Pediatrics

Pericarditis

OVERVIEW: What every practitioner needs to know

Are you sure your patient has pericarditis? What are the typical findings for this disease?

Pericarditis is defined as an inflammatory reaction involving the two layers of fibroserous pericardium in response to infectious or non-infectious injury. Chronic pericarditis is defined as pericarditis lasting for >3months. Pericarditis is termed recurrent pericarditis when pericarditis relapses after weaning of medications. The precise incidence of pericarditis is unknown with evidence that it can often pass unnoticed.

Precordial chest pain is often the presenting symptom in adults with pericarditis, in children this can be less so. The chest pain is usually worse when supine or with any movement of the chest and relieved with sitting upright or leaning forward. The site, radiation, severity and character of the chest pain can be highly variable. Other nonspecific symptoms that can be associated with pericarditis include vomiting, cough, dyspnea, abdominal pain and fever.

Physical examination findings:

While its absence does not exclude pericarditis, the presence of a pericardial rub over the precordial area upon auscultation is a typical finding. It is usually best heard in the left parasternal area and in a sitting position or leaning forward. With increasing size of the pericardial effusion, the heart sounds may sound muffled and the pericaridal rub may abate.

What physical examination findings are associated with cardiac tamponade physiology?

Cardiac tamponade is associated with significant pericardial effusion and may show the following on examination: narrow pulse pressure, pulsus paradoxus (i.e. >10mmHg exaggeration of the normal respiratory variation in blood pressure), hypotension, tachycardia, increased venous pressure, hepatomegaly, edema, tachypnea, and fever. The patient often appears anxious and distressed, often with cyanosis and dyspnea.

What other disease/condition shares some of these symptoms?

In adults the symptoms of chest pain from pericarditis can often be confused with symptoms from myocardial infarction. Myocardial infarction is obviously much rarer in the pediatric population so the pediatric care provider often is wrestling with a reordered differential diagnosis for chest pain including nonorganic (idiopathic or psychogenic) causes as well as organic causes such as costochondritis, pneumonic causes (e.g., asthma, pneumothorax, sickle cell crisis, etc), esophagitis, other heart disease (e.g., arrhythmias, myocarditis, cardiomyopathies, mitral valve prolapse, and certain structural heart disease lesions).

What caused this disease to develop at this time?

There are both infectious and non-infectious causes of pericarditis. Approximately 40-85% of pericarditis cases are idiopathic. See Table I.

Table I.

Infectious causes
VIRAL(almost always associated with myocarditis) BACTERIAL PARASITIC FUNGAL
Enteroviruses (Coxsakie A and B) Streptococcus (pneumonia, pyogenes, viridans) Echinococcus Histoplasma
Adenovirus Meningococcus Toxoplasmosis Actinomycosis
Influenza A and B Staphylococcus (aureus, epidermidis) Candidiasis
Varicella Mycoplasma pneumonia Blastomycosis
Human immunodeficiency virus Mycobacterium tuberculosis Aspergillosis
Mumps Coxiella burnetii
Ebstein Barr virus Gonococcus
Hepatitis B Listeria monocytogenes
Hepatitis C Spirochetes
Rubella Salmonella
Parvovirus B19 Haemophilus influenza
Human herpes virus 6 Tularemia

Non-infectious causes:

Autoimmune: Systemic lupus erythematosus, Rheumatoid arthritis,

Sjogren syndrome, systemic sclerosis, rheumatic fever, sarcoidosis,

Wegener granulomatosis, Reiter syndrome, ankylosing spondylitis,

Behcet syndrome, autoreactive pericarditis

Metabolic: Uremia, chylopericardium, myxedema

Neoplastic: Primary and metastatic, radiotherapy, bleding diathesis

Drug related: Procainamide, Hydralazine, Isoniazid, Phenytoin

Iatrogenic

Direct trauma

Post operative (post pericardiotomy syndrome)

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

Based on the history, laboratory testing can be performed to pinpoint a certain etiology. In general, inflammatory markers (CRP, ESR) and cardiac enzymes (troponin and BNP) are recommended. For infectious diseases, appropriate cultures and viral serology should be considered.

Characteristic Electrocardiogram findings:

1. ST segment elevation in limb and precordial leads (especially in the LV area).

2. 2-3 days: ST segment begin to normalize. Small, upright T wave.

3. 2-4 weeks (may persist for 2 months): Inverted T waves with isoelectric ST segment.

4. Pericardial effusion: low QRS voltages (<5mm amplitude in limb leads) in multiple leads.

Would imaging studies be helpful? If so, which ones?

