Pediatric Myocarditis & Pericarditis: Symptoms, Treatment, Emergency Department Management & Care | EB Medicine
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Myocarditis And Pericarditis In The Pediatric Patient: Validated Management Strategies

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Table of Contents
 
Table of Contents
  1. Abstract
  2. Case Presentations
  3. Introduction
  4. Critical Appraisal of the Literature
  5. Etiology And Pathophysiology
    1. Myocarditis
  6. Differential Diagnosis
  7. Prehospital Care
  8. Emergency Department Evaluation
    1. Primary Survey
      1. Perform Pericardiocentesis For Cardiac Tamponade Or Large Pericardial Effusion
    2. History
    3. Physical Examination
  9. Diagnostic Studies
    1. Myocarditis
      1. Electrocardiography
      2. Chest Radiography
      3. Troponin
      4. Inflammatory Markers
      5. Echocardiography
      6. Additional Studies
        • Microbiologic Studies
        • Brain Natriuretic Peptide
        • Other Laboratory Investigations
        • Cardiac Magnetic Resonance Imaging
        • Endomyocardial Biopsy
    2. Pericarditis
      1. Electrocardiography
      2. Chest Radiography
      3. Troponin
      4. Inflammatory Markers
      5. Echocardiography
      6. Pericardiocentesis
      7. Additional Studies
        • Microbiologic Studies
        • Brain Natriuretic Peptide
        • Other Laboratory Studies
        • Cardiac Computed Tomography/Magnetic Resonance Imaging
  10. Treatment
    1. Myocarditis
      1. Supportive Therapies – Diuretics, Cardiac Support, And Antidysrhythmic Medications
      2. Immunosuppressive Agents
      3. Intravenous Immunoglobulin
      4. Antivirals And Antibiotics
      5. Myocarditis Treatment Summary
    2. Pericarditis
      1. Supportive Therapies And Nonsteroidal Anti- Inflammatory Drugs
      2. Colchicine
      3. Corticosteroids
      4. Antivirals And Antibiotics
      5. Surgical Interventions
      6. Pericarditis Treatment Summary
  11. Special Populations
    1. Patients With Congenital Heart Disease Or Recent Cardiac Surgery
  12. Controversies And Cutting Edge
    1. Myocarditis
  13. Disposition
  14. Summary
  15. Risk Management Pitfalls In The Management Of Myocarditis And Pericarditis In Pediatric Patients
  16. Time- And Cost-Effective Strategies
  17. Case Conclusions
  18. Clinical Pathway For Emergency Management Of Myocarditis And Pericarditis In The Pediatric Patient
  19. Tables and Figures
    1. Table 1. Etiologies Of Myocarditis
    2. Table 2. Etiologies Of Pericaditis
    3. Table 3. Common Etiologies Of Pericardial Effusion In Children
    4. Table 4. Indications For Pericardiocentesis And Possible Complications
    5. Figure 1. Concave ST-Segment Increases In Lateral Leads (V4-V6) In Myocarditis
    6. Figure 2. Electrocardiogram Demonstrating Concave-Up ST-Segment Elevation And PR Depression With Diffuse T-Wave Inversion In Acute Pericarditis
  20. References

Abstract

Myocarditis and pericarditis are inflammatory conditions of the heart commonly caused by viral and autoimmune etiologies, although many cases are idiopathic. Emergency clinicians must maintain a high index of suspicion for these conditions, given the rarity and often nonspecific presentation in the pediatric population. Children with myocarditis may present with a variety of symptoms, ranging from mild flu-like symptoms to overt heart failure and shock, whereas children with pericarditis typically present with chest pain and fever. The cornerstone of therapy for myocarditis includes aggressive supportive management of heart failure, as well as administration of inotropes and antidysrhythmic medications, as indicated. Children often require admission to an intensive care setting. The acute management of pericarditis includes recognition of tamponade and, if identified, the performance of pericardiocentesis. Medical therapies may include nonsteroidal anti-inflammatory drugs and colchicine, with steroids reserved for specific populations. This review focuses on the evaluation and treatment of children with myocarditis and/or pericarditis, with an emphasis on currently available medical evidence.

Case Presentations

A previously healthy 4-year-old boy with symptoms of chest pain, difficulty breathing, and fever is brought to the ED. His parents note that the symptoms started 1 week prior, and they are flu-like, with general malaise, muscle weakness, and episodes of vomiting. His fever started 3 days prior to evaluation, and he has developed a cough with progressive difficulty breathing over that time, as well. The child points to the left mid-chest when asked about his pain. In triage, he is noted to have a heart rate of 180 beats/min and normal blood pressure for age. He is febrile to 38.8°C, has a respiratory rate of 38 breaths/min, and an oxygen saturation of 91% breathing room air. On examination, you note a pale, ill-appearing child. You auscultate crackles in the bilateral lung bases and a gallop rhythm on cardiac examination, although heart sounds are somewhat diminished. Capillary refill is sluggish. His liver edge is palpable 3 cm below the costal margin. What are the first steps in the immediate management of this patient? What diagnostic workup should be performed? Are there any indications for immediate echocardiography and/or immediate cardiology consultation? What is the appropriate disposition for this patient?

