they have carotid FMD. and they don't have any disease elsewhere on that negative imaging initially. I have had patients where again, like five to seven years later, I've done a repeat CTA and I have anecdotally very few, but occasionally have picked up like an incidental Incident new aneurysm in those patients, but current scientific statements and guidelines don't really address that. And in general, we don't do frequent serial CTAs on people who've had SCAD.
Kanny:So, Ellen, I have a question for you. I know you're have a big interest in heart disease in women and in your clinic there. As we kind of spend our last few minutes talking more specifically about coronary dissection, do you mind reminding our listeners, as a cardiology consultant, when should bells be going off in our head that this may not be a typical, ischemic event and may be more likely to be a coronary dissection? Well,
Ellen:certainly we do see scattered or, or spontaneous coronary dissections more commonly, I think, in women. And oftentimes we have to remember that if we have our postpartum or peripartum patients at times who have chest pain, they may have any a coronary dissection as well. So I think, you know, as people get older. You start thinking usual things, common things are common. You think of atherosclerotic disease, but certainly in patients who you are surprised that they're coming in with an acute coronary event, you look at the cath films and you have to look very carefully for the dissections at times, but, but, but having a high suspicion is something that's really important in those people who you're, you're pretty surprised that they're coming in, particularly in women coming in with these syndromes. Is that
Heather:your experience, Heather? Yeah. Yeah, for sure. I think you have to have the suspicion. You have to really scrutinize those calf films. I think, as mentioned, I would particularly give a shout out to Jacqueline saw at Vancouver General Hospital, who's done a lot of work on the angiographic classification of scad and raising awareness of scad in the calf lab. And the scads have these very distinctive patterns. They're actually divided into three groups. And I think, With rate giving a classification system and raising awareness in the, in the interventional community, there's a lot more awareness of SCAD. It's actually, it's interesting. I've actually had a few patients who were labeled as having Takotsubo or myocarditis, but when you get the cath films and you really scrutinize, there's that type two SCAD of the LAD. There's that branch OM SCAD. So I think you really have to have a high index of suspicion and really scrutinize those calf films. And I, I'm not an interventional cardiologist, but I have great relationships with my colleagues and we'll, my, my interventionalists, and we'll share films back and forth. And really, do you think this could be SCAD and really look for these classic types of SCAD on the imaging? So, Heather
Kanny:My understanding as well, is that for more recent series. FMD and SCAD that, the age range is a bit wider than maybe we thought in the past and that, this can occur in in middle age or elderly patients. Has that been your experience with the more patients that you see as well? For sure.
Heather:I think for SCAD, I think, I think when we start seeing really SCAD, sometimes there's dissections that are related to an atherosclerotic lesion, but We absolutely are seeing a wider range of ages for both FMD
Kanny:And Ellen, with you, you as an imager you know, my understanding in reading CCTA is that it just is much more challenging to distinguish a dissection from other types of occlusion. Has that been your experience, too, so that we still have to kind of rely on angiography to really. kind of confirm that a dissection is occurring versus, say, a CCTA?
Ellen:I think for the most part, we do rely on the invasive angiography and looking at those films very carefully. I think maybe over time, we might be able, as imaging with CCTA gets better over time and even more and more granularity, it might be something we can use. But I think for the most part, at the current time, it's with invasive angiography that we'll make that
Heather:diagnosis. And I will just add on that, if I may, that I think coronary CTA in my own experience has been, has been able to detect major SCAD. I mean main LAD SCAD, main RCA SCAD. I think it is really challenging with the smaller branches. Yeah, I
Kanny:agree as well. So maybe in the last couple minutes Heather, we can just talk a little bit about management of. Yeah, scared. I think I think most of us clinicians who are now kind of familiar with the condition who see it as a consultant understand that, patients do very, very well with conservative management. I think our interventional colleagues have also learned over the years that intervening can be problematic. And that most patients do well, is that still kind of the current framework when we make this diagnosis to focus on conservative management? And then what medical therapy specifically has has some evidence base, and improving outcomes here?
Heather:Well, I, I. I do think there's still opportunity for recognition of SCAD in the cath lab and appropriate management. As a referral center for SCAD and participating in, we have, there's actually an international SCAD registry for which we're a site. We see a number of patients and I have seen even recently patients who had a SCAD for whom the lesion was not recognized as SCAD and there was actually good flow you know, TIMI 3 flow. But there was an attempt at intervention that led to propagation of dissection, very long segment stenting and a suboptimal outcome. So, I, I think not everyone has learned that. We need to recognize SCAD and that the majority of patients with SCAD, especially if they're not having ongoing chest pain. If there's to me good to me three or maybe to me to flow can be managed conservatively. That being said, there are patients who having. STEMIs who have occluded LADs, who are having ventricular tachycardia, who are critically ill, who need revascularization. It's, it's beyond my strike zone to address techniques, but there is a subset of patients who do need revascularization. We've had a few patients who've had very severe SCAD, very high risk SCAD, left main SCAD who needed even emergency coronary artery bypass grafting. So a subset of patients We'll need intervention, but you're I completely agree. The large majority can be managed medically generally with anti platelet therapy beta blockers blood pressure control. There's a lot of interest in ongoing research in terms of the optimal anti platelet regimen for patients with scab. There's a study that. We did with the iSCAD registry that showed there's a lot of heterogeneity in terms of what regimens are being prescribed, even among the SCAD registry sites dual versus single, what combinations of dual, and there's really no clear data. There's been. Some data that has suggested that perhaps a dual antiplatelet therapy may not be the best therapy for patients who have SCADs, especially the types, the most common type of the type 2 SCAD, which is that tapered lesion due to a mural hematoma, and maybe single antiplatelet therapy is better. But I think there are people in the international FMD research community who are hopefully designing and implementing and getting funded some research studies to address this. But for now, it's antiplatelet therapy, definitely beta blocker and blood pressure control.
Kanny:Awesome. Well I'd love to talk about so many other aspects of this because I think it's been a great discussion. I think we're kind of up against our time. I do want to remind our listeners. I will put, you know, the references that you alluded to Heather in our show notes with a couple of review articles as well. For a further reference, I guess I just want to thank you both. I think it's been a great discussion. I think it's great to have you as a resource here in Northeast Ohio and for Ohio in general. And we look forward to following progress as we learn more about this condition. Thanks to questions like you. So thanks Ellen. And thanks Heather. Both for joining us today. Thank you. Kenny.
Heather:Thank you.
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