Cited by 848Viral Infections and Immunology ResearchPericarditis and Cardiac TamponadeCardiomyopathy and Myosin Studies

Management of Acute Myocarditis and Chronic Inflammatory Cardiomyopathy: An Expert Consensus Document

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Ammirati, Enrico, Frigerio, Maria, Adler, Eric D., Basso, Cristina, Birnie, David H., Brambatti, Michela, Friedrich, Matthias G., Klingel, Karin +8 more·2020·Circulation: Heart Failure

Abstract

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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Crossref · 10.1161/circheartfailure.120.007405

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  • Cardiotropic viruses such as adenoviruses and enteroviruses can directly damage the heart, leading to the development of myocarditis.

    “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]

    The ‘Virus-Induced and Immune-Mediated Lymphocytic AM and Infl-CMP’ section says enteroviral replication can directly injure cardiomyocytes and cause myocarditis. But ‘Definitions, Epidemiology, and Pathophysiology’ says it is not known which viruses other than enteroviruses directly damage human tissue, so the claim’s inclusion of adenoviruses as an established direct cause is not supported. Page numbers are unavailable in our copy. (LLM, conf=0.89)

    Experimental — accuracy not yet measured
  • Certain viruses including HIV, hepatitis C virus, influenza A virus, and influenza B virus are thought to trigger myocarditis by activating the immune system.

    “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]

    The section on virus-induced and immune-mediated myocarditis says influenza can trigger immune-mediated lymphocytic myocarditis and describes a suspected immune mechanism. It does not identify influenza A or B separately, or support the same mechanism for HIV or hepatitis C virus (searched those terms in our copy). (LLM, conf=0.87)

    Experimental — accuracy not yet measured
  • Up to 20% of giant cell myocarditis cases are associated with existing autoimmune disorders such as inflammatory bowel disease, thyroiditis, and thymoma.

    “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]

    The GCM > Etiology and Pathogenesis section reports associated autoimmune disorders in approximately 20% of giant cell myocarditis cases, particularly inflammatory bowel diseases and thyroid disorders. It does not specifically name thyroiditis or mention thymoma, so the full list of examples is not supported by our copy. No page number is available in the indexed passage. (LLM, conf=0.88)

    Experimental — accuracy not yet measured
  • Giant cell myocarditis and eosinophilic necrotizing myocarditis are traditionally classified as autoimmune myocarditis due to their strong association with existing autoimmune conditions.

    “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]

    The GCM Etiology and Pathogenesis section reports associated autoimmune disorders in approximately 20% of cases. The Eosinophilic Myocarditis Etiology and Pathogenesis section lists autoimmune-related systemic conditions among several possible associations, but also describes drug hypersensitivity and parasitic infection. The Definitions section classifies these forms by infiltrating cell type; our copy does not say that both are traditionally classified as autoimmune because of a strong association with pre-existing autoimmune conditions. Page numbers were not available in the indexed passages. (LLM, conf=0.86)

    Experimental — accuracy not yet measured
  • In giant cell myocarditis and cardiac sarcoidosis, macrophages are a significant contributor to the formation of multinucleated giant cells or granulomas.

    “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]

    The Sarcoidotic Myocarditis, Etiology and Pathogenesis section says activated macrophages can lead to granulomas and that macrophages within them form multinucleated giant cells. The GCM, Etiology and Pathogenesis section lists macrophages and giant cells among the cells involved, but does not say macrophages contribute to giant-cell formation in GCM. Page numbers are not available in our copy. (LLM, conf=0.87)

    Experimental — accuracy not yet measured

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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.
Venue
Circulation: Heart Failure
Published
2020-11
Volume
13
Issue
11
Pages
e007405-e007405
ISSN
  • 1941-3289 (ISSN-L)
  • 1941-3297
OpenAlex
W3103940138
Cited by
811
References
123
Citations by year
2026: 122 · 2025: 155 · 2024: 185 · 2023: 150 · 2022: 137 · 2021: 60 · 2020: 1 · 2014: 1
FWCI
53.83
Type
journal article
Language
en
Open access
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 › Cardiology and Cardiovascular Medicine › 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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