Identification of a novel presumed cardiac sarcoidosis category for patients at high risk of disease

Rosenbaum, Andrew; Kolluri, Nikhil; Elwazir, Mohamed Y.; Kapa, Suraj; Ezzeddine, Omar F. Abou; Bois, John P.; Chareonthaitawee, Panithaya; Schmidt, Tyler; Cooper, Leslie T.

doi:10.1016/j.ijcard.2021.04.022

Cited by 36 publications

(28 citation statements)

References 31 publications

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“…Histology is characterized by the presence of epithelioid granulomas with associated giant cells and lymphocytes, well-defined areas of inflammation and fibrosis, and absence of significant myocardial necrosis ( 1 ). Therefore, quite often the diagnosis of CS can be supported by clinical and imaging findings with contrast-enhanced CMRI and FDG-PET ( 1 , 173 ). Based on this assumption, it must be accepted that if the diagnosis of CS relies on clinical and imaging criteria, we could face the risk of treating with immunosuppressive therapies patients with other inflammatory or non-inflammatory cardiomyopathies that are potentially less responsive to long-term steroid therapy or might be potentially harmed by the treatment.…”

Section: Cardiac Sarcoidosismentioning

confidence: 99%

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

et al. 2022

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The field of inflammatory disease of the heart or “cardio-immunology” is rapidly evolving due to the wider use of non-invasive diagnostic tools able to detect and monitor myocardial inflammation. In acute myocarditis, recent data on the use of immunomodulating therapies have been reported both in the setting of systemic autoimmune disorders and in the setting of isolated forms, especially in patients with specific histology (e.g., eosinophilic myocarditis) or with an arrhythmicburden. A role for immunosuppressive therapies has been also shown in severe cases of coronavirus disease 2019 (COVID-19), a condition that can be associated with cardiac injury and acute myocarditis. Furthermore, ongoing clinical trials are assessing the role of high dosage methylprednisolone in the context of acute myocarditis complicated by heart failure or fulminant presentation or the role of anakinra to treat patients with acute myocarditis excluding patients with hemodynamically unstable conditions. In addition, the explosion of immune-mediated therapies in oncology has introduced new pathophysiological entities, such as immune-checkpoint inhibitor-associated myocarditis and new basic research models to understand the interaction between the cardiac and immune systems. Here we provide a broad overview of evolving areas in cardio-immunology. We summarize the use of new imaging tools in combination with endomyocardial biopsy and laboratory parameters such as high sensitivity troponin to monitor the response to immunomodulating therapies based on recent evidence and clinical experience. Concerning pericarditis, the normal composition of pericardial fluid has been recently elucidated, allowing to assess the actual presence of inflammation; indeed, normal pericardial fluid is rich in nucleated cells, protein, albumin, LDH, at levels consistent with inflammatory exudates in other biological fluids. Importantly, recent findings showed how innate immunity plays a pivotal role in the pathogenesis of recurrent pericarditis with raised C-reactive protein, with inflammasome and IL-1 overproduction as drivers for systemic inflammatory response. In the era of tailored medicine, anti-IL-1 agents such as anakinra and rilonacept have been demonstrated highly effective in patients with recurrent pericarditis associated with an inflammatory phenotype.

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Section: Cardiac Sarcoidosismentioning

confidence: 99%

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

et al. 2022

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“…To have a clinical diagnosis of CS, participants must have met the 2014 Heart Rhythm Society consensus statement definition 13 . Patients with presumed CS, those without histologic evidence of CS but had cardiac magnetic resonance (CMR) or cardiac positron emission tomography (PET) with imaging findings consistent with CS and unexplained high‐grade AV block or ventricular arrythmia, were also included 14 . Participants must have been at least 18 years of age at the time of diagnosis and provided research consent.…”

Section: Methodsmentioning

confidence: 99%

“…Given inclusion of patients since the year 2000, advancement in CRT device therapy, cardiac imaging and medical therapy may limit clinical response to CRT. We also included 16 patients with presumed CS It is possible that false‐positive classification may occurred, such as patients having arrhythmogenic cardiomyopathy 14 . Lastly, as our center is a tertiary academic center, there may be a selection bias for patients with more refractory disease.…”

Section: Limitationsmentioning

confidence: 99%

“…We retrospectively reviewed all patients with a CS diagnosis who underwent implantation of a CRT device at the Mayo Clinic (Arizona, Florida, and Minnesota campuses) between January 13 Patients with presumed CS, those without histologic evidence of CS but had cardiac magnetic resonance (CMR) or cardiac positron emission tomography (PET) with imaging findings consistent with CS and unexplained high-grade AV block or ventricular arrythmia, were also included. 14…”

