Enrico Ammirati1, Giacomo Veronese2, Michela Brambatti3, Marco Merlo4, Manlio Cipriani5, Luciano Potena6, Paola Sormani5, Tatsuo Aoki7, Koichiro Sugimura7, Akinori Sawamura8, Takahiro Okumura8, Sean Pinney9, Kimberly Hong3, Palak Shah10, Öscar Braun11, Caroline M Van de Heyning12, Santiago Montero13, Duccio Petrella5, Florent Huang14, Matthieu Schmidt14, Claudia Raineri15, Anuradha Lala9, Marisa Varrenti2, Alberto Foà6, Ornella Leone6, Piero Gentile4, Jessica Artico4, Valentina Agostini6, Rajiv Patel10, Andrea Garascia5, Emeline M Van Craenenbroeck12, Kaoru Hirose16, Akihiro Isotani16, Toyoaki Murohara8, Yoh Arita17, Alessandro Sionis18, Enrico Fabris4, Sherin Hashem19, Victor Garcia-Hernando18, Fabrizio Oliva5, Barry Greenberg3, Hiroaki Shimokawa7, Gianfranco Sinagra4, Eric D Adler20, Maria Frigerio5, Paolo G Camici21. 1. De Gasperis Cardio Center, Niguarda Hospital, Milano, Italy. Electronic address: enrico.ammirati@ospedaleniguarda.it. 2. De Gasperis Cardio Center, Niguarda Hospital, Milano, Italy; Department of Health Sciences, University of Milano-Bicocca, Monza, Italy. 3. Division of Cardiology, Department of Medicine, University of California San Diego, La Jolla, California. 4. Cardiothoracic Department, Azienda Sanitaria Universitaria Integrata (ASUITS), University of Trieste, Trieste, Italy. 5. De Gasperis Cardio Center, Niguarda Hospital, Milano, Italy. 6. Academic Hospital S. Orsola-Malpighi, Bologna, Italy. 7. Tohoku University Graduate School of Medicine, Sendai, Japan. 8. Nagoya University Graduate School of Medicine, Nagoya, Japan. 9. Icahn School of Medicine at Mount Sinai, New York, New York. 10. Inova Heart and Vascular Institute, Falls Church, Virginia. 11. Department of Cardiology, Clinical Sciences, Lund University and Skåne University Hospital, Lund, Sweden. 12. Department of Cardiology, Antwerp University Hospital, Edegem, Belgium. 13. Department of Cardiology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute IIB Sant Pau, CIBER-CV, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain; Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Medical Intensive Care Unit, Paris Cedex 13, France. 14. Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Medical Intensive Care Unit, Paris Cedex 13, France. 15. Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy. 16. Kokura Memorial Hospital, Kitakyushu, Japan. 17. Japan Community Healthcare Organization, Osaka Hospital, Osaka, Japan. 18. Department of Cardiology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute IIB Sant Pau, CIBER-CV, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain. 19. Department of Pathology, University of California, San Diego, La Jolla, California. 20. Division of Cardiology, Department of Medicine, University of California San Diego, La Jolla, California. Electronic address: eradler@ucsd.edu. 21. Vita Salute University and San Raffaele Hospital, Milano, Italy.
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% 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.
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.
Authors: Carsten Tschöpe; Sophie Van Linthout; Sebastian Jäger; Robert Arndt; Tobias Trippel; Irene Müller; Ahmed Elsanhoury; Susanne Rutschow; Stefan D Anker; Heinz-Peter Schultheiss; Matthias Pauschinger; Frank Spillmann; Kathleen Pappritz Journal: ESC Heart Fail Date: 2020-07-14
Authors: Kaj Ekström; Anne Räisänen-Sokolowski; Jukka Lehtonen; Hanna-Kaisa Nordenswan; Mikko I Mäyränpää; Markku Kupari Journal: ESC Heart Fail Date: 2020-04-28
Authors: Ivana Jurcova; Jan Rocek; William Bracamonte-Baran; Michael Zelizko; Ivan Netuka; Jana Maluskova; Josef Kautzner; Daniela Cihakova; Vojtech Melenovsky; Jiri Maly Journal: ESC Heart Fail Date: 2020-06-02