Chest x-ray (although may have unspecific findings, it is easily accessible with low radiation exposure) findings:  Epicardial halo (luscent lines within the cardiopericardial shadow); Waterbottle silhouette (globular cardiomegaly with sharp margins) in large pericardial effusions.

Echocardiogram (relatively accessible in medical centers, no radiation exposure, non-invasive; technical expertise required) will help show: Effusion size and location, pericardial thickness, cardiac tamponade physiology (collapse of right sided chambers, early diastolic septal bounce, exaggerated respiratory changes in atrioventricular valve inflow patterns), constrictive versus restrictive physiology, cardiac function, associated structural heart disease.

Computed tomography (CT) (for further imaging indicated for better identification of anatomy/structure): Pericardial thickening and calcification, localization, loculations, associated organ disease (lungs and liver), coronary arteries, chamber dimensions.

Cardiac Magnetic Resonance (may not be readily accessible to all medical centers; requires expertise in acquiring images and interpretation): pericardial thickening, pleural effusion, pericardial inflammation and masses, cardiac function, pericardial effusion, myocarditis.

If you are able to confirm that the patient has pericarditis, what treatment should be initiated?

Most cases of acute pericarditis are self limited. In those cases where definitive etiology has been determined, directed therapy against the inciting disease should be initiated. The goal is complete treatment of the pathogen or causative illness. Bacterial pericarditis requires prompt antibiotic therapy as well as strong consideration for surgical evacuation of pericardial fluid.

In general, the current standard of care for the most common forms of pericarditis (idiopathic and viral) is to use non-steroidal inflammatory drugs (NSAID), as the key point in treatment to control inflammation and pain. (See Table II)

Table II.

Analgesics used to manage pain from pericarditis
MEDICATION ADULT DOSE PEDIATRIC DOSE
Ibuprofen 400-600mg PO TID 30-50mg/kg/day PO divided q8 (max 2.4g/day)
ASA 2-4g/day divided PO 3-4x/day 6-=90mg/kg/day divided 2-4x/day
Colchicine (controversial) 0.5mg PO BID; 0.5mg PO daily for <70kg < 5 years old:0.5mg PO daily; >5 years:1-1.5mg/day divided 2-3x/day

Bedrest is often recommended as well.

There is a fair amount of debate regarding the use of corticosteroids for the treatment of pericarditis. If used, corticosteroids should be tapered as quickly as possible to avoid potential side effects. Overall corticosteroid therapy should be restricted to connective tissue disease and autoreactive causes but have been used effectively in refractory and recurrent cases of pericarditis.

Pericardiocentesis in cases of hemodynamically significant pericardial effusion is considered to be both diagnostic and therapeutic. A needle is inserted at the subxiphoid area at a 30-45 degree angle directed toward the left shoulder. The needle is aspirated as it is being advanced. This can be performed with electrocardiogram, echocardiogram and/or fluoroscopy guidance. The pericardial fluid should be sent for testing for cell analysis and likely pathogens.

In cases of constrictive pericarditis, pericardiectomy may be performed by a cardiothoracic surgeon. Although some relief may be achieved, it is possible that symptoms cannot be completely eliminated.

What are the adverse effects associated with each treatment option?

Serious side effects from non-steroidal anti-inflammatory drugs (NSAIDs) are uncommon.

Risks for pericardiocentesis are: cardiac puncture, arrhythmia, infection, pneumopericardium, puncture of nearby anatomic structures.

What are the possible outcomes of pericarditis?

For acute pericarditis from idiopathic and most viral causes, it is generally benign and self-limited, with 60% recovering in 1 week and 80% in 3 weeks. Up to 30% of patients have recurrent pain and friction rub.

What causes this disease and how frequent is it?

Approximately 40-85% of pericarditis cases are idiopathic, most of which are suspected to be viral in etiology. Infectious diseases cause approximately 2/3 of pericarditis cases. Echovirus ad Coxsakie virus are thought be more commonly involved than other viruses. Mycobacterium is most common for bacterial causes with 4-5% occurrence. Fungal etiology is expected more in immunocompromised patients.

1/3 of cases are non-infectious in etiology, with <10% caused by autoimmune disorders, while neoplastic pericarditis occur in 5-7%.

How do these pathogens/genes/exposures cause the disease?

Once exposed to inciting agents or event, an inflammatory reaction occurs at the pericardium with release of chemokines that induces local vasodilatation and increased vascular permeability. This results in protein and fluid leak into the pericardial space. Progression in size may lead to cardiac tamponade physiology, affecting diastolic filling and an increase in systemic and pulmonary pressures resulting in cardiac output compromise and hemodynamic instability.

What complications might you