Author + information
- Received March 11, 2019
- Revision received April 23, 2019
- Accepted April 29, 2019
- Published online July 15, 2019.
- Enrico Ammirati, MD, PhDa,∗ (, )@ospniguarda,
- Giacomo Veronese, MDa,b,
- Michela Brambatti, MD, MSc,
- Marco Merlo, MDd,
- Manlio Cipriani, MDa,
- Luciano Potena, MDe,
- Paola Sormani, MDa,
- Tatsuo Aoki, MD, PhDf,
- Koichiro Sugimura, MDf,
- Akinori Sawamura, MD, PhDg,
- Takahiro Okumura, MD, PhDg,
- Sean Pinney, MDh,
- Kimberly Hong, MDc,
- Palak Shah, MD, MSi,
- Öscar Braun, MD, PhDj,
- Caroline M. Van de Heyning, MD, PhDk,
- Santiago Montero, MDl,m,
- Duccio Petrella, MDa,
- Florent Huang, MDm,
- Matthieu Schmidt, MDm,
- Claudia Raineri, MDn,
- Anuradha Lala, MDh,
- Marisa Varrenti, MDa,b,
- Alberto Foà, MDe,
- Ornella Leone, MDe,
- Piero Gentile, MDd,
- Jessica Artico, MDd,
- Valentina Agostini, PhDe,
- Rajiv Patel, MDi,
- Andrea Garascia, MDa,
- Emeline M. Van Craenenbroeck, MD, PhDk,
- Kaoru Hirose, MDo,
- Akihiro Isotani, MDo,
- Toyoaki Murohara, MD, PhDg,
- Yoh Arita, MD, PhDp,
- Alessandro Sionis, MDl,
- Enrico Fabris, MDd,
- Sherin Hashem, MD, PhDq,
- Victor Garcia-Hernando, MDl,
- Fabrizio Oliva, MDa,
- Barry Greenberg, MDc,
- Hiroaki Shimokawa, MDf,
- Gianfranco Sinagra, MDd,
- Eric D. Adler, MDc,∗∗ (, )@ericadler17,
- Maria Frigerio, MDa,∗ and
- Paolo G. Camici, MDr,∗
- aDe Gasperis Cardio Center, Niguarda Hospital, Milano, Italy
- bDepartment of Health Sciences, University of Milano-Bicocca, Monza, Italy
- cDivision of Cardiology, Department of Medicine, University of California San Diego, La Jolla, California
- dCardiothoracic Department, Azienda Sanitaria Universitaria Integrata (ASUITS), University of Trieste, Trieste, Italy
- eAcademic Hospital S. Orsola-Malpighi, Bologna, Italy
- fTohoku University Graduate School of Medicine, Sendai, Japan
- gNagoya University Graduate School of Medicine, Nagoya, Japan
- hIcahn School of Medicine at Mount Sinai, New York, New York
- iInova Heart and Vascular Institute, Falls Church, Virginia
- jDepartment of Cardiology, Clinical Sciences, Lund University and Skåne University Hospital, Lund, Sweden
- kDepartment of Cardiology, Antwerp University Hospital, Edegem, Belgium
- lDepartment 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
- mSorbonne Université, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Medical Intensive Care Unit, Paris Cedex 13, France
- nIstituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
- oKokura Memorial Hospital, Kitakyushu, Japan
- pJapan Community Healthcare Organization, Osaka Hospital, Osaka, Japan
- qDepartment of Pathology, University of California, San Diego, La Jolla, California
- rVita Salute University and San Raffaele Hospital, Milano, Italy
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.
- acute myocarditis
- endomyocardial biopsy
- eosinophilic myocarditis
- fulminant myocarditis
- giant cell myocarditis
↵∗ Drs. Frigerio and Camici share senior authorship.
Dr. Pinney is a consultant for Abbott, CareDx, and Medtronic. Dr. Shah has received grant support from Abbott, Medtronic, and Merck; and is a consultant for NuPulse CV and Ortho Clinical Diagnostics. Dr. Schmidt has received lecture fees from Getinge, Drager, and Xenios. Dr. Adler is a consultant for Abbott and Medtronic. Dr. Camici is a consultant for Servier. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Listen to this manuscript's audio summary by Editor-in-Chief Dr. Valentin Fuster on JACC.org.
- Received March 11, 2019.
- Revision received April 23, 2019.
- Accepted April 29, 2019.
- 2019 American College of Cardiology Foundation
This article requires a subscription or purchase to view the full text. If you are a subscriber or member, click Login or the Subscribe link (top menu above) to access this article.