Transcription:
Northwestern Medicine Pericardial Disease Program: Vision, Diagnosis and Treatment Options

Melanie Cole (Host): Welcome to Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. And joining me today is Dr. Mohamed Al-Kazaz. He's an Assistant Professor of Medicine and Cardiology at Northwestern Medicine. And he's here to speak to us about pericardial disease and treatment options provided by Northwestern Medicine.

Dr. Al-Kazaz, thank you so much for joining us today. I'd like you to start by telling us a little bit about the prevalence of pericardial disease, what you've seen in the trends, the scope of the issue that we're discussing here today.

Dr Mohamed Al-Kazaz: Thank you so much, Melanie, for having me and having the time to do this. So, pericardial disease encompasses a wide range of pathologies, and this could range from simple first episode of viral pericarditis, inflammation in the pericardial sac, all the way to constriction pericarditis, which is kind of one of the end stages of the disease that requires surgery.

So, it's getting more interest, given the evolution and the advances of the field from diagnostic and therapeutic standpoint. It's a disease that's very common, particularly in the setting of chest pain. For example, 5% of patients who come to the emergency room for some sort of chest pain end up having pericarditis. And it's quite an illness because if patients don't recognize it and providers don't recognize it and provide the early aggressive treatment and not just short courses and kind of taper quickly, they might end up with recurrences. Because from those who, let's say, get pericarditis, which is one of the most common forms of pericardial disease, 1% to 2% end up with cardiac tamponade, which is a life-threatening emergency. Fifteen percent of those patient might end up with myocardial involvement myocarditis, and that could be ranging from mild cases to fulminant. And from those with pericarditis disease, some of them might progress into a more complicated case of pericarditis rather than just the simple, straightforward run-of-the-mill we deal with. Around 15% to 30% of those might have recurrences of pericarditis. And from those, around 6% or so might have multiple recurrences. We don't know exactly how many of them will progress to chronic constrictive pericarditis, whether with reversible or irreversible etiology, but maybe in the realm of 1% to 2%.

The good news is we have a lot we can do to intervene and offer treatment for those. However, the scope of pericardial disease is beyond just acute pericarditis. We have pericardial effusions. We have pericardial congenital issues or cyst or otherwise. And what we can look for those patients is beyond the obvious, look for etiologies. Sometimes pericardial effusion or pericarditis could be the first presentation of a systemic illness, and a thorough detailed evaluation can reveal some of them and provide the appropriate care for them.

Melanie Cole (Host): Dr. Al-Kazaz, as clinicians are often faced and you mentioned about diagnostic with several diagnostic questions relating to the various pericardial syndromes, is there a criteria for diagnosis that's been established? Under what would you diagnose this issue? Please speak about this and why it remains challenging based on the vast spectrum of manifestations.

Dr Mohamed Al-Kazaz: Yeah. So, every disease entity of pericardial disease has its own diagnostic criteria. For example, early recognition of the symptoms could be difficult. Some of those patients, some of them can be misdiagnosed as pericarditis, and they don't have it, and they can go that direction of therapy and diagnostics. And the same applies to constriction, which is even a harder diagnosis.

But we can start with pericarditis. The straightforward is people need to have a few of these criteria. Typical chest pains worse with breathing, what's called pleuritic chest pain, kind of positional. They prefer leaning forward over sitting back. These positional characteristics of the chest pain can help guide that diagnostic therapy. But obviously, we have to look at other causes, some EKG changes, whether diffuse ST elevations. But even those, they might be tricky because some younger people might have them as a normal variant or early repolarization, looking and detecting effusions on echo could be supportive of that. On examination, hearing a friction rub from the inflammation, but that can be challenging. That can be supplemented by biomarkers such as inflammatory markers, CRP or, even in this time and age, a cardiac MRI, which has really been a huge advancement in the field from a diagnostic and therapeutic standpoint.

In terms of constriction, that requires really early recognition and understanding, especially in a patient with heart failure who don't have a clear reason or their workup reveals that the myocardium is okay, it's unclear if it's myocardial or something else. You have to have a high degree of suspicion and using the right tools. That's why we're trying to build a center of excellence for pericardial disease treatment as we do for mitral valve disease or coronary artery disease, where we have all the needed specialties, especially as the complexity of those pericardial disease patients is expanding. We need to have dedicated expertise to see them, to help recognize them early, whether it's at the level of the referring physicians or, let's say, a primary care, a cardiologist, sends a patient for shortness of breath, for an echo. And if the technologist notices some changes, we're trying to teach them and we're trying to expand the expertise, especially in our centers, to recognize them because the clinician might not have recognized that constriction is the reason why this patient is having recurrent ascites or right sided heart failure. Therefore, the early recognition, the right imaging, referring to the right expertise at centers of excellence will, one, improve the outcomes for the patient from not just a survival standpoint, but also from a quality of life standpoint.

