Myocarditis With Complete Heart Block:

Challenges In Diagnosis And Treatment

Resource Center

M YO C A R D I T I S
Myocarditis With Complete
Heart Block: Challenges in
Diagnosis and Treatment

DEEPAK SHARMA, MD
Rapides Regional Medical Center

Dr Sharma is faculty in the department of emergency

medicine at Rapides Regional Medical Center in
Alexandria, La. He is also a preceptor for family practice
residents during their emergency medicine rotation.

Volume 52 - Issue 4 - April 2012

ABSTRACT: Children with acute myocarditis often

present with dyspnea at rest, exercise intolerance,
syncope, tachypnea, tachycardia, and hepatomegaly.
Various arrhythmias, complete heart block, and
tachycardia with metabolic acidosis may be present.
Fulminant myocarditis is associated with acute onset
of cardiovascular compromise. ECG may show sinus,
atrial, and ventricular tachycardia and, occasionally,
varying degrees of heart block, including complete
heart block. Cardiac enzyme levels are frequently
high. Findings on echocardiography to assess left
ventricular function may be indicative of the severity
of myocarditis and may have prognostic implications.

Myocarditis, or inflammation of the myocardial

muscle, is a rare potentially fatal disease in children.
It can result from multiple causes, including infection,
exposure to cardiac toxins, and autoimmune disease.
Viral infections, especially those caused by
enteroviruses, are the most common causes of
myocarditis in children and adolescents. The clinical
manifestations of myocarditis are nonspecific and
can be acute or chronic, although most children with
myocarditis have an acute presentation. Its
occurrence can be sporadic; however, epidemic
clusters and seasonal and geographic variations
have been reported.1,2

Here, I present a case of fatal fulminant myocarditis

with complete heart block and review the clinical and
management aspects of the condition in children.

CASE

A 13-year-old girl was brought to the emergency

department (ED) after a second episode of syncope
within the past 2 days. The first episode, which was
described by the family as very brief, perhaps less
than 5 seconds, occurred at home and was not
preceded by any palpitations or prodromal
symptoms. The second episode occurred at school
during her regular physical activity and lasted about
30 to 40 seconds. The patient had no significant
medical or family history. Height and weight were
average for age. She had been an active child and
participated in school sports without any complaints
of dizziness, dyspnea, palpitations, or chest pain. She
had no recent illnesses or symptoms suggestive of a
viral illness and no recent immunizations. She was
not taking any medications.

In the ED. The patient was awake and oriented, with

a supine blood pressure (BP) of 86/64 mm Hg, heart
rate of 78 beats per minute, respiration rate of 20
breaths per minute, and temperature of 36.4ºC
(97.6ºF). When the patient was asked to stand, she
had a near-syncopal episode. Her sitting BP dropped
to 63/44 mm of Hg, without any significant increase
in heart rate.

Telemetry monitoring was immediately established

and showed brief episodes of bradycardia, like the
one shown in Figure 1. An intravenous fluid bolus was
given with improvement in symptoms.

Results of laboratory tests, including serum

electrolyte levels, complete blood cell counts, drug
screens, and urine pregnancy test, were normal.
Serum cardiac markers were abnormal: creatine
phosphokinase, 1064 U/L (normal, 150 U/L); creatine
kinase (CK)—MB fraction, 89.5 ng/mL (normal, 0 to 5
ng/mL); ratio of CK-MB to total CK (relative index),
8.4%; troponin T, 48.4 ng/mL (normal, less than 0.05
ng/mL); and brain natriuretic peptide, 403 pg/mL
(normal, less than 100 pg/mL).

A chest film showed no cardiomegaly or pulmonary

congestion.

Hospital course. The patient was admitted to the

pediatric ICU, where an ECG indicated complete
heart block and the development of myocardial
necrosis (Figure 2). A stat echo---cardiogram was
also performed. This showed global left ventricular
hypokinesia, with an estimated ejection fraction of
15% to 20%. Serum complement levels (C3 and C4)
were within normal limits, and polymerase chain
reaction (PCR) analysis was negative for Enterovirus
RNA.

Pediatric cardiology was consulted. Complete heart

block secondary to myocarditis was diagnosed, and
treatment with dobutamine and milrinone was
started.

