In this brief review we describe the salient features of pericarditis in children and highlight the differences when compared to adult pericarditis.
Pericarditis in children is infrequent with sparse epidemiological data. Pericarditis accounts for <0.2% of the emergency visits of children without prior heart disease presenting with chest pain to a tertiary pediatric emergency setting.1 As in adults, childhood pericarditis syndromes such as acute, chronic, recurrent, constrictive, effusive-constrictive and effusion/tamponade have been described. 2,3 There is a changing trend of the underlying etiology of pediatric pericarditis, with infectious pericarditis becoming uncommon and post-cardiotomy syndrome being a major underlying etiology.4,5 In a large database study of hospitalized pediatric patients with pericarditis and pericardial effusions, post-cardiac surgery (54%), neoplasia (13%), renal (13%), idiopathic or viral pericarditis (5%) and rheumatologic (5%) were the major underlying etiologies.5 Pediatric pericarditis in the emergency room setting is also more likely to be secondary to post-cardiac surgery or due to a systemic illness, although idiopathic or viral pericarditis occur more frequently.4 Post-cardiotomy syndrome is common after surgical closure of secundum atrial septal defects (ASD), with incidence as high as 28%.6 ASD closure is also a risk factor for recurrent pericarditis, which is fortunately uncommon in childhood but can have a long protracted course with frequent recurrences.3 Idiopathic or viral pericarditis occurs more frequently in adolescents especially in males.5 Pericardial effusion occurring in the setting of bone marrow transplantation (BMT) has been increasingly recognized. This is of unclear etiology but is thought to be related to underlying serositis. Graft versus host disease and transplant associated thrombotic microangiopathy have been noted to be risk-factors.7
The clinical presentation of pericarditis in children is similar to that of adults with certain caveats. Post-cardiotomy syndrome tends to occur within 1-2 weeks of the surgery. Infants and young children tend to be fussy and have decreased feeding with tachycardia being an important physical sign. Small pericardial effusion noted on the routine post-operative echocardiograms might be predictive of post-pericardiotomy syndrome. Pericardial effusion that occurs post-BMT requires a high degree of suspicion, since this can be asymptomatic for a prolonged period of time. Particular attention to resting tachycardia is needed and routine surveillance echocardiograms have been instituted in some institutions. Chest pain is rarely due to an underlying primary cardiac etiology in children, but the characteristics of the pain along with EKG changes and inflammatory markers might be helpful.8 Early repolarization tends to be a benign common finding in adolescents and might mimic the ST-segment elevation noted with pericarditis.
Given the limited patient population and number of studies on pediatric pericarditis it is not surprising that there is lack of evidence-based guidelines for multi-modality imaging in pediatric pericarditis unlike those in adults by the American Society of Echocardiography and European Society of Cardiology.9,10, 11 Although echocardiography appears as a class I recommendation for adult patients with acute pericarditis, it is not uncommon for children with suspected acute pericarditis to be treated empirically without echocardiography. In children with complicated clinical course or with history of recurrent pericarditis, imaging with cardiac MRI (CMR) or cardiac CT might be appropriate, although evidence is lacking. CMR has become an integral part of the multi-modality imaging in adult pericarditis, however in younger children the need for sedation might be a limiting factor.
Non-steroidal anti-inflammatory drugs (NSAID) are most frequently used to treat childhood pericarditis, with the use of colchicine restricted to few centers.5 The European Society of Cardiology (ESC) recommends high dose NSAIDs as the first line therapy for pediatric pericarditis, with colchicine as a second line therapy.11 A recently completed systematic review demonstrated lack of evidence to support or discourage the use of colchicine in pediatric pericarditis.12 In adult and pediatric patients with refractory recurrent pericarditis, there is now multiple studies demonstrating safety and efficacy of interleukin-1 receptor antagonists such as anakinra.13 [Note that anakinra is an off-label use for pericarditis] In selected patients, pericardectomy can also be safely performed in childhood for refractory pericarditis with excellent outcome. 14
Future long-term well powered studies are needed to address the role of CMR, colchicine and newer anti-inflammatory agents such as anakinra in pediatric pericarditis along with understanding the underlying pathophysiology of specific pericardial etiologies such as post-BMT pericardial effusion.
- Drossner DM, Hirsh DA, Sturm JJ et al. Cardiac disease in pediatric patients presenting to a pediatric ED with chest pain. Am J Emerg Med 2011;29:632-8.
- Zurick AO, 3rd, Klein AL. Effusive-constrictive pericarditis. Journal of the American College of Cardiology 2010;56:86.
- Raatikka M, Pelkonen PM, Karjalainen J, Jokinen EV. Recurrent pericarditis in children and adolescents: report of 15 cases. Journal of the American College of Cardiology 2003;42:759-764.
- Roodpeyma S, Sadeghian N. Acute pericarditis in childhood: a 10-year experience. Pediatric cardiology 2000;21:363-367.
- Shakti D, Hehn R, Gauvreau K, Sundel RP, Newburger JW. Idiopathic pericarditis and pericardial effusion in children: contemporary epidemiology and management. Journal of the American Heart Association 2014;3:e001483.
- Heching HJ, Bacha EA, Liberman L. Post-pericardiotomy syndrome in pediatric patients following surgical closure of secundum atrial septal defects: incidence and risk factors. Pediatr Cardiol 2015;36:498-502.
- Lerner D, Dandoy C, Hirsch R, Laskin B, Davies SM, Jodele S. Pericardial effusion in pediatric SCT recipients with thrombotic microangiopathy. Bone Marrow Transplant 2014;49:862-3.
- Kane DA, Fulton DR, Saleeb S, Zhou J, Lock JE, Geggel RL. Needles in hay: chest pain as the presenting symptom in children with serious underlying cardiac pathology. Congenit Heart Dis 2010;5:366-73.
- Klein AL, Abbara S, Agler DA et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2013;26:965-1012.e15.
- Cosyns B, Plein S, Nihoyanopoulos P et al. European Association of Cardiovascular Imaging (EACVI) position paper: Multimodality imaging in pericardial disease. European heart journal cardiovascular Imaging 2015;16:12-31.
- Authors/Task Force M, Adler Y, Charron P et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC)Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). European heart journal 2015;36:2921-2964.
- Alabed S, Perez-Gaxiola G, Burls A. Colchicine for children with pericarditis: systematic review of clinical studies. Arch Dis Child 2016.
- Baskar S, Klein AL, Zeft A. The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review. Cardiol Res Pract 2016;2016:7840724.
- Thompson JL, Burkhart HM, Dearani JA, Cetta F, Oh JK, Schaff HV. Pericardiectomy for pericarditis in the pediatric population. The Annals of Thoracic Surgery 2009;88:1546-1550.
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