A previously healthy 12-year-old girl presents to your ED with chest pain and fever. Her chest pain has progressively worsened over the last 5 days, and it is described as stabbing. The pain is located over the middle of her chest, without radiation, and it is improved by sitting upright and leaning forward. Fever has been present for the past 2 days and has not resolved with antipyretics. In triage, the patient had an episode of vomiting. Her vital signs are: axillary temperature, 39°C; heart rate, 120 beats/min; normal blood pressure for age; respiratory rate, 30 breaths/min; and oxygen saturation, 96% on room air. On examination, the child appears to be in significant pain. Her pulmonary examination is unremarkable. On cardiac auscultation, you appreciate a friction rub with audible heart sounds. There is no murmur or gallop rhythm, and capillary refill time is normal. She has mild tenderness in the epigastrium. What historical features and examination findings raise concern? What are the initial steps in management of this child? What diagnostic workup should be performed? What is the appropriate disposition for this patient?

Introduction

Myocarditis is an inflammatory disease of the myocardium, occasionally extending to the epicardium and pericardium, which can lead to nonischemic dilated cardiomyopathy (DCM) and chronic heart failure.1 There are many causes of myocarditis, though a systemic viral illness is most commonly implicated.2 Presentation can be acute, subacute, or progressive/ chronic.3 Initial presentation often includes a prodromal flu-like illness, including respiratory and gastrointestinal symptoms.2 Specific symptoms may include cough, dyspnea, vomiting, myalgias, and significant tachycardia out of proportion to the degree of fever.4 More-severe cases may also present with heart failure, ventricular dysrhythmia, myocardial infarction, new-onset heart block, or cardiogenic shock.2 Given the variable presentation and disease course, a high index of suspicion is required. Pericarditis is an inflammatory disease of the pericardium, and it often presents with fever and chest pain.5,6 Mild cases are likely often self-limiting, so the incidence, especially in children, is unknown. More-severe cases can progress to pericardial effusion, pericardial constriction, recurrent pericarditis, or cardiac tamponade.6,7 The underlying etiology of pericarditis is quite variable and most commonly includes infection, malignancy, and rheumatologic conditions.6,7

This issue of Pediatric Emergency Medicine Practice provides an evidence-based approach to the evaluation and management of myocarditis and pericarditis in the pediatric patient, with an emphasis on recent advances in diagnosis and treatment.

Critical Appraisal Of The Literature

A literature review was performed using the keywords myocarditis or pericarditis in Ovid MEDLINER and PubMed, focusing on children aged 0 to 18 years. Well-designed randomized controlled trials and prospective and retrospective studies were included. Commonly referenced pediatric and adult studies, as well as historical publications, were also included. A search of the Cochrane Database of Systematic Reviews yielded 4 relevant publications, which were primarily comprised of adult studies.8-11 The websites of the American Heart Association (AHA) (www.heart.org) and the American Academy of Pediatrics (AAP) (www.aap.org) were searched for guidelines pertaining to myocarditis or pericarditis in children, and none were found. Commonly cited guidelines related to the diagnosis and management of pericardial diseases, published in 2004 by the European Society of CardiologyR (ESC) and revised in 2013, were reviewed.1,12,13 Canadian Cardiovascular Society (CCS) guidelines on the management of heart failure in children were also reviewed,14 as were other commonly cited guidelines related to the management of children with myocarditis.15,16 We identified 1 position statement from the ESC Working Group on Myocardial and Pericardial Disease pertaining to the evaluation of myocarditis.1

The literature on pediatric myocarditis mainly consists of case reports and series, small retrospective and prospective studies, and small randomized controlled trials, with primary outcome measures including death, transplant-free survival, and/or improvement in cardiac function. Larger well-designed randomized controlled trials are lacking, which is, in part, attributable to the rarity of such cases in the pediatric population as well as to discrepancies in the diagnosis of myocarditis.17 Myocarditis has historically been diagnosed using the Dallas criteria, which include pathologic evidence of inflammation and myocyte necrosis on endomyocardial biopsy samples.18 However, several studies have shown that the Dallas criteria are insufficient in many cases, even with adequate biopsy samples.19,20 As a result, many studies include “presumed” myocarditis or DCM, which may lead to the inclusion of etiologies distinct from myocarditis.4,21-24

Early literature on pediatric pericarditis predominantly consists of case reports describing specific infectious and systemic etiologies, with a paucity of robust studies. The literature has since shifted to focus on the role of corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs).12,13,25-28 More-recent investigation has focused on the use of colchicine for recurrent pericarditis. In 2013, Imazio et al published a randomized controlled trial among adults and showed that 4 patients would need to be treated with colchicine in addition to conventional NSAID therapy in order to prevent 1 episode of recurrence.29 A subsequent Cochrane Review concluded that there is moderatequality evidence that the addition of colchicine to NSAID therapy significantly reduces recurrence.8 The data for colchicine use in children with recurrent pericarditis remains limited to 1 small retrospective study and case reports.27,30,31

