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Management of Acute Myocarditis and Chronic Inflammatory Cardiomyopathy: An Expert Consensus Document
Open accessAbstract
Myocarditis is an inflammatory disease of the heart that may occur because of infections, immune system activation, or exposure to drugs. The diagnosis of myocarditis has changed due to the introduction of cardiac magnetic resonance imaging. We present an expert consensus document aimed to summarize the common terminology related to myocarditis meanwhile highlighting some areas of controversies and uncertainties and the unmet clinical needs. In fact, controversies persist regarding mechanisms that determine the transition from the initial trigger to myocardial inflammation and from acute myocardial damage to chronic ventricular dysfunction. It is still uncertain which viruses (besides enteroviruses) cause direct tissue damage, act as triggers for immune-mediated damage, or both. Regarding terminology, myocarditis can be characterized according to etiology, phase, and severity of the disease, predominant symptoms, and pathological findings. Clinically, acute myocarditis (AM) implies a short time elapsed from the onset of symptoms and diagnosis (generally <1 month). In contrast, chronic inflammatory cardiomyopathy indicates myocardial inflammation with established dilated cardiomyopathy or hypokinetic nondilated phenotype, which in the advanced stages evolves into fibrosis without detectable inflammation. Suggested diagnostic and treatment recommendations for AM and chronic inflammatory cardiomyopathy are mainly based on expert opinion given the lack of well-designed contemporary clinical studies in the field. We will provide a shared and practical approach to patient diagnosis and management, underlying differences between the European and US scientific statements on this topic. We explain the role of histology that defines subtypes of myocarditis and its prognostic and therapeutic implications.
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The source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“cardiotropic viruses such as adenoviruses and enteroviruses can directly damage the heart, leading to the development of myocarditis”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“certain viruses including HIV, hepatitis C virus, influenza A virus, and influenza B virus are thought to trigger myocarditis by activating the immune system”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“with up to 20% of giant cell myocarditis cases associated with existing autoimmune disorders such as inflammatory bowel disease, thyroiditis, and thymoma.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Giant cell myocarditis and eosinophilic necrotizing myocarditis are traditionally classified as autoimmune myocarditis due to their strong association with existing autoimmune conditions”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“In well-known forms of autoimmune myocarditis such as giant cell myocarditis and cardiac sarcoidosis, macrophages have been identified as a significant contributor to the formation of multinucleated giant cells or granulomas”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measured
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In the citing paper
“suggesting potential alternative pathogenesis such as autoimmunity, hypersensitivity, or medication usage.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Lymphocytic myocarditis is primarily associated with viral infections, characterized by histological observations of myocardial infiltration predominantly by T lymphocytes”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Notably, in some cases of lymphocytic myocarditis, these viruses tested negative, while autoantibodies specific to the heart were present”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“which can be pleuritic and positional, similar to that of pericarditis, or pressure-like mimicking the pain of acute coronary syndrome.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“the most commonly reported symptom is chest pain (82% to 95% of cases)”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Sarcoidosis is a systemic autoimmune syndrome characterized by the formation of nonnecrotizing granulomas, a histological hallmark, found in various organs including the lung and heart.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“an unnoticed history of viral infection may contribute to the migration and activation of autoreactive T cells in the heart. 7,10,31,107”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
Experimental — accuracy not yet measuredThe cited source says what the claim attributes to it.
In the citing paper
“Giant cell myocarditis is a rare but rapidly progressing condition associated with a high risk of death or the necessity for heart transplantation.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
The cited source says what the claim attributes to it.
In the citing paper
“Histopathologic observations reveal myocardial infiltration of multinucleated giant cells along with lymphocytes and eosinophils in this condition.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
The cited source says what the claim attributes to it.
