Pathophysiological Gaps, Diagnostic Challenges, and Uncertainties in Cardiac Sarcoidosis

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ABSTRACT

Cardiac sarcoidosis can mimic any cardiomyopathy in different stages. Noncaseating granulomatous inflammation can be missed, because of the nonhomogeneous distribution in the heart. The current diagnostic criteria show discrepancies and are partly nonspecific and insensitive. Besides the diagnostic pitfalls, there are controversies in the understanding of the causes, genetic and environmental background, and the natural evolution of the disease. Here, we review the current pathophysiological aspects and gaps that are relevant for future cardiac sarcoidosis diagnostics and research.

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Most cited references 66

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Myocarditis and inflammatory cardiomyopathy: current evidence and future directions

Carsten Tschöpe, Enrico Ammirati, Biykem Bozkurt … (2020)

Inflammatory cardiomyopathy, characterized by inflammatory cell infiltration into the myocardium and a high risk of deteriorating cardiac function, has a heterogeneous aetiology. Inflammatory cardiomyopathy is predominantly mediated by viral infection, but can also be induced by bacterial, protozoal or fungal infections as well as a wide variety of toxic substances and drugs and systemic immune-mediated diseases. Despite extensive research, inflammatory cardiomyopathy complicated by left ventricular dysfunction, heart failure or arrhythmia is associated with a poor prognosis. At present, the reason why some patients recover without residual myocardial injury whereas others develop dilated cardiomyopathy is unclear. The relative roles of the pathogen, host genomics and environmental factors in disease progression and healing are still under discussion, including which viruses are active inducers and which are only bystanders. As a consequence, treatment strategies are not well established. In this Review, we summarize and evaluate the available evidence on the pathogenesis, diagnosis and treatment of myocarditis and inflammatory cardiomyopathy, with a special focus on virus-induced and virus-associated myocarditis. Furthermore, we identify knowledge gaps, appraise the available experimental models and propose future directions for the field. The current knowledge and open questions regarding the cardiovascular effects associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are also discussed. This Review is the result of scientific cooperation of members of the Heart Failure Association of the ESC, the Heart Failure Society of America and the Japanese Heart Failure Society.

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HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis.

David Birnie, William H Sauer, Frank Bogun … (2014)

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Cardiac Sarcoidosis.

David Birnie, Pablo Nery, Andrew C T Ha … (2016)

Clinically manifest cardiac involvement occurs in perhaps 5% of patients with sarcoidosis. The 3 principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. An estimated 20% to 25% of patients with pulmonary/systemic sarcoidosis have asymptomatic cardiac involvement (clinically silent disease). In 2014, the first international guideline for the diagnosis and management of CS was published. In patients with clinically manifest CS, the extent of left ventricular dysfunction seems to be the most important predictor of prognosis. There is controversy in published reports as to the outcome of patients with clinically silent CS. Despite a paucity of data, immunosuppression therapy (primarily with corticosteroids) has been advocated for the treatment of clinically manifest CS. Device therapy, primarily with implantable cardioverter-defibrillators, is often recommended for patients with clinically manifest disease.

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Author and article information

Contributors

Laura Ueberham:

ORCID: https://orcid.org/0000-0002-6645-3155

laura.ueberham@medizin.uni-leipzig.de

Journal

Journal ID (nlm-ta): J Am Heart Assoc

Journal ID (iso-abbrev): J Am Heart Assoc

Journal ID (doi): 10.1002/(ISSN)2047-9980

Journal ID (publisher-id): JAH3

Journal ID (hwp): ahaoa

Title: Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

Publisher: John Wiley and Sons Inc. (Hoboken )

ISSN (Electronic): 2047-9980

Publication date (Electronic): 09 March 2023

Publication date Collection: 21 March 2023

Volume: 12

Issue: 6 ( doiID: 10.1002/jah3.v12.6 )

Electronic Location Identifier: e027971

Affiliations

[ ¹ ] Klinik und Poliklinik für Kardiologie Universitätsklinikum Leipzig Leipzig Germany

[ ² ] Cardiopathology Institute for Pathology, Eberhard Karls Universität Tübingen Tübingen Germany

[ ³ ] Department of Electrophysiology Heart Center Leipzig at University of Leipzig Leipzig Germany

[ ⁴ ] Klinik und Poliklinik für Nuklearmedizin Universitätsklinikum Leipzig Leipzig Germany

Author notes

[*] [* ]Correspondence to: Laura Ueberham, MD, Universitätsklinikum Leipzig, Klinik und Poliklinik für Kardiologie, Liebigstraße 20, Haus 4, 04103 Leipzig, Germany. Email: laura.ueberham@ 123456medizin.uni-leipzig.de

Author information

Laura Ueberham https://orcid.org/0000-0002-6645-3155

Andreas Hagendorff https://orcid.org/0000-0001-6288-7565

Karin Klingel https://orcid.org/0000-0003-0203-2498

Ingo Paetsch https://orcid.org/0000-0002-3528-7174

Cosima Jahnke https://orcid.org/0000-0001-9145-3467

Ulrich Laufs https://orcid.org/0000-0003-2620-9323

Article

Publisher ID: JAH38172 Other ID: JAHA/2022/027971

DOI: 10.1161/JAHA.122.027971

PMC ID: 10111513

PubMed ID: 36892055

SO-VID: 80af1844-b4d8-4504-b8f7-76f3966efda7

License:

This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

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Figures: 3, Tables: 2, Pages: 9, Words: 5456

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source-schema-version-number 2.0

cover-date 21 March 2023

details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:6.2.6 mode:remove_FC converted:21.03.2023

ScienceOpen disciplines: Cardiovascular Medicine

Keywords: cardiac inflammatory disease,cardiac sarcoidosis,myocarditis,sarcoidosis diagnostic criteria,inflammatory heart disease,arrhythmias,nuclear cardiology and pet,magnetic resonance imaging (mri)

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ScienceOpen disciplines: Cardiovascular Medicine

Keywords: cardiac inflammatory disease, cardiac sarcoidosis, myocarditis, sarcoidosis diagnostic criteria, inflammatory heart disease, arrhythmias, nuclear cardiology and pet, magnetic resonance imaging (mri)

Pathophysiological Gaps, Diagnostic Challenges, and Uncertainties in Cardiac Sarcoidosis

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ABSTRACT

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Cardiovascular Innovations and Applications

Most cited references 66

Myocarditis and inflammatory cardiomyopathy: current evidence and future directions

HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis.

Cardiac Sarcoidosis.

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