I speak about these two disease entities, especially the recurrent pericarditis and the constriction, because those are the most challenging aspects of it and require multidisciplinary approach to it. Similarly to effusions, you know, I mean, they could be simply related to inflammatory process or malignancies. What treatments do we offer them, whether surgical or medical? It's a complex decision-making, especially as those patient ends up being very complex from a cardiac standpoint and a medical standpoint.

Melanie Cole (Host): Wow, you made such good points. And it is such a complex situation that does require that multidisciplinary comprehensive approach. So, tell us about your experience in treating patients with the various pericardial diseases, and tell us a little bit about some of the options at Northwestern Medicine for treatment.

Dr Mohamed Al-Kazaz: So, Northwestern Medicine is committed to a patient-centered and multidisciplinary approach. It's a very collaborative environment. And this is kind of the essence and the heart of cardiovascular disease treatment in general and pericardial disease in particular. So for example, to get the right diagnosis, we need multidisciplinary interaction between the cardiologist, the specialist in pericardial disease, as well as the cardiac imaging staff and faculty who have expertise in looking at some of the nuances, differentiating pericardial fat from fluid, from thickening, from different aspects in MRI. So for example, from a diagnostic standpoint, we need state-of-the-art, which we have in cardiac imaging, whether echo, the expertise to do it, cardiac MRI, CT, besides our research protocols, we're just talking about the center of care clinical aspects. So, good, accurate, well done imaging is the cornerstone of it.

Then diagnostically, it's followed by specialized interventional cardiologists who would do dedicated studies for simultaneous right and left heart catheterization to confirm the diagnosis if needed. At this time and age, our imaging has so advanced with the right expertise in discussion, we might not need to do that invasive diagnostic testing, but it's an option in some of those less clear cut cases.

The second aspect of it in terms of what do we do with those patient once we have them, the first thing, whether let's say is constriction or recurrent pericarditis is having the right multidisciplinary people seeing them. For example, if their recurrent pericarditis is driven by an autoimmune disease or a systemic process, we get our rheumatologist to see them and chip in and kind of have a discussion about what's the best next step. If it's a constriction case with no clear identifiable etiology and based on MRI imaging, there is no clear reason to suspect reversibility, and we think it's permanent and burned out, we need to have pericardiectomy and we need those to be a radical pericardiectomy, which means complete removal and resection, and only a few surgeons can do as a good job as we want because of the risk of recurrence and the outcomes from those surgeries in the past were not great. But now, with the new advances in expertise, early recognition and the skills of our surgeons, we have been able to have excellent outcomes. We have Dr. Doug Johnston, the Chief of our Cardiac Surgery, who specializes in pericardiectomy and having the privilege to work with him. He's fantastic and he's so skillful that we have great outcome because reaching the right diagnosis is not as helpful in those constriction pericarditis cases, if we are not able to provide them the therapy with the best outcome and the least amount of risk. So if they need surgery, we end up offering to them at the lowest risk with the highest benefit, so the multidisciplinary team, rheumatology, cardiology, cardiothoracic surgery, interventional cardiology, cardiac imaging, as well as oncology in some cases, if those were related to cancer and cancer, etiologies or malignancies.

As you can see, it takes a village to take care of some of those patients because they're so complex and we work closely with each other to come as a heart team to make the right decision for them, let's say, when they need surgery. However, some of those recurrent pericarditis patients or some of those patients with reversible constriction, they might have a big component of inflammatory process, beside the systemic illnesses and other etiologies and they could be idiopathic, and we offer them advanced therapies besides just the typical ibuprofen and NSAID and colchicine. Historically, we used steroids for some of those refractory patients. But the paradigm has shifted for treatment of those pericarditis patients to pull the trigger earlier for some of those biologic agents that we have, whether rilonacept has been shown in Rhapsody trial or anakinra, which target IL-1 interleukin, some of the pro-inflammatory cytokines we have, and with biodifferent mechanisms, they have shown freedom from recurrences of pericarditis, much better than other traditional therapies and we're able to wean them off steroids or high-dose ibuprofen or indomethacin, which have kind of a worse side effects profile, especially some of those patients who are younger.

