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Myocarditis associated with immune checkpoint inhibitors presents with an often-severe clinical phenotype with arrhythmias and concurrent myositis. This condition tends to occur early after treatment onset and is associated with a high fatality rate. Diagnosis may be challenging, and treatment algorithms are still evolving. This review will provide an overview of immune checkpoint inhibitor mechanism of action and how it relates to myocarditis pathophysiology, diagnostic algorithms and potential pitfalls, and emerging treatment approaches published until May 2023. We will focus on the state of the field and potential new directions in research and patient care. We will also provide consensus-based diagnostic and therapeutic algorithms endorsed by major societies. The field needs more evidence-based approaches to risk stratification so that therapy can be tailored toward less cardiotoxic alternatives in high-risk patients. For diagnostic and therapeutic approaches, data from animal models are unlikely to provide conclusive evidence given the complexity of the human immune system. We strongly invite practitioners in the field to contribute every case to the ongoing multicenter registries.

Immune checkpoint inhibitor-related myocarditis: current understanding and potential diagnostic and therapeutic strategies