Diagnosis and Management of Pericarditis

Podcast Transcript

Announcer:

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

Allan Klein, MD:

Hello, I'm Dr. Allan Klein from Cleveland Clinic. I direct the Pericardial Center, and with me is Dr. Debbie Kwon. Debbie, tell me about yourself?

Deborah Kwon, MD:

Yes. My name is Debbie Kwon. I'm a cardiologist at Cleveland Clinic as well, and have the pleasure of being able to work with Allan in the Pericardial Center. I'm also the Director of Cardiac MRI at Cleveland Clinic.

Allan Klein, MD:

There's a new ACC concise clinical guidance document on pericarditis. I understand there's some new definitions. I understand also there's a new ESC guideline on pericarditis and myocarditis, so a lot of action in this field. How would you approach, with those new guidelines, pericarditis? Some new definitions or things you want to talk about with that?

Deborah Kwon, MD:

Yeah. Yeah. Well, maybe I can briefly talk about the ESC guidelines, what changed there, and then you can speak on the ACC guidelines as you were intimately part of that, one of the leading authorities and authors on that.

I think the novel thing with the ESC guidelines is they actually decided to put things into a continuous spectrum. In the past, it was pericarditis and then myocarditis, whereas now they're recognizing that it's an inflammatory pathway that can impact both the myocardium and the pericardium. It's a continuum, an inflammatory continuum. When patients come for chest pain, we should be thinking of both of those entities and the type of EKG, the blood work with troponin, ESR (erythrocyte sedimentation rate), CRP (C‑reactive protein). In the patients where there's significant concern, a cardiac MRI can be used to diagnose both myocarditis and pericarditis.

Allan Klein, MD:

I think they call that IMPS. Inflammatory myocardial pericardial syndrome, IMPS.

That's the ESC guidelines. Now, with the ACC guidance document, we came up with some new definitions. To diagnose pericarditis specifically, you need the classic pleuritic chest pain going to your left shoulder, and then you need two or more parameters. Now, in these new guidelines, CRP, blood tests, and CMR (cardiovascular magnetic resonance) or CT for evidence of inflammation are raised equal to the other clinical guidelines. You need two of four of those things.

For example, you had the classic pain, and you had elevated CRP and a positive MRI. We call that definite. If you just have the pain and maybe you just have a large effusion, it's possible. If you just have the pain with nothing, it's unlikely. That's a little bit stronger, a stronger fit for the diagnosis.

We do a lot of baseline testing. The standard things, as Dr. Kwon mentioned, EKG, an echocardiogram, blood tests, including CRP or sed rate, troponin. People who we suspect have autoimmune disease may get an ANA or rheumatoid factor as well.

Not everybody needs an MRI, but maybe you could just mention where MRI can be useful, like for the complicated cases or to the rule-outs. Where do you think the MRI would play a role?

Deborah Kwon, MD:

I think when we see patients coming in with pericarditis and their heart rate is not super elevated, it's very classic symptoms – their ESR, CRP are not that elevated, but they're elevated – those patients probably can be treated with empiric therapy with colchicine and NSAIDs.

But I would say that the patients who definitely have recurrent pericarditis, where the baseline therapies are not resolving their symptoms, or on echo, if you start to see constrictive physiology, or if there is a very large pericardial effusion, and you're concerned that there may be more significant pericarditis. I would also venture to say patients with very elevated heart rates and fever. Those suggest that maybe there's more severe pericarditis or inflammation that would be refractory to the typical doses of colchicine and NSAIDs.

Allan, in your practice, when do you decide to pull the trigger and get the cardiac MRI?

Allan Klein, MD:

Okay. In my practice, similar to yours, a lot of the patients, over a third of our patients, are from out of state, and they're coming with chest pain. Often, we anticipate what they may need, but these are all very complicated cases. In complicated pericarditis, multiple recurrences, failing the standard, you're thinking of escalating therapy to the biologics. I think multimodality imaging, especially CMR, would be very, very important because if it's very severe, you want to escalate. If it's not so severe, if you don't find too much, maybe you could stay with what you're taking.

