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Myocarditis

Key sources
The following summarized guidelines for the evaluation and management of myocarditis are prepared by our editorial team based on guidelines from the Japanese Circulation Society (JCS 2023), the European Society of Cardiology (ESC 2023; 2022; 2013), the European Society of Cardiology (ESC/ESTRO/EHA/IC-OS 2022), the American Heart Association (AHA 2020; 2016), the European Society of Medical Oncology (ESMO 2020), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019), the American Heart Association (AHA/HRS/ACC 2018), the Canadian Cardiovascular Society (CCS 2017), the American Heart Association (AHA/ACC 2015), and the European Society of Cardiology (ESC/EACTS 2015).
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Guidelines

1.Screening and diagnosis

Diagnosis: as per JCS 2023 guidelines, suspect myocarditis in patients having common cold-like symptoms (respiratory symptoms, gastrointestinal symptoms) followed by signs and symptoms associated with HF or arrhythmias.
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2.Diagnostic investigations

History and physical examination: assess the following in patients with suspected myocarditis:
Situation
Guidance
History of present illness
Time of onset and course of symptoms
Past medical history
Skin rash (including insect bites)
History of medication, vaccination, and ingestion of harmful materials
Recent travel history
History of autoimmune disease
Physical examination
Fever and tachycardia (as signs of infection)
Bradycardia or tachycardia (indicating arrhythmia)
Low cardiac output (tachycardia, hypotension, cold extremities)
Left-sided HF (hypoxemia, third or fourth heart sound, moist rales)
Right-sided HF (jugular venous distention, hepatomegaly, peripheral edema)
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  • ECG

  • TTE

  • MRI

  • Nuclear imaging

  • Laboratory tests (inflammatory markers)

  • Laboratory tests (cardiac biomarkers)

  • Laboratory tests (metabolic panel)

  • Laboratory tests (serology)

3.Diagnostic procedures

Coronary angiography: consider obtaining selective coronary angiography in all patients with clinically suspected myocarditis.
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  • Endomyocardial biopsy

4.Medical management

Setting of care: as per JCS 2023 guidelines, consider hospitalizing patients with acute myocarditis and obtaining monitoring for at least 48 hours, even with stable hemodynamics or no HF symptoms.
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  • Management of fulminant myocarditis (inotropic support)

  • Management of fulminant myocarditis (circulatory support)

  • Management of HF

  • Management of cardiac arrhythmias (general principles)

  • Management of cardiac arrhythmias (antiarrhythmic drugs)

  • Management of cardiac arrhythmias (sedation)

  • Management of cardiac arrhythmias (cardiac pacing)

  • Management of cardiac arrhythmias (ICD implantation)

  • Management of cardiac arrhythmias (ECMO)

  • Management of cardiac arrhythmias (catheter ablation)

  • Immunosuppressive therapy

  • Antiviral therapy

5.Nonpharmacologic interventions

Physical activity restrictions: as per JCS 2023 guidelines, consider advising refraining from intense exercise for 6 months after onset in patients with improved HF symptoms, cardiac enzymes, abnormal ECG, and image findings.
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6.Surgical interventions

Heart transplantation
Defer cardiac transplantation in the acute phase of myocarditis as recovery may occur.
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Consider performing cardiac transplantation in the acute phase of myocarditis in hemodynamically unstable patients, including with giant cell myocarditis, when optimal pharmacological support and mechanical assistance failed to stabilize the patient.
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7.Specific circumstances

Neonatal patients, evaluation: obtain an ECG and echocardiography to diagnose myocarditis in neonatal patients.
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  • Neonatal patients (management)

  • Pediatric patients (evaluation)

  • Pediatric patients (immunosuppressive therapy)

  • Pediatric patients (advanced cardiac life support)

  • Pediatric patients (patients with vasculitides)

  • Patients with myopericarditis

  • Patients with giant cell myocarditis (immunosuppressive therapy)

  • Patients with giant cell myocarditis (management of cardiac arrhythmia)

  • Patients with giant cell myocarditis (heart transplantation)

  • Patients with eosinophilic myocarditis

  • Patients with immune checkpoint inhibitor-induced myocarditis (evaluation)

  • Patients with immune checkpoint inhibitor-induced myocarditis (management)

  • Patients with immune checkpoint inhibitor-induced myocarditis (restarting ICI therapy)

8.Follow-up and surveillance

Serial clinical and imaging assessment: as per ESC 2023 guidelines, consider obtaining contrast-enhanced cardiac MRI in the serial follow-up and for the assessment of therapeutic response in patients with inflammatory cardiomyopathy.
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  • Serial endomyocardial biopsy

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