The patient initially responded well to treatment, with

a heart rate of 120 beats per minute and
normalization of supine BP. However, she continued
to have transient episodes of complete heart block;
in one instance, atropine, 0.5 mg IV push, was
required. Eight hours post admission, ventricular
tachycardia developed, and the patient’s pulse was
lost. Despite all resuscitative e!orts, she could not be
revived and died 2 hours later.

An autopsy was requested by the admitting team.

Unfortunately, the family declined it. n

INCIDENCE

The true incidence of myocarditis in children is

unknown. This is, in part, because there are no
sensitive and specific diagnostic tests for the
condition, also its presentation is very variable. In
one retrospective study from a tertiary Canadian
center, the estimated prevalence of myocarditis was
0.5 cases per 10,000 visits.3

ETIOLOGY

Multiple causes have been attributed to the

development of myocarditis in children (Table). In
infants and children who died of sudden cardiac
death, inflammatory infiltrates in the myocardium
have been found in 3% to 40% on autopsy.4-8

CLINICAL MANIFESTATIONS

The clinical presentation of myocarditis is diverse.

The plethora of signs and symptoms on presentation
ranges from acute and fulminant to chronic and
insidious. Clinical manifestations also vary
depending on the cause. Viral myocarditis frequently
manifests as a prodrome of fever, myalgia, and
malaise several days before heart failure. Infants may
have fever, lethargy, poor feeding, diaphoresis with
feeding, and failure to thrive.

Children with acute myocarditis often present with

dyspnea at rest, exercise intolerance, syncope,
tachypnea, tachycardia, and hepatomegaly.9-12
Predominant respiratory symptoms often lead to the
incorrect initial diagnosis of lower respiratory tract
infection.3 Patients may have chest pain or present in
fulminant cardiogenic shock.13 Various arrhythmias,
complete heart block, and tachycardia with
metabolic acidosis may be present. Metabolic
acidosis and tachycardia may be important
indicators of the extent of myocardial
involvement.1,11,14,15 Fulminant myocarditis is
associated with acute onset of cardiovascular
compromise.16 In some cases, myocarditis may be
totally asymptomatic and present as sudden death.

DIAGNOSTIC STUDIES

Various diagnostic studies are available to assist with

the identification of myocarditis. Chest radiographs
frequently show cardiomegaly and pulmonary
congestion. However, the initial chest film findings in
acute myocarditis can be normal. ECG may show low
voltages and inverted T waves.9-11 Sinus, atrial, and
ventricular tachycardia may be seen, and
occasionally, varying degrees of heart block,
including complete heart block, may occur.1,11,14,15,17-19

Cardiac enzyme levels are frequently high and are

indicative of myocardial necrosis. Myocarditis
correlates better with cardiac troponin I or T than
with CK-MB in patients who have biopsy-proven
myocarditis.20,21

Echocardiography should be performed to assess left

ventricular function and the degree of mitral
regurgitation.12 Left ventricular size may be indicative
of the severity of myocarditis.16

MRI with gadolinium contrast can document the

location and extent of inflammation.22-24 Other
diagnostic tests, such as acute and convalescent
antibody tests, and testing for systemic diseases may
be helpful.

The role of endomyocardial biopsy has been

extensively studied. The Dallas criteria, developed by
a panel of cardiac pathologists, are most widely used
by investigators for histopathological diagnosis and
classification of myocarditis.25,26 However,
endomyocardial biopsy lacks sensitivity. Newer
techniques of in situ hybridization and PCR
amplification of viral DNA improve the sensitivity.

TREATMENT

The mainstays of treatment of acute fulminant

myocarditis are monitoring, supportive care, and
standard regimens for heart failure. The overall
treatment of myocarditis can be divided into the
following components:

•Heart failure.

•Arrhythmias.

•Myocardial damage.