Risk Management Pitfalls In The Management Of Myocarditis And Pericarditis In Pediatric Patients

  1. “This kid just has gastroenteritis.”
    Myocarditis is a challenging diagnosis to make prior to overt symptoms of heart failure, and a high index of suspicion is required. When symptoms do not fit a typical picture, further consideration should be given to alternate diagnoses. Children with myocarditis often present with a flu-like illness and tachycardia out of proportion to the degree of fever.
  2. “The troponin is negative, so my patient can’t have myocarditis.”
    Troponin levels may have sufficient sensitivity to rule out myocarditis, but the test performance depends on the cut-off level defining a positive test. Current evidence suggests that troponin I and T lack adequate specificity in cases of pediatric myocarditis. While a negative troponin is reassuring, emergency clinicians should interpret this result in the context of the cut-off value used at their facility.
  3. “I gave 60 mL/kg of intravenous fluid to a child with myocarditis, and now he’s getting worse.”
    Children with myocarditis often present in shock, which prompts aggressive intravenous fluid administration. Failure to respond to an initial fluid bolus should raise concern for a cardiogenic cause, such as myocarditis. In cardiogenic shock, poor cardiac contractility leads to the development of pulmonary edema. Clinically, patients will develop labored breathing and crackles/rales on examination. Treatment should include inotropes and intravenous diuretics, such as furosemide.
  4. “I diagnosed my patient with myocarditis, admitted her to the floor team since she was stable, and didn’t consult cardiology.”
    rompt consultation with a pediatric cardiologist should be obtained in all cases of suspected myocarditis. Admission planning should start early and in conjunction with a pediatric intensivist, as patients can decompensate quickly. If there is no pediatric intensive care unit or cardiovascular intensive care unit at your center, plans for transfer to an appropriate center should be arranged early.
  5. “My patient has myocarditis with signs of hemodynamic compromise. I’ll start her on furosemide, and hopefully she’ll turn around without inotropes.”
    While diuresis is an essential component of treatment, inotropic support should not be withheld if patients present with signs of hemodynamic instability. Peripheral venous access should be obtained promptly. Providers should aim to restore cardiac contractility when choosing a vasopressor. Milrinone is the agent of choice; however, this may not be available in all emergency departments. Epinephrine is another excellent choice, with the addition of dobutamine, if needed.
  6. “My patient was crashing, and it looked like tamponade. I performed a pericardiocentesis over the anterior chest using a 10-mL syringe and a 22-gauge needle.”
    Pericardiocentesis is a potentially life-saving procedure, and knowledge of appropriate technique is critical. Clinicians should quickly sterilize the precordium, just below the xiphoid process. If there is time, local lidocaine should be infiltrated and sedation used as tolerated. A 2.5-inch or 3.5-inch 18-gauge spinal needle should be attached to a 20-mL to 50-mL syringe and inserted at a 45° angle just below and to the left of the xiphoid process, directed towards the left scapular tip. Maintain gentle suction on the syringe while slowly inserting the needle. If promptly available, point-of-care cardiac ultrasound should be used to visualize the procedure. Continuous cardiac monitoring should be used throughout the procedure. Ectopic beats or ST-segment elevation may indicate cardiac irritation from increased needle depth insertion.
  7. “The pericardiocentesis is done, but the patient is getting worse. What could have gone wrong?”
    Complications from pericardiocentesis are common. The most common complication is ventricular puncture, which may lead to hemopericardium. Other complications include dysrhythmia, pneumothorax, coronary artery or vein laceration, diaphragmatic perforation, puncture of the peritoneal cavity, and vasovagal episodes.
  8. “The ECG doesn’t show diffuse ST-segment elevation, so my patient can’t have pericarditis.”
    Diffuse ST-segment elevation occurs in the acute phase of the disease. Children with delayed presentation or recurrent disease may have diffusely inverted T waves or low-voltage QRS complexes.
  9. “Steroids can’t hurt, right?”
    Steroids increase the risk for development of recurrent pericarditis, and they are only recommended in refractory cases, or in cases where the underlying medical condition would be treated with such therapy (eg, autoimmune disease, known giant-cell myocarditis, or eosinophilic myocarditis).
  10. “I diagnosed my patient with pericarditis and treated him with high-dose aspirin.” Ibuprofen is the treatment of choice for acute pericarditis due to the beneficial effects on coronary blood flow and the minimal side-effect profile. Although no pediatric studies have compared different NSAIDs in the treatment of acute pericarditis, aspirin use in pediatric patients should be limited to patients with pericarditis after myocardial infarction or patients with risk of thrombosis.

Tables And Figures

Table 1. Etiologies Of Myocarditis

Table 2. Etiologies Of Pericaditis

References

Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study will be included in bold type following the references cited in this paper, as determined by the author, will be noted by an asterisk (*) next to the number of the reference.

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Authors

Kelly R. Bergmann, DO; Anupam Kharbanda, MD; Lauren Haveman, MD

Publication Date

July 2, 2015

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