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Cited by 62Viral Infections and Immunology ResearchSarcoidosis and Beryllium Toxicity ResearchCardiac tumors and thrombi

Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25‐Year Nationwide Cohorts

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Nordenswan, Hanna‐Kaisa, Lehtonen, Jukka, Ekström, Kaj, Räisänen‐Sokolowski, Anne, Mäyränpää, Mikko I., Vihinen, Tapani, Miettinen, Heikki, Kaikkonen, Kari +8 more·2021·Journal of the American Heart Association
DOI 10.1161/jaha.120.019415PMC PMC8174201
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Abstract

Background: Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) share many histopathologic and clinical features. Whether they are parts of a one‐disease continuum has been discussed. Methods and Results: We compared medical record data of 351 CS and 28 GCM cases diagnosed in Finland since the late 1980s and followed until February 2018 for a composite end point of cardiac death, aborted sudden death, and heart transplantation. Heart failure was the presenting manifestation in 50% versus 15% ( P <0.001), and high‐grade atrioventricular block in 21% versus 43% ( P =0.044), of GCM and CS, respectively. At presentation, left ventricular ejection fraction was ≤50% in 81% of cases of GCM versus in 48% of CS ( P =0.004). The median (interquartile range) of plasma NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) was 5273 (2782–11309) ng/L on admission in GCM versus 859 (290–1950) ng/L in CS ( P <0.001), and cardiac troponin T exceeded 50 ng/L in 17 of 19 cases of GCM versus in 48 of 239 cases of CS ( P <0.001). The 5‐year estimate of event‐free survival was 77% (95% CI, 72%–82%) in CS versus 27% (95% CI, 10%–45%) in GCM ( P <0.001). By Cox regression analysis, GCM predicted cardiac events with a hazard ratio of 5.16 (95% CI, 2.82–9.45), which, however, decreased to 1.58 (95% CI, 0.71–3.52) after inclusion of markers of myocardial injury and dysfunction in the model. Conclusions: GCM differs from CS in presenting with more extensive myocardial injury and having worse long‐term outcome. Yet the key determinant of prognosis appears to be the extent of myocardial injury rather than the histopathologic diagnosis.

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  • In fulminant myocarditis, severe conduction abnormalities such as second- and third-degree atrioventricular blocks are common.

    “In fulminant myocarditis, severe conduction abnormalities such as second- and third-degree atrioventricular blocks are common”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [255]

    The Results (Characteristics of the Study Groups, Table 1; page unavailable) report third-degree or Mobitz II second-degree atrioventricular block as the main presenting manifestation in 21% of giant cell myocarditis cases and 43% of cardiac sarcoidosis cases. This supports the occurrence of severe conduction abnormalities, but the study does not establish that they are common in fulminant myocarditis generally. (LLM, conf=0.88)

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  • The incidence of sudden cardiac death from acute myocarditis in young adults is about 6% to 10%.

    “given an incidence of about 6% to 10% of sudden cardiac death from acute myocarditis in young adults.”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [255]

    The Results (Long-Term Outcome and Its Predictors; page unavailable) report cardiac deaths among patients with cardiac sarcoidosis or giant cell myocarditis, not the incidence of sudden cardiac death from acute myocarditis in young adults. Searches for the 6%–10% figure and for young adults with acute myocarditis found no passage addressing that estimate. (LLM, conf=0.73)

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Authors
Nordenswan, H., Lehtonen, J., Ekström, K., Räisänen‐Sokolowski, A., Mäyränpää, M. I., Vihinen, T., Miettinen, H., Kaikkonen, K., Haataja, P., Kerola, T., Rissanen, T. T., Kokkonen, J., Alatalo, A., Pietilä‐Effati, P., Utriainen, S., & Kupari, M.
Affiliations
  • University of Helsinki (FI)
  • Department of Pathology University of Helsinki and Helsinki University Central Hospital Helsinki Finland
  • Turku University Hospital (FI)
  • Kuopio University Hospital (FI)
  • Oulu University Hospital (FI)
  • Tampere University Hospital (FI)
  • Päijät-Hämeen Keskussairaala (FI)
  • North Karelia Central Hospital (FI)
  • Central Finland Health Care District (FI)
  • Seinäjoen Keskussairaala (FI)
  • Vaasa Central Hospital Vaasa Finland
  • South Karelia Central Hospital (FI)
Corresponding author
Nordenswan, Hanna‐Kaisa
ORCID
Venue
Journal of the American Heart Association
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2021-03-16
Volume
10
Issue
6
Pages
e019415-e019415
ISSN
2047-9980 (ISSN-L)
DOI
10.1161/jaha.120.019415
PMC
PMC8174201
OpenAlex
W3134828210
Cited by
62
References
0
Citations by year
2026: 10 · 2025: 6 · 2024: 12 · 2023: 21 · 2022: 11 · 2021: 2
FWCI
7.71
Type
journal article
Language
en
Open access
Licence
cc-by-nc-nd
Locations
  • Journal of the American Heart Association (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • UTUPub (University of Turku) (submittedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
  • Työväentutkimus Vuosikirja (submittedVersion)
  • Tampere University Institutional Repository (Tampere University) (submittedVersion, cc-by-nc-nd)
  • UTUPub (University of Turku) (submittedVersion, cc-by-nc-nd)
Funders
Helsingin Yliopisto
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Viral Infections and Immunology Research
  • Health Sciences › Medicine › Physiology › Sarcoidosis and Beryllium Toxicity Research
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac tumors and thrombi
Keywords
cardiac sarcoidosis, giant cell myocarditis, prognosis, heart failure, atrioventricular block, NT-proBNP, cardiac troponin T, myocardial injury, event-free survival, cardiovascular mortality, heart transplantation, sudden cardiac death, long-term outcomes, disease continuum, left ventricular ejection fraction, national registry, risk factors, histopathology, immunosuppressive therapy
MeSH terms
Adult, Aged, Cause of Death, Female, Follow-Up Studies, Forecasting, Humans, Male, Middle Aged, Cardiomyopathies, Myocardium, Population Surveillance, Retrospective Studies, Sarcoidosis, United States, Biomarkers, Giant Cells, Incidence, Survival Rate, Natriuretic Peptide, Brain

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