And so, we have advances from three aspects. One is in imaging, because we're using our imaging like MRI, not just to diagnose them, but to quantify the disease, to help guide the therapy. Is there reversibility? Is there inflammation? Can we use biologic as some of the stuff I mentioned? So, it's guiding the therapy as well. We have advances in our therapeutic option beyond the traditional anti-inflammatories like indomethacin or NSAID or colchicine for the recurrent pericarditis. We have these rilonacept and anakinra. We have advances in our surgical technique for those burned-out constrictive pericarditis with recurrent heart failure or those with recurrent pericarditis refractory to all lines of therapy, we end up doing some of, in those cases, pericardiectomy, removal of the pericardium. We need to do a good job doing radical complete removal and so on. We even have expertise in dealing with radiation heart related-disease because our cancer patient, let's say lymphoma patient in the past, they used to get higher dose of radiation that can impact the myocardial as well as the pericardial disease, and those patients are very risky to take a heart surgery. But with careful perioperative management, we can get them through it safely with the best outcome possible. But it takes a village and it takes a lot of people who have great expertise. That's why having a center of excellence is key in dealing with those complex pericardial cases.

Melanie Cole (Host): This is such a fascinating topic and what a real multidisciplinary approach that is. You offered so many options that really make Northwestern Medicine so unique. As we wrap up, Dr. Al-Kazaz, I'd like you to speak about any advancements that are on the horizon. Anything you would like to see or research, publications, anything you'd like other providers to know about? And leave us with one parting expert piece of information as this can be diagnostically challenging for clinicians. Please just leave your best advice for providers listening who may treat patients with pericardial disease.

Dr Mohamed Al-Kazaz: The field is evolving and there's a lot of interest in pericardial disease, having a Renaissance. And the advances we see that I'm looking forward to on couple levels, one is a basic science level where we're trying to figure out some of the mechanistic reasons for pericarditis or effusions and understanding the role of, let's say, of the lymphatic system, finding ways to avoid draining some of those effusion, with procedures like pericardiocentesis and using medical therapies is still early on, but it's ideas that hopefully down the road prevent procedures and allow us to offer treatments to patients without being invasive. It's early on from basic science, all the way to translational and clinical research where we have advances in imaging that's not only just looking at edema with MRI or inflammation, but looking at the quantification of them looking at earlier recognition, guiding the therapy. And the third aspect to it is the biologics, those biologics are still kind of third line, let's say, treatment therapy. But the paradigm for treatment is shifting that maybe we should use them earlier on. Maybe those patients with CRP that's elevated, that's an inflammatory marker, maybe we should now wait to use steroids. Maybe we should just pull up the trigger on rilonacept and anakinra before the steroids to stir some of the side effects. There's some limitation to them, but that change in paradigm is interesting and I'm looking forward to see where the field will take it. We're advancing therapies in the pericarditis realm, constriction realm from a surgical and medical standpoint, improving diagnostic and, more importantly, understanding some of the mechanistic issues behind them to see if we can push the field forward.

In terms of advice for patient or providers, whether it is for acute pericarditis, recurrent pericarditis, or even constriction for that matter with reversible or irreversible etiology, I want to say one thing. The earlier of the recognition we have of these processes, the higher the chance we can find a reversible etiology on systemic or organ-specific levels, such as the heart, because it'll allow us to give maybe more aggressive anti-inflammatories, recognize the systemic illness that was missed and treat them and avoid the advanced therapies such as radical pericardiectomy offered surgically. So, the earlier recognition, the aggressive therapy early on are key aspect to the management of pericardial disease. And we do that in collaboration with our primary care doctor, general cardiologist, inpatient, outpatient, because the earlier we recognize it, the more we think of constriction as a reason for shortness of breath or right-sided heart failure, maybe it's not the case, but we should think about it and decide is it worth to work it up or it's unlikely. The reason for that, the more we look for it, the more we're going to find it. And the more we find, the earlier we recognize it, the more aggressive therapy we can offer and hopefully reverse the process before it becomes permanent or we reach the stage of burned-out pericardium, and we need surgical intervention from experts like Dr. Johnston. If needed be, we will take care of it. But the hope is we push the field more and more to earlier recognition and earlier treatments.

Melanie Cole (Host): What an excellent episode this was, Dr. Al-Kazaz. Thank you so much for joining us today and sharing your incredible expertise for other providers. To refer your patient or for more information, visit our website at breakthroughsforphysicians.nm.org/cardiovascular to get connected with one of our providers.

And that wraps up this episode of Better Edge, a Northwestern Medicine Podcast for physicians. Please always remember to subscribe, rate, and review this podcast and all the other Northwestern Medicine podcasts. I'm Melanie Cole. Thanks so much for joining us today.