Heart failure. In the acute phase, diuretics are used

after load-reducing agents and inotropic drugs.
Intravenous agents, such as dopamine, dobutamine,
and milrinone, help prevent circulatory collapse.
Sedation, intubation, and mechanical ventilation
decrease metabolic demand in patients with
cardiogenic shock. More severe disease may require
extracorporeal membrane oxygenation or a
ventricular assist device. These modalities were
unavailable at my institution, and the patient’s rapid
decline limited the treatment options. For patients
who progress to chronic heart failure, diuretics,
angiotensin-converting enzyme inhibitors, digoxin,
and aldosterone inhibitors are well-accepted
therapies. Treatment with ß-blockers has not been
shown to improve survival rate in children with
chronic heart failure, in contrast to that in adults with
chronic heart failure.

Table-Majorcausesofmyocarditis
Infectiouscauses
Viral Bacterial Spirochetal Rickettsial
CoxsackieBvirus Diphtheria Syphilis Typhus
Echovirus Tuberculosis Leptospirosis Qfever
Epstein-Borrvirus Salmonella Relapsingfever RockyMountainspottedfever
Cytomegalovirus Staphylccoccal Lymedisease
Adenovirus Gonococcal Protozoal
HIV Clostridiol Mycotic Chagasdisease(SouthAmerican
HepotitisBandC Brucellosis Condidiosis tryponosomisis)
Rubeol Psittacosis Histoplasmosis SleepingsicknessAfrican
Rubella Tetanus Sporctrichosis trypanosomiasis}
Varicella Tularemia Coccidiomycosis Toxoplosmosis
Mumps Streptococcal Aspergillosis Malaria
Vaccinia(smallpoxvaccine) legionello Blastomycosis Leishmaniasis
Variola Meningococcal Cryptococcosis Amebiasis
Parvovirus Haemophilus Actinomyocosis
InfluenzaAandB Pneumococcal Mucormycosis Helminthic
Herpesvirus Cholera Nocardia Trichinosis
Poliomyelitis Chlamydia Echinococcosis
Robies Mycoplasma Schistosomiasis
Arbovirus Ascariasis
Dengue Filariosis
Yellowfever Parogonimiasis
Strongyloidiasis

Noninfectiouscauses
Cardiotoxins Hypersensitivityreactions Systemicdisorders
Catecholamines Antibiotics/penicillins, Collogen-vasculardiseases
Anthracyclines cepholosporins,sulfonamides) Sarcoidosis
Cyclophosphamide Diuretics(thiazide,Icopl Celiocdisease
Cocaine Dobutomine Kawasakidisease
Heavymetals(copper,lead,iron) Lithium Hypereosinophilia
Alcohol Tetonustoxoid Wegenergranulmotosis
Arsenic Clozapine Thyrotoxicosis
Carbonmonoxide Methyldopa Inflammatoryboweldisease
Methysergide Insectbites(bee,wasp,spider, [Crohndisease,ulcerativecolitis)
scorpion)
Snakebites Radiation

Arrhythmias. Although arrhythmias are common in

myocarditis and may exacerbate the symptoms of
heart failure, treatment with most antiarrhythmic
drugs often leads to hemodynamic instability. Thus,
antiarrhythmic therapy is used only when the
benefits outweigh the risks.

Supraventricular or ventricular arrhythmia associated

with acute hemodynamic instability should be
cardioverted without delay. Supraventricular
arrhythmias in myocarditis have diverse
mechanisms, and a single treatment strategy may
not work for all patients so therapy must be
individualized.

High-grade ventricular arrhythmias should be treated

cautiously. Lidocaine is the first-line treatment.
During resuscitation attempts, this patient was given
lidocaine among other ACLS drugs. The safety profile
of amiodarone, which is widely used in adults, is less
well demonstrated in children.27

Complete heart block, an often transient conduction

abnormality, may be an indication for transvenous
pacing. This patient was placed in a telemetric
monitoring room with bedside transcutaneous
pacing capabilities; however, she did not require
pacing.

Myocardial damage. The hallmark of myocarditis is

myocardial injury. It is caused by both direct viral
damage and the immune response to infection.
Immunosuppressive therapy has a limited role in
myocarditis, as shown in the Myocarditis Treatment
Trial.28

Use of corticosteroid therapy in children with

myocarditis and its e!ect on left ventricular function
have been incompletely studied. Most studies have
been small with multiple limitations.

