What Causes Myocarditis?

Patient Presentation
An 8-year-old female came to the pediatric intensive care unit after a viral prodrome about 2 weeks before that did not improve. She has continued fatigue, shortness of breath, dyspnea, and lethargy. Over the past 24 hours she could not get up to use the restroom and had developed abdominal pain. She was brought to the local emergency room, where they noted her to have sinus tachycardia with abnormal QRS complexes but no specific AV block. A point-of-care-ultrasound showed tachycardia but with decreased overall ventricular motion. The patient was transferred by ambulance to a regional children’s hospital.

The past medical history showed her to be a healthy female who was fully vaccinated. The family history was negative for cardiac problems except for a great grandfather who had a myocardial infarction and was a smoker. The review of systems was positive for fever, rhinorrhea, cough, sore throat, and conjunctivitis.

The pertinent physical exam showed a heart rate of 108 beats/minutes, respiratory rate of 32/minute, blood pressure was 86/58 and oxygen saturation was 93%. Capillary refill was 3 seconds. She appeared very tired but had no obvious cyanosis. HEENT revealed bilateral conjunctivitis without purulent exudate, and she had pharyngitis with exudate. Her peripheral pulses were present but decreased. Heart was normal S1, S2 with a Grade I-II murmur best heard at lower left sternal boarder. She had diminished lung sounds at the bases and her liver was 4 cm below the costal margin and she complained of tenderness with liver palpitation. Spleen was not palpable. She had 1+ edema of her feet bilaterally and her hands appeared somewhat edematous.

The diagnosis of mild cardiac failure of indeterminant cause at that time was made. She was started on fluid resuscitation and inotropic medication. Echocardiography showed thickened myocardium and LV enlargement. It was thought this was due to myocarditis and cardiac magnetic resonance imaging was consistent with it. Laboratory evaluation eventually identified adenovirus as the cause. She slowly recovered but did need treatment for a tachyarrhythmia. She was discharged after 17 days in the hospital.

Discussion
Myocarditis is inflammation of the cardiac myocytes which can results in myocardial edema, injury and/or necrosis. It can be acute or chronic. Pericarditis is the most common inflammatory heart disease with myocarditis being second. The incidence is about 1.4 – 2.1 cases per 100,000 children/year. Prognosis is worse in newborns and about 5-20% of patients require heart transplantation due to cardiac failure.

Myocarditis is one cause of cardiomyopathy which is a broader term where there is functional or structural problems with the heart muscle. Cardiomyopathy is one cause of cardiac failure where the heart cannot adequately pump enough blood. Hypertrophic cardiomyopathy is one cause of primary cardiomyopathy that can cause cardiac failure. Myocarditis is an acquired or secondary cause of cardiomyopathy (often dilated) which again can lead to cardiac failure.

Patients with myocarditis can have a variety of presentations. Many are due to the poorly functioning myocardium including fatigue, shortness of breath, arrhythmias and syncope. It can also present fulminantly with patients in cardiogenic shock or sudden cardiac death. Clinical characteristics can include:

  • History
    • Viral prodrome (very common)
    • Arrhythmias
    • Sudden cardiac death
    • Syncope
  • Signs
    • Cyanosis
    • Decreased peripheral pulses
    • Edema
    • Hepatomegaly
    • Murmur and/or gallop
    • Peripheral edema
    • Respiratory distress
    • Tachycardia
    • Tachypnea
  • Symptoms
    • Fatigue (very common)
    • Cough
    • Dyspnea/shortness of breath
    • Fever
    • Nausea/emesis/diarrhea
    • Pain
      • Abdominal
      • Chest
    • Palpitations
    • Rhinorrhea

Diagnosis is made based on findings on electrocardiogram, echocardiogram and cardiac magnetic resonance imaging usually. Endomyocardial biopsy still remains a standard but is not necessary in all cases to make the diagnosis.

Treatment remains often supportive as many of the causes cannot be directly treated themselves. Treatment of arrhythmias and maintenance of cardiac output are critical. Immunotherapy and corticosteroids may be considered but the American Heart Association in 2021 stated “[t]here are insufficient data in children to permit evidence-based recommendations for myocarditis-specific immunotherapy.”

Learning Point
Causes of myocarditis predominate with viral etiologies but there are many others including:

  • Infectious
    • Viral
      • Adenovirus
      • Coxsackie virus
      • COVID
      • Enteroviruses
      • HIV
      • Human herpesvirus 6
      • Parvovirus B-19
      • Influenza
      • Rubella
      • Rubeola
    • Bacterial
      • Borrelia burgdorferi
      • Haemophilus influenza
      • Staphylococcus
      • Streptococcus
    • Fungal
      • Candida
      • Histoplasma
    • Parasites
      • Trypanosoma cruzi
      • Rickettsia rickettsii
  • Drugs/chemical
    • Anthracycline chemotherapy
    • Antibiotics
    • Diuretic
    • Central nervous system
      • Clonidine
    • Substance abuse
      • Alcohol
      • Cocaine
  • Autoimmune disease
    • Kawasaki disease
    • Giant cell
    • Systemic disease
      • Systemic Lupus erytematosus
  • Other
    • Graft rejection
    • Insect venom
    • Radiation
Questions for Further Discussion
1. How does pericarditis present? A review can be found here
2. What are different types of cardiomyopathy? A review can be found here
3. What are clinical presentations of adenovirus?
4. What are causes of sudden cardiac death? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews. Information prescriptions for patients can be found at MedlinePlus for this topic: Cardiomyopathy

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Law YM, Lal AK, Chen S, et.al. Diagnosis and Management of Myocarditis in Children. Circulation. Circulation. 2021 Aug 10;144(6):e123-e135.

Mille F, Burstein D. Diagnosis and Management of Pediatric Heart Failure. Indian J Pediatr. 2023;90(5):492-500. doi:10.1007/s12098-022-04433-4

Tsatsopoulou A, Protonotarios I, Xylouri Z, et al. Cardiomyopathies in children: An overview. Hellenic J Cardiol. 2023;72:43-56. doi:10.1016/j.hjc.2023.02.007

Williams JL, Jacobs HM, Lee S. Pediatric Myocarditis. Cardiol Ther. 2023;12(2):243-260. doi:10.1007/s40119-023-00309-6

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa