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

Enrico Ammirati, Giacomo Veronese, Michela Brambatti, M. Merlo, Manlio Cipriani, Luciano Potena, P. Sormani, Tatsuo Aoki, Koichiro Sugimura, Akinori Sawamura, Takahiro Okumura, Sean Pinney, Kimberly Hong, P. Shah, Öscar Braun, Caroline M. Van de Heyning, Santiago Montero, Duccio Petrella, Florent Huang, Matthieu SchmidtClaudia Raineri, Anuradha Lala, Marisa Varrenti, Alberto Foà, Ornella Leone, P. Gentile, Jessica Artico, Valentina Agostini, R. Patel, Andrea Garascia, Emeline M. Van Craenenbroeck, K. Hirose, Akihiro Isotani, Toyoaki Murohara, Yoh Arita, Alessandro Sionis, Enrico Fabris, Sherin Hashem, Victor Garcia-Hernando, Fabrizio Oliva, B. Greenberg, Hiroaki Shimokawa, Gianfranco Sinagra, Eric D. Adler, M. Frigerio, Paolo G. Camici

Research output: Contribution to journalArticlepeer-review

145 Scopus citations


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.

Original languageEnglish
Pages (from-to)299-311
Number of pages13
JournalJournal of the American College of Cardiology
Issue number3
StatePublished - 23 Jul 2019


  • acute myocarditis
  • endomyocardial biopsy
  • eosinophilic myocarditis
  • fulminant myocarditis
  • giant cell myocarditis
  • outcome


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