Intravenous g globulin has been tried, considering its

e!icacy in other immune-mediated diseases, and
experimental studies in animals also support its
e!icacy.29,30 High doses of intravenous
immunoglobulin (IVIG) have demonstrated greater
ventricular function in smaller studies.31 Hence, use
of IVIG (2 g/kg over 24 hours) is recommended for
children with endomyocardial biopsy–proven
myocarditis. The use of corticosteroids and
immunosuppressive agents is recommended for
patients with myocarditis associated with systemic
autoimmune disease.

PROGNOSIS

The prognosis for patients with acute myocarditis

varies and depends on clinical presentation, ejection
fraction, and pulmonary artery pressure.32,33 Limited
information is available on the natural course of
myocarditis in children. This is because of the small
number of patients a!ected, poor data collection,
lack of biopsy confirmation of myocarditis, and
inclusion of other causes of myocardial dysfunction.
Complete recovery is more likely to occur in adults
with fulminant rather than acute myocarditis.
However, similar data are not available in children.

References
1. Mounts AW, Amr S, Jamshidi R, et al. A cluster of
fulminant myocarditis cases in children, Baltimore,
Maryland, 1997. Pediatr Cardiol. 2001;22:34-39.

2. Strikas RA, Anderson LJ, Parker RA. Temporal and

geographic patterns of isolates of nonpolio enterovirus in
the United States, 1970-1983. J Infect Dis. 1986;153:346-
351.

3. Freedman SB, Haladyn JK, Floh A, et al. Pediatric

myocarditis: emergency department clinical findings and
diagnostic evaluation. Pediatrics. 2007;120:1278-1285.

4. Forcada P, Beigelman R, Milei J. Inapparent myocarditis

and sudden death in pediatrics. Diagnosis by
immunohistochemical staining. Int J Cardiol. 1996;56:93-
97.

5. Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden

cardiac death during competitive sports activities in
Minnesota high school athletes. J Am Coll
Cardiol. 1998;32:1881-1884.

6. Neuspiel DR, Kuller LH. Sudden and unexpected

natural death in childhood and
adolescence.JAMA. 1985;254:1321-1325.

7. Topaz O, Edwards JE. Pathologic features of sudden

death in children, adolescents, and young
adults. Chest. 1985;87:476-482.

8. Niimura I, Maki T. Sudden cardiac death in

childhood. Jpn Circ J. 1989;53:1571-1580.

9. Bonadio WA, Losek JD. Infants with myocarditis

presenting with severe respiratory distress and
shock. Pediatr Emerg Care. 1987;3:110-113.

10. Press S, Lipkind RS. Acute myocarditis in infants.

Initial presentation. Clin Pediatr (Phila).1990;29:73-76.

11. Greenwood RD, Hadas AS, Fyler DC. The clinical

course of primary myocardial disease in infants and
children. Am Heart J. 1976;92:549-560.

12. Towbin JA. Cardiomyopathies. In: Moller JH, Ho!man

JI, eds. Pediatric Cardiovascular Medicine. New York:
Churchill Livingstone; 2000:758.

13. Bradin SA. Cardiogenic shock secondary to viral

myocarditis. Consultant For Pediatricians.2008;7:190-192.

14. Wang JN, Tsai YC, Lee WL, et al. Complete

atrioventricular block following myocarditis in
children. Pediatr Cardiol. 2002;23:518-521.

15. Batra AS, Epstein D, Silka MJ. The clinical course of

acquired complete heart block in children with acute
myocarditis. Pediatr Cardiol. 2003;24:495-497.

16. Felker GM, Boehmer JP, Hruban RH, et al.

Echocardiographic findings in fulminant and acute
myocarditis. J Am Coll Cardiol. 2000;36:227-232.

17. Ander DC, Heilpern KL. Myocarditis. In: Chan TC,

Brady WJ, Harrigan RA, et al. ECG in Emergency Medicine
and Acute Care. Philadelphia: Elsevier Mosby; 2006:204-
206.

18. Friedman RA, Kearney DL, Moak JP, et al. Persistence

of ventricular arrhythmia after resolution of occult
myocarditis in children and young adults. J Am Coll
Cardiol. 1994;24:780-783.

19. Balaji S, Wiles HB, Sens MA, Gillette

PC. Immunosuppressive treatment for myocarditis and
borderline myocarditis in children with ventricular ectopic
rhythm. Br Heart J. 1994;72:354-359.

