Clinical Strategy for the Diagnosis and Treatment of Immune Checkpoint Inhibitor-Associated Myocarditis: A Narrative Review

Lorenz H. Lehmann, Jennifer Cautela, Nicolas Palaskas, Alan H. Baik, Wouter C. Meijers, Yves Allenbach, Joachim Alexandre, Tienush Rassaf, Oliver J. Müller, Mandar Aras, Aarti H. Asnani, Anita Deswal, Michal Laufer-Perl, Franck Thuny, Mathieu Kerneis, Salim S. Hayek, Stéphane Ederhy, Joe Elie Salem*, Javid J. Moslehi*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

61 Scopus citations


Importance: In the last decade, immune checkpoint inhibitors (ICIs) have been approved for the treatment of many cancer types. Immune checkpoint inhibitor-associated myocarditis has emerged as a significant and potentially fatal adverse effect. Recognizing, diagnosing, and treating ICI-associated myocarditis poses new challenges for the practicing clinician. Here, the current literature on ICI-associated myocarditis is reviewed. Observations: Clinical presentation and cardiac pathological findings are highly variable in patients with ICI-associated myocarditis. Although endomyocardial biopsy is the criterion standard diagnostic test, a combination of clinical suspicion, cardiac biomarkers (specifically troponin), and cardiac imaging, in addition to biopsy, is often needed to support the diagnosis. Importantly, the combination of a cytotoxic T-lymphocyte-associated protein 4 inhibitor with a programmed cell death protein 1 or programmed death-ligand 1 inhibitor increases the risk of developing ICI-associated myocarditis. Conclusion and Relevance: This review aims to provide a standardized diagnostic and therapeutic approach for patients with suspected ICI-associated myocarditis. A complete history of recent cancer treatments and physical examination in combination with cardiac biomarkers, cardiac imaging, and endomyocardial biopsy represent a pragmatic diagnostic approach for most cases of ICI-associated myocarditis. The addition of novel biomarkers or imaging modalities is an area of active research and should be evaluated in larger cohorts..

Original languageEnglish
Pages (from-to)1329-1337
Number of pages9
JournalJAMA Cardiology
Issue number11
StatePublished - Nov 2021
Externally publishedYes


FundersFunder number
Bayer Israel
Bundesministerium für Forschung
Novartis Israel
Pfizer Israel
National Institutes of HealthLE 3570/2-1, 3570/3-1
National Heart, Lung, and Blood Institute
AstraZenecaR01 HL141466, R01 HL155990, R01 HL156021
Boehringer Ingelheim
Takeda Pharmaceutical Company
Andrew Sabin Family Foundation
Deutsche Forschungsgemeinschaft01KC2006B
Fédération Française de Cardiologie
Novo Nordisk
Institut Servier1R01HL153384-01


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