In the citing paper
“various conditions including infections, drugs, hypersensitivity, and autoimmune factors may be associated with the onset and progress of this disease.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [31]
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Citations
Cites
Vos, T., Barber, R. M., Bell, B., Bertozzi-Villa, A., Biryukov, S., Bolliger, I., Charlson, F., Davis, A., Degenhardt, L., Dicker, D., et al. (2015) · The Lancet · doi:10.1016/s0140-6736(15)60692-4 · Cited by 6,414
Caforio, A. L. P., Pankuweit, S., Arbustini, E., Basso, C., Gimeno-Blanes, J., Felix, S. B., Fu, M., Heliö, T., Heymans, S., Jahns, R., et al. (2013) · European Heart Journal · doi:10.1093/eurheartj/eht210 · Cited by 3,479
Self-citationCardiovascular Magnetic Resonance in Myocarditis: A JACC White Paper
Friedrich, M. G., Sechtem, U. P., Schulz‐Menger, J., Holmvang, G., Alakija, P., Cooper, L. T., White, J. A., Abdel‐Aty, H., Gutberlet, M., Prasad, S. K., et al. (2009) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2009.02.007 · Cited by 2,523
Self-citationFulminant Myocarditis with Combination Immune Checkpoint Blockade
Johnson, D. B., Balko, J. M., Compton, M. L., Chalkias, S., Gorham, J., Xu, Y., Hicks, M., Puzanov, I., Alexander, M. R., Bloomer, T. L., et al. (2016) · New England Journal of Medicine · doi:10.1056/nejmoa1609214 · Cited by 2,308
Self-citationCardiovascular Magnetic Resonance in Nonischemic Myocardial Inflammation
Ferreira, V. M., Schulz-Menger, J., Holmvang, G., Kramer, C. M., Carbone, I., Sechtem, U., Kindermann, I., Gutberlet, M., Cooper, L. T., Liu, P., et al. (2018) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2018.09.072 · Cited by 2,059
Self-citationAl‐Khatib, S. M., Stevenson, W. G., Ackerman, M. J., Bryant, W. J., Callans, D. J., Curtis, A. B., Deal, B. J., Dickfeld, T. M., Field, M. E., Fonarow, G. C., et al. (2018) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2017.10.054 · Cited by 1,902
Cardiac Involvement in a Patient With Coronavirus Disease 2019 (COVID-19)
Inciardi, R. M., Lupi, L., Zaccone, G., Italia, L., Raffo, M., Tomasoni, D., Cani, D., Cerini, M., Farina, D., Gavazzi, E., et al. (2020) · JAMA Cardiology · doi:10.1001/jamacardio.2020.1096 · Cited by 1,896
Self-citationMyocarditis in Patients Treated With Immune Checkpoint Inhibitors
Mahmood, S. S., Fradley, M. G., Cohen, J. V., Nohria, A., Reynolds, K. L., Heinzerling, L. M., Sullivan, R. J., Damrongwatanasuk, R., Chen, C. L., Gupta, D., et al. (2018) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2018.02.037 · Cited by 1,608
Self-citationHaslam, A., & Prasad, V. (2019) · JAMA Network Open · doi:10.1001/jamanetworkopen.2019.2535 · Cited by 1,413
Research works printed but not linked: 1
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Lyon, A. R., López‐Fernández, T., Couch, L. S., Asteggiano, R., Aznar, M. C., Bergler-Klein, J. K., Boriani, G., Cardinale, D. M., Córdoba, R., Cosyns, B., et al. (2022) · European Heart Journal · doi:10.1093/eurheartj/ehac244 · Cited by 3,457
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Metadata
- Authors
- Ammirati, E., Frigerio, M., Adler, E. D., Basso, C., Birnie, D. H., Brambatti, M., Friedrich, M. G., Klingel, K., Lehtonen, J., Moslehi, J. J., Pedrotti, P., Rimoldi, O. E., Schultheiss, H.-P., Tschöpe, C., Cooper, L. T., & Camici, P. G.
- Affiliations
- Vita-Salute San Raffaele University (IT)
- Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda (IT)
- University of California San Diego (US)
- University of Padua (IT)
- Ottawa Heart Institute (CA)
- Ionis Pharmaceuticals (United States) (US)
- McGill University (CA)
- Universitätsklinikum Tübingen (DE)
- Helsinki University Hospital (FI)
- Vanderbilt University Medical Center (US)
- IBFM CNR, Segrate, Italy (O.E.R.).
- Institute for Cardiac Diagnostics and Therapy, Berlin, Germany (H.-P.S.).
- Berlin-Brandenburger Centrum für Regenerative Therapien (DE)
- Mayo Clinic (US)
- Corresponding author
- Ammirati, Enrico
- Camici, Paolo G.
- ORCID
- Venue
- Circulation: Heart Failure
- Published
- 2020-11
- Volume
- 13
- Issue
- 11
- Pages
- e007405-e007405
- ISSN
- 1941-3289 (ISSN-L)
- 1941-3297
- PMC
- PMC7673642
- OpenAlex
- W3103940138
- Semantic Scholar
- beb20d26bcaef45ecb6c5c865df17930929b84c5
- Cited by
- 811
- References
- 123
- Citations by year
- 2026: 122 · 2025: 155 · 2024: 185 · 2023: 150 · 2022: 137 · 2021: 60 · 2020: 1
- FWCI
- 53.83
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation Heart Failure (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Funders
- Deutsches Zentrum für Herz-Kreislaufforschung
- Bundesministerium für Bildung und Forschung
- Regione del Veneto
- National Heart, Lung, and Blood Institute (R01HL141466, R01HL155990)
- Topics
- Health Sciences › Medicine › Infectious Diseases › Viral Infections and Immunology Research
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Pericarditis and Cardiac Tamponade
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Keywords
- acute myocarditis, dilated cardiomyopathy, myocarditis, cardiac magnetic resonance imaging, viral myocarditis, myocardial fibrosis, histopathology, prognosis, treatment guidelines, expert consensus
- MeSH terms
- Acute Disease, Cardiology, Chronic Disease, Humans, Myocarditis, Terminology as Topic, Predictive Value of Tests, Risk Factors, Treatment Outcome, Consensus
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