The other thing in my practice, similar to yours, nowadays, a third of the people coming with chest pain may not even have it. What do we call it? We call that fake pericarditis. In other words, they're assuming they have pericarditis, but we look at it very objectively. We don't see elevated CRP. They never really had significant effusion. The MRIs are negative on a few occasions. They got chest pain, but it doesn't mean it's always pericarditis. You don't want to give them a biologic if that's not what they have because there could be some side effects. So, it's a good rule out as well.

Deborah Kwon, MD:

Yes.

Allan Klein, MD:

Maybe you could talk about how often you have to do these now? This is an expensive test. Not everybody has access to this or has the right technique. If somebody was in an active case, very hot LGE (late gadolinium enhancement) and edema, and you put them on therapy. Over the next two years, probably do it once or twice, just to see if it got better. People want to know that.

Deborah Kwon, MD:

Yes, I think the cardiac MRI can be very helpful to guide the treatment. Especially when they're coming for the first time, their first recurrence, or even their second or third recurrence, and it's uncertain how bad the inflammation is and how far you have to escalate the therapies to really resolve the pericarditis. I think that's where cardiac MRI can be especially helpful because it's no longer guessing. Do I go all the way to the top dose of prednisone, or do I now start the biologic? Sometimes it's not clear. Especially when patients are already on some treatment, the ESR, CRP may not be as reflective of the actual amount of inflammation that there is.

I think I also wanted to highlight with cardiac MRI, there can be some heterogeneity in terms of the technique at different centers. What we've noticed is that the fat surrounding the heart can also look enhanced. Sometimes it can be mistaken for pericardial inflammation and can masquerade, I guess, as pericarditis. As referring doctors who don't read cardiac MRI, that can be confusing because they're just going based on their report. What we have identified is that it's really important to suppress the fat on the cardiac MRI so that you can discern the difference between normal epicardial fat versus inflammation. This is really important again, because it's guiding the treatment.

Allan Klein, MD:

Now, sometimes we see patients coming in with elevated troponins. They may have a lower ejection fraction, and the echo, the MRI may suggest myocarditis, reduced EF. How often does that occur?

Deborah Kwon, MD:

Yes, that's a fantastic question. In fact, just before we came and did this podcast, one of my colleagues asked me to review a cardiac MRI of someone who just had an ASD (atrial septal defect) closure, had pericarditis and got an MRI. This patient has ring-like LGE around their myocardium. This patient has myopericarditis and probably has a genetic underlying issue that caused the ring-like LGE or enhancement that is also associated with their pericarditis.

When it comes to myocarditis, there's this idea of a two-hit hypothesis where patients may have a predilection towards, or they have a genetic cardiomyopathy that predisposes them to, potentially a viral infection and then manifesting in a myocardial injury because they have that predisposition. Because many of us get viral infections and we don't get myocarditis, but it may be the people who actually develop the myocarditis that have a genetic predilection to that.

In my practice, I've started to get genetic testing for underlying cardiomyopathy in anybody who has myocarditis so that we can identify those patients. Some of these patients with specific genetic mutations should be treated differently or have different risk profiles, and then their family members can also be treated as well. I think it's important for us to identify when the patients have myopericarditis. That's a different risk profile than just pericarditis alone.

Allan Klein, MD:

Dr. Kwon mentioned some complications that you can have with this. Most pericardial syndromes, you can have acute, you can have recurrent, you can have fluid effusions, tamponade, and what we call constrictive pericarditis. The constrictive pericarditis comes in two forms. One is burnt-out. Patients are all swollen. They have ascites. They have leg swelling. Often, they may need surgery.

But there's a group that we may see early on that are still inflamed. We call that transient inflammatory constriction, where anti-inflammatories may have some role. Debbie, I'm sure you've seen some of these folks. I guess if you catch it early, you could add some anti-inflammatories, see if you could help with that and maybe avoid the surgery. That's an interesting group.

Deborah Kwon, MD:

Yes, definitely. I think that's another key patient cohort. When they come in with constriction, and you get a cardiac echo, you might not be able to tell the difference between a transient constriction, which is all inflammation, versus burnt-out constriction, which is all fibrotic.

