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      Cardiac magnetic resonance in giant cell myocarditis: a matched comparison with cardiac sarcoidosis

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          Abstract

          Aims

          Giant cell myocarditis (GCM) is an inflammatory cardiomyopathy akin to cardiac sarcoidosis (CS). We decided to study the findings of GCM on cardiac magnetic resonance (CMR) imaging and to compare GCM with CS.

          Methods and results

          CMR studies of 18 GCM patients were analyzed and compared with 18 CS controls matched for age, sex, left ventricular (LV) ejection fraction and presenting cardiac manifestations. The analysts were blinded to clinical data. On admission, the duration of symptoms (median) was 0.2 months in GCM vs. 2.4 months in CS (P = 0.002), cardiac troponin T was elevated (>50 ng/L) in 16/17 patients with GCM and in 2/16 with CS (P < 0.001), their respective median plasma B-type natriuretic propeptides measuring 4488 ng/L and 1223 ng/L (P = 0.011). On CMR imaging, LV diastolic volume was smaller in GCM (177 ± 32 mL vs. 211 ± 58 mL, P = 0.014) without other volumetric or wall thickness measurements differing between the groups. Every GCM patient had multifocal late gadolinium enhancement (LGE) in a distribution indistinguishable from CS both longitudinally, circumferentially, and radially across the LV segments. LGE mass averaged 17.4 ± 6.3% of LV mass in GCM vs 25.0 ± 13.4% in CS (P = 0.037). Involvement of insertion points extending across the septum into the right ventricular wall, the “hook sign” of CS, was present in 53% of GCM and 50% of CS.

          Conclusion

          In GCM, CMR findings are qualitatively indistinguishable from CS despite myocardial inflammation being clinically more acute and injurious. When matched for LV dysfunction and presenting features, LV size and LGE mass are smaller in GCM.

          Graphical Abstract

          Graphical Abstract

          Summary of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance in giant cell myocarditis (GCM) and cardiac sarcoidosis (CS). GCM and CS have indistinguishable LGE distribution but, for matched LV dysfunction, the quantity of LGE is lower in GCM.

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          Most cited references39

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          Cardiovascular magnetic resonance in myocarditis: A JACC White Paper.

          Cardiovascular magnetic resonance (CMR) has become the primary tool for noninvasive assessment of myocardial inflammation in patients with suspected myocarditis. The International Consensus Group on CMR Diagnosis of Myocarditis was founded in 2006 to achieve consensus among CMR experts and develop recommendations on the current state-of-the-art use of CMR for myocarditis. The recommendations include indications for CMR in patients with suspected myocarditis, CMR protocol standards, terminology for reporting CMR findings, and diagnostic CMR criteria for myocarditis (i.e., "Lake Louise Criteria").
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            Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart. A statement for healthcare professionals from the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association.

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              Myocarditis and inflammatory cardiomyopathy: current evidence and future directions

              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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                Author and article information

                Contributors
                Journal
                Eur Heart J Cardiovasc Imaging
                Eur Heart J Cardiovasc Imaging
                ehjcimaging
                European Heart Journal Cardiovascular Imaging
                Oxford University Press (US )
                2047-2404
                2047-2412
                April 2023
                10 January 2023
                10 January 2023
                : 24
                : 4
                : 404-412
                Affiliations
                Heart and Lung Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                Radiology, HUS Diagnostic Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                Heart and Lung Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                Heart and Lung Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                Radiology, HUS Diagnostic Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                University of Minnesota Medical School, Cardiovascular Division, Department of Medicine , 420 Delaware St SE, MMC 508, Minneapolis, Minnesota 55455, USA
                Heart and Lung Center, Helsinki University Hospital and University of Helsinki , Haartmaninkatu 4, 00029 Helsinki, Finland
                Author notes
                Corresponding author E-mail: pauli.poyhonen@ 123456hus.fi

                Conflict of interest: P.P. was supported by a Finnish government grant for medical research. J.L. was supported by Finnish Cardiac Society and a Finnish government grant for medical research. C.S. was supported by National Institutes of Health (NIH) grants K23HL132011 and R03HL15711. S.S. has received honoraria for lectures from Finnish Society of Chest Radiology, The Radiological Society of Finland and Society of Radiographers in Finland.

                Author information
                https://orcid.org/0000-0002-3031-5391
                https://orcid.org/0000-0003-0114-114X
                https://orcid.org/0000-0003-1889-3858
                https://orcid.org/0000-0001-8877-4999
                https://orcid.org/0000-0002-5813-3477
                Article
                jeac265
                10.1093/ehjci/jeac265
                10029848
                36624560
                80564ccd-61b5-4c7e-85e9-27b68dbefd99
                © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License ( https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 14 August 2022
                : 16 December 2022
                Page count
                Pages: 9
                Categories
                Original Paper
                AcademicSubjects/MED00200
                Eurheartj/31
                Eurheartj/33
                Eurheartj/48
                Eurheartj/51
                Editor's Choice

                Cardiovascular Medicine
                giant cell myocarditis,cardiac sarcoidosis,cardiac magnetic resonance

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