Section: Study Cohortmentioning

confidence: 99%

“…We also included 16 patients with presumed CS It is possible that falsepositive classification may occurred, such as patients having arrhythmogenic cardiomyopathy. 14 Lastly, as our center is a tertiary academic center, there may be a selection bias for patients with more refractory disease. However, this study does significantly contribute to the limited literature on the topic.…”

Section: Limitationsmentioning

confidence: 99%

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Cardiac resynchronization therapy response in cardiac sarcoidosis

Shabtaie

Sehrawat

Lee

et al. 2022

Cardiovasc electrophysiol

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Introduction: Cardiac sarcoidosis (CS) is a nonischemic cardiomyopathy (NICM) characterized by infiltration of noncaseating granulomas involving the heart with highly variable clinical manifestations that can include conduction abnormalities and systolic heart failure. Cardiac resynchronization therapy (CRT) has shown significant promise in NICM, though little is known about its efficacy in patients with CS. Objective: To determine if CRT improved cardiac remodeling in patients with CS. Methods: We retrospectively reviewed all patients with a clinical or histological diagnosis of CS who underwent CRT implantation at the Mayo Clinic enterprise from 2000 to 2021. Baseline characteristics, imaging parameters, heart failure hospitalizations and need for advanced therapies, and major adverse cardiac events (MACE) were assessed.Results: Our cohort was comprised of 55 patients with 61.8% male and a mean age of 58.7 ± 10.9 years. Eighteen (32.7%) patients had definite CS, 21 (38.2%) had probable CS, while 16 (29.1%) had presumed CS, and 26 (47.3%) with extracardiac sarcoidosis. The majority underwent CRT-D implantation (n = 52, 94.5%) and 3(5.5%) underwent CRT-P implantation with 67.3% of implanted devices being upgrades from prior pacemakers or implantable cardioverter defibrillators. At 6 months postimplantation there was no significant improvement in ejection fraction (34.8 ± 10.9% vs. 37.7 ± 14.2%, p = .331) or left ventricular end-diastolic diameter (58.5 ± 10.2 vs. 57.5 ± 8.1 mm, p = .236), though mild improvement in left ventricular end systolic diameter (49.1 ± 9.9 vs. 45.7± 9.9 mm, p < .0001). Within the first 6 months postimplantation, 5 (9.1%) patients sustained a heart failure hospitalization.At a mean follow-up of 4.1± 3.7 years, 14 (25.5%) patients experienced a heart

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Endomyocardial biopsy in the diagnosis of cardiac sarcoidosis

Mälkönen,

Lehtonen,

Pöyhönen

et al. 2024

European J of Heart Fail

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AimsWe set out to assess the utility of endomyocardial biopsy (EMB) in cardiac sarcoidosis (CS). Historically, EMB sensitivity in CS is only ≤25%, but comprehensive analyses of its current diagnostic performance are not available.Methods and resultsThe data of 260 consecutive patients with CS (mean age 49 years, 60% female) meeting the Heart Rhythm Society diagnostic criteria were analysed retrospectively. Overall, 216 patients (83%) had undergone EMB, 47 with repeat procedures. EMB overall sensitivity was 38%, rising to 49% after repeat biopsies. On logistic regression analysis, positive EMB was predicted independently by presentation with ventricular tachyarrhythmia with an odds ratio (OR) of 3.8 (95% confidence interval [CI] 1.2–12.0, p = 0.021), left ventricular ejection fraction ≤45% (OR 3.7, 95% CI 1.5–9.1, p = 0.004), elevation of cardiac troponins (OR 2.7, 95% CI 1.1–6.4, p = 0.024), and presence of late gadolinium enhancement in left ventricular mid‐apical septal segments on magnetic resonance imaging (OR 4.1, 95% CI 1.2–13.8, p = 0.024). EMB sensitivity, counting in repeats, was 16% in patients (n = 37) without any independent predictor versus 38%, 60%, 79%, and 88% in those with 1 (n = 76), 2 (n = 62), 3 (n = 33), and 4/4 (n = 8) predictors, respectively. The rate of serious complications was 0.7% without mortality or permanent harm. Positive EMB was not an independent predictor of prognosis.ConclusionThe sensitivity of EMB in CS depends on the extent, activity, and location of myocardial involvement, being the higher the more severe CS is. Its use should rely on weighing the pre‐test likelihood and individual value of positive biopsy against the procedural risks.

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Identification of a novel presumed cardiac sarcoidosis category for patients at high risk of disease

Cited by 36 publications

References 31 publications

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

Cardiac resynchronization therapy response in cardiac sarcoidosis

Endomyocardial biopsy in the diagnosis of cardiac sarcoidosis

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