In these situations, actually, CT scan should also be added into the diagnostic pathway because when you see calcification around the heart, that really clues you in that it’s more likely they're now in the burnt-out stage. I'd say that's probably the Achilles heel of cardiac MRI is that we're not able to see the calcification around the pericardium as well as with CT. In that scenario, when somebody's coming in with constrictive physiology, obviously the first-line test is always echo. I would say MRI, but then they should also get a non-contrast CT to look for calcification.

Allan Klein, MD:

A message also for the surgeons out there would be not to do the surgery when it's very inflamed because that's not a good outcome. For the surgeon to peel an orange, take off the lining, when it's all inflamed, you could pull off some other things, some important vital structures. We often say calm it down with the anti-inflammatories, whether it's NSAIDs and colchicine, biologics, even possibly steroids. Calm it down and either get it ready for the surgery, or maybe you never need the surgery. There's a lot of research in that area.

Talking about research, we're the hub of a lot of clinical trials. As you know, Cleveland Clinic was involved with the pivotal trials for the biologic called the RHAPSODY trial, and we were involved with a MAvERIC-Pilot study, that's on CBD. It's not THC. It's hemp. There are other trials out there.

There's a new biologic. The trade name is KPL, and it's for people with active recurrent pericarditis. It's a new monoclonal antibody, and there's a substudy of that looking at for stable patients. There's even a pill that will allow anti-inflammatory properties that we're testing as well. I mentioned the CBD. It's a solution of hemp that we're testing on people that have trouble getting off the biologics.

There's other trials in development. It's a really hot area. For those clinicians out there, if you have a patient you think may be eligible, definitely do refer them. We have a whole research team that could screen them and look at this. I can say, in this area, we're making a big impact. It's an old disease, but it's a new renaissance, both from diagnostics, whether echo, MRI, lab tests. Now we have the therapies. We have all these new biologics, and we have surgery. We have it all covered here at Cleveland Clinic.

We have excellent surgery at the Cleveland Clinic. You want to talk about this radical pericardiectomy that they do here? Not every center could do that, right?

Deborah Kwon, MD:

Yes. Correct. Yes. In the past, we used to do partial pericardiectomy. I think many centers are still doing the partial pericardiectomy. We noticed that constriction can recur if there's still some of the pericardium left behind, both when it's fibrotic, but also inflammatory. Many of the surgeons here at Cleveland Clinic are doing a radical pericardiectomy, and that's really demonstrated to have improved outcomes because now none of the remaining pericardium could continue to impact the hemodynamics, as well as the inflammatory component.

Allan Klein, MD:

I think we do roughly between 50 and 60 radical pericardiectomies per year. You have to be a highly trained surgeon. They often do it on cardiopulmonary bypass. As Dr. Kwon mentioned, they remove most of the pericardium. No pericardium left behind because the pain can still come back. I would say it's very, very successful. That's definitely an option.

In somebody who's had this, there are two scenarios. One would be the constriction where you have heart failure that’s not getting better, or such intractable recurrent pain every time you taper the medicine that comes back. After several years, surgery is definitely an option, and we have excellent surgeons who do this. Not every hospital has that, and we have that. We're blessed to have that here at Cleveland Clinic.

Deborah Kwon, MD:

Yeah. At Cleveland Clinic, as Dr. Klein mentioned, I think recently we published our outcomes. The patients who had radical pericardiectomy had very good outcomes compared to, in our past literature, demonstrating poorer outcomes in the patients going for partial pericardiectomy. In the historical literature, many times it’s because maybe they have waited too long before they actually went for surgery.

Here at Cleveland Clinic, we're very blessed to have a very wide array of therapeutics, many clinical trials that are ongoing. But even in the most refractory cases or the burnt-out cases, we have phenomenal surgeons who provide excellent outcomes for the full array of the types of treatments that we can offer.

Allan Klein, MD:

Very good. Thank you.

Deborah Kwon, MD:

Great. Thank you.

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