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.
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?
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.
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
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.
Kelly R. Bergmann, DO; Anupam Kharbanda, MD; Lauren Haveman, MD
July 2, 2015