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Pediatric Myocarditis

Learning Objectives

Core Knowledge & Clinical Reasoning

  • [ ] Apply diagnostic certainty strata (suspected, probable, definite) using clinical, laboratory, and imaging findings
  • [ ] Interpret cardiac MRI using Lake Louise criteria (edema, hyperemia, necrosis/fibrosis)
  • [ ] Differentiate fulminant from acute myocarditis and adjust management accordingly
  • [ ] Evaluate indications for endomyocardial biopsy in atypical or refractory cases

Acute Management

  • [ ] Formulate acute management plan including hemodynamic support and arrhythmia monitoring
  • [ ] Determine when to escalate to mechanical circulatory support (ECMO, VAD)

Communication & Counseling

  • [ ] Counsel families on prognosis, expected recovery timeline, and potential for chronic cardiomyopathy
  • [ ] Discuss return-to-play criteria and restrictions with patients and families (minimum 3-6 months)

Systems-Based Practice

  • [ ] Coordinate follow-up surveillance including serial echo, Holter, and exercise testing before activity clearance
  • [ ] Ensure appropriate school accommodations during recovery period

Key Guidelines

2021 AHA Scientific Statement: Diagnosis and Management of Myocarditis in Children Circulation. 2021;144:e123-e135

2025 ESC Guidelines on Inflammatory Myocardial and Pericardial Syndromes European Heart Journal. 2025

Diagnostic Certainty Strata

Level Definition
Biopsy-proven Histopathologic confirmation (Dallas criteria)
Clinically suspected, CMR-confirmed Lake Louise criteria positive
Clinically suspected Clinical presentation + supportive findings
Possible myocarditis Some features, diagnosis uncertain

Etiology

Viral (Most Common)

  • Enteroviruses (coxsackievirus)
  • Adenovirus
  • Parvovirus B19
  • Human herpesvirus 6
  • SARS-CoV-2
  • Influenza

Other Causes

  • Autoimmune (giant cell, eosinophilic)
  • Drug-induced (checkpoint inhibitors)
  • Toxic
  • Idiopathic

Clinical Presentation

Spectrum

  • Fulminant: Rapid hemodynamic deterioration
  • Acute: Typical presentation over days-weeks
  • Chronic active: Ongoing inflammation
  • Chronic persistent: Low-grade, may cause DCM

Common Symptoms

  • Chest pain
  • Heart failure symptoms
  • Palpitations/arrhythmias
  • Preceding viral illness

Diagnostic Evaluation

ECG

  • Abnormalities in ~90% of cases
  • ST-T changes
  • Low voltages
  • Arrhythmias (PVCs, VT)
  • Heart block

Biomarkers

  • Troponin: Elevated but NOT universally present
  • BNP/NT-proBNP: Correlates with LV dysfunction
  • CRP/ESR: Nonspecific inflammation

Echocardiography

  • LV dysfunction (global or regional)
  • Pericardial effusion
  • Chamber dilation
  • Valvular regurgitation

Cardiac MRI

Now cornerstone for noninvasive diagnosis

Lake Louise Criteria (Updated 2018): - T2-based criteria: Myocardial edema (T2 mapping, T2-weighted imaging) - T1-based criteria: Non-ischemic injury (LGE, T1 mapping, ECV)

Positive = ≥1 T2 criterion + ≥1 T1 criterion

Endomyocardial Biopsy

Indications: - New-onset HF with hemodynamic compromise - Suspected giant cell myocarditis - Failure to respond to treatment - Arrhythmias with suspected cardiac involvement

Management

Supportive Care (Primary Therapy)

  • Similar to acute heart failure management
  • Diuretics for congestion
  • Avoid NSAIDs (animal data suggests harm)

Inotropic Support

  • Milrinone: First-line for inotropy
  • Avoids catecholamine excess
  • Dobutamine as alternative

Mechanical Circulatory Support

  • Early consideration for high-risk patients
  • ECMO for fulminant myocarditis
  • VAD as bridge to recovery or transplant

Immunomodulation

  • IVIG: Limited evidence; center-specific protocols
  • Corticosteroids: Limited evidence; may be harmful if viral etiology
  • Reserve for suspected autoimmune or giant cell

Activity Restriction

  • Minimum 3-6 months of exercise restriction
  • Critical for preventing SCD during healing

Return to Activity Criteria

ALL must be met: 1. Normal LV function by echocardiography 2. No arrhythmias on Holter monitoring 3. No arrhythmias during exercise stress testing 4. Normal/improving biomarkers 5. CMR shows resolved inflammation (if repeated) 6. ≥3-6 months from acute illness

Prognosis

  • ~60-80% recover completely
  • ~10-20% develop chronic DCM
  • ~10% require transplant or die
  • Fulminant myocarditis: Paradoxically better long-term prognosis if survive acute phase

Board Pearls

Pearl: CMR is cornerstone for noninvasive diagnosis

Lake Louise criteria: T2 (edema) + T1 (injury) findings

Pearl: Milrinone is first-line inotrope

Avoids catecholamine toxicity in inflamed myocardium

Pearl: Return-to-play: 3-6 months minimum

Requires normal echo, Holter, AND exercise stress test

Self-Assessment

Q1: A 14-year-old presents with chest pain and dyspnea 1 week after a viral URI. ECG shows diffuse ST elevations. Troponin is mildly elevated. Echo shows EF of 48%. What is the next best diagnostic test?

Answer **Answer**: Cardiac MRI **Rationale**: This presentation is consistent with clinically suspected myocarditis. CMR with Lake Louise criteria is now the cornerstone for noninvasive diagnosis. It can demonstrate myocardial edema (T2) and injury (T1/LGE), confirming the diagnosis and assessing severity.

Q2: A 12-year-old had biopsy-proven myocarditis 2 months ago. EF normalized to 58%. He wants to return to soccer. Is he cleared?

Answer **Answer**: No - too soon; needs additional testing **Rationale**: Guidelines recommend minimum 3-6 months restriction AND must have: normal echo (met), normal Holter, AND normal exercise stress test before clearance. He has only reached 2 months and hasn't completed the required testing protocol.

References

  • Law YM, et al. Circulation. 2021;144:e123-e135
  • 2025 ESC Inflammatory Myocardial/Pericardial Syndromes Guidelines