20. Smith SC, Ladenson JH, Mason JW, Ja!e AS.

Elevations of cardiac troponin I associated with
myocarditis. Experimental and clinical
correlates. Circulation. 1997;95:163-168.

21. Lauer B, Niederau C, Kühl U, et al. Cardiac troponin T

in patients with clinically suspected myocarditis. J Am Coll
Cardiol. 1997;30:1354-1359.

22. Friedrich MG, Strohm O, Schulz-Menger J, et al.

Contrast media-enhanced magnetic resonance imaging
visualizes myocardial changes in the course of viral
myocarditis. Circulation. 1998;97:
1802-1809.

23. Laissy JP, Messin B, Varenne O, et al. MRI of acute

myocarditis: a comprehensive approach based on various
imaging sequences. Chest. 2002;122:1638-1648.

24. Roditi GH, Hartnell GG, Cohen MC. MRI changes in

myocarditis—evaluation with spin echo, cine MR
angiography and contrast enhanced spin echo
imaging. Clin Radiol. 2000;55:752-758.

25. Aretz HT, Billingham ME, Edwards WD, et al.

Myocarditis. A histopathologic definition and
classification. Am J Cardiovasc Pathol. 1987;1:3-14.

26. Cooper LT, Baughman KL, Feldman AM, et al. The role
of endomyocardial biopsy in the management of
cardiovascular disease: a scientific statement from the
American Heart Association, the American College of
Cardiology, and the European Society of
Cardiology. Circulation. 2007;116:
2216-2233.

27. Saul JP, Scott WA, Brown S, et al. Intravenous

amiodarone for incessant tachyarrhythmias
in children: a randomized, double-blind, antiarrhyth-mic
drug trial. Circulation. 2005;112:
3470-3477.

28. Mason JW, O’Connell JB, Herskowitz A, et al. A clinical

trial of immunosuppresive therapy for myocarditis. The
Myocarditis Treatment Trial Investigators. N Engl J
Med. 1995;333:
269-275.

29. Weller AH, Hall M, Huber SA. Polyclonal

immunoglobulin therapy protects against cardiac damage
in experimental coxsackievirus-induced myocarditis. Eur
Heart J. 1992;13:115-119.

30. George J, Barshack I, Malka E, et al. The e!ect of

intravenous immunoglobulins on the progression of
experimental autoimmune myocarditis in the rat. Exp Mol
Pathol. 2001;71:55-62.

31. Drucker NA, Colan SD, Lewis AB, et al.

Gamma-globulin treatment of acute myocarditis in the
pediatric population. Circulation. 1994;89:
252-257.

32. Starling RC, Cooper LT, Dec GW, et al. Left ventricular
diameter predicts recovery in acute cardiomyopathy:
results of the IMAC 2 Trial [abstract
3002]. Circulation. 2007;116:II671-II672.

33. Magnani JW, Danik HJ, Dec GW Jr, DiSalvo

TG. Survival in biopsy-proven myocarditis: a long-term
retrospective analysis of the histopathologic, clinical, and
hemodynamic predictors. Am Heart J. 2006;151:463-470.

ADVERTISEMENT

Current Consultant Issue

Previous Issues

Early View

ADVERTISEMENT

RESEARCH SUMMARIES

Evidence Against Using a Chronotropic

Strategy for Blood Pressure Control

Prevalence of Patients Developing

Varicella-Zoster Reactivation After
mRNA COVID-19 Vaccine

Maternal COVID-19 Vaccination

Protects Against Infection in Early
Infancy

COVID-19 Pandemic Had Little Impact

on Sickle Cell Disease Outcomes

Fentanyl, Stimulant Overdose Deaths

Increase 50-Fold in More Than a
Decade

Trending Articles

Ptosis and Rhythmic Eye Movement in a

Young Child

What Is This Rash on an Older Man’s

Groin?

Progressive Renal Failure in a 24-Year-

Old Man

HIV Prevention and Management

Among Adolescents

Respiratory Viruses in Winter 2023-

2024: Vaccination Against RSV

ADVERTISEMENT

RESOURCES

JOURNAL

ABOUT

Editorial Description

Section Editors

Contact Us