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Fulminant Versus Acute Nonfulminant Myocarditis in Patients With Left Ventricular Systolic Dysfunction

Ammirati, Enrico ; Veronese, Giacomo ; Brambatti, Michela ; Merlo, M. ; Cipriani, Manlio ; Potena, Luciano ; Sormani, P. ; Aoki, Tatsuo ; Sugimura, Koichiro and Sawamura, Akinori , et al. (2019) In Journal of the American College of Cardiology 74(3). p.299-311
Abstract

Background: Fulminant myocarditis (FM) is a form of acute myocarditis characterized by severe left ventricular systolic dysfunction requiring inotropes and/or mechanical circulatory support. A single-center study found that a patient with FM had better outcomes than those with acute nonfulminant myocarditis (NFM) presenting with left ventricular systolic dysfunction, but otherwise hemodynamically stable. This was recently challenged, so disagreement still exists. Objectives: This study sought to provide additional evidence on the outcome of FM and to ascertain whether patient stratification based on the main histologic subtypes can provide additional prognostic information. Methods: A total of 220 patients (median age 42 years, 46.3%... (More)

Background: Fulminant myocarditis (FM) is a form of acute myocarditis characterized by severe left ventricular systolic dysfunction requiring inotropes and/or mechanical circulatory support. A single-center study found that a patient with FM had better outcomes than those with acute nonfulminant myocarditis (NFM) presenting with left ventricular systolic dysfunction, but otherwise hemodynamically stable. This was recently challenged, so disagreement still exists. Objectives: This study sought to provide additional evidence on the outcome of FM and to ascertain whether patient stratification based on the main histologic subtypes can provide additional prognostic information. Methods: A total of 220 patients (median age 42 years, 46.3% female) with histologically proven acute myocarditis (onset of symptoms <30 days) all presenting with left ventricular systolic dysfunction were included in a retrospective, international registry comprising 16 tertiary hospitals in the United States, Europe, and Japan. The main endpoint was the occurrence of cardiac death or heart transplantation within 60 days from admission and at long-term follow-up. Results: Patients with FM (n = 165) had significantly higher rates of cardiac death and heart transplantation compared with those with NFM (n = 55), both at 60 days (28.0% vs. 1.8%, p = 0.0001) and at 7-year follow-up (47.7% vs. 10.4%, p < 0.0001). Using Cox multivariate analysis, the histologic subtype emerged as a further variable affecting the outcome in FM patients, with giant cell myocarditis having a significantly worse prognosis compared with eosinophilic and lymphocytic myocarditis. In a subanalysis including only adults with lymphocytic myocarditis, the main endpoints occurred more frequently in FM compared with in NFM both at 60 days (19.5% vs. 0%, p = 0.005) and at 7-year follow up (41.4% vs. 3.1%, p = 0.0004). Conclusions: This international registry confirms that patients with FM have higher rates of cardiac death and heart transplantation both in the short- and long-term compared with patients with NFM. Furthermore, we provide evidence that the histologic subtype of FM carries independent prognostic value, highlighting the need for timely endomyocardial biopsy in this condition.

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published
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keywords
acute myocarditis, endomyocardial biopsy, eosinophilic myocarditis, fulminant myocarditis, giant cell myocarditis, outcome
in
Journal of the American College of Cardiology
volume
74
issue
3
pages
13 pages
publisher
Elsevier USA
external identifiers
  • pmid:31319912
  • scopus:85068254694
ISSN
0735-1097
DOI
10.1016/j.jacc.2019.04.063
language
English
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yes
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4bfdc397-319c-4840-b2a0-60934c994d7f
date added to LUP
2019-07-09 13:34:22
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2020-07-08 04:59:41
@article{4bfdc397-319c-4840-b2a0-60934c994d7f,
  abstract     = {<p>Background: Fulminant myocarditis (FM) is a form of acute myocarditis characterized by severe left ventricular systolic dysfunction requiring inotropes and/or mechanical circulatory support. A single-center study found that a patient with FM had better outcomes than those with acute nonfulminant myocarditis (NFM) presenting with left ventricular systolic dysfunction, but otherwise hemodynamically stable. This was recently challenged, so disagreement still exists. Objectives: This study sought to provide additional evidence on the outcome of FM and to ascertain whether patient stratification based on the main histologic subtypes can provide additional prognostic information. Methods: A total of 220 patients (median age 42 years, 46.3% female) with histologically proven acute myocarditis (onset of symptoms &lt;30 days) all presenting with left ventricular systolic dysfunction were included in a retrospective, international registry comprising 16 tertiary hospitals in the United States, Europe, and Japan. The main endpoint was the occurrence of cardiac death or heart transplantation within 60 days from admission and at long-term follow-up. Results: Patients with FM (n = 165) had significantly higher rates of cardiac death and heart transplantation compared with those with NFM (n = 55), both at 60 days (28.0% vs. 1.8%, p = 0.0001) and at 7-year follow-up (47.7% vs. 10.4%, p &lt; 0.0001). Using Cox multivariate analysis, the histologic subtype emerged as a further variable affecting the outcome in FM patients, with giant cell myocarditis having a significantly worse prognosis compared with eosinophilic and lymphocytic myocarditis. In a subanalysis including only adults with lymphocytic myocarditis, the main endpoints occurred more frequently in FM compared with in NFM both at 60 days (19.5% vs. 0%, p = 0.005) and at 7-year follow up (41.4% vs. 3.1%, p = 0.0004). Conclusions: This international registry confirms that patients with FM have higher rates of cardiac death and heart transplantation both in the short- and long-term compared with patients with NFM. Furthermore, we provide evidence that the histologic subtype of FM carries independent prognostic value, highlighting the need for timely endomyocardial biopsy in this condition.</p>},
  author       = {Ammirati, Enrico and Veronese, Giacomo and Brambatti, Michela and Merlo, M. and Cipriani, Manlio and Potena, Luciano and Sormani, P. and Aoki, Tatsuo and Sugimura, Koichiro and Sawamura, Akinori and Okumura, Takahiro and Pinney, Sean and Hong, Kimberly and Shah, P. and Braun, Öscar and Van de Heyning, Caroline M. and Montero, Santiago and Petrella, Duccio and Huang, Florent and Schmidt, Matthieu and Raineri, Claudia and Lala, Anuradha and Varrenti, Marisa and Foà, Alberto and Leone, Ornella and Gentile, P. and Artico, Jessica and Agostini, Valentina and Patel, R. and Garascia, Andrea and Van Craenenbroeck, Emeline M. and Hirose, K. and Isotani, Akihiro and Murohara, Toyoaki and Arita, Yoh and Sionis, Alessandro and Fabris, Enrico and Hashem, Sherin and Garcia-Hernando, Victor and Oliva, Fabrizio and Greenberg, Barry H. and Shimokawa, Hiroaki and Sinagra, Gianfranco and Adler, Eric D. and Frigerio, M. and Camici, Paolo G.},
  issn         = {0735-1097},
  language     = {eng},
  month        = {07},
  number       = {3},
  pages        = {299--311},
  publisher    = {Elsevier USA},
  series       = {Journal of the American College of Cardiology},
  title        = {Fulminant Versus Acute Nonfulminant Myocarditis in Patients With Left Ventricular Systolic Dysfunction},
  url          = {http://dx.doi.org/10.1016/j.jacc.2019.04.063},
  doi          = {10.1016/j.jacc.2019.04.063},
  volume       = {74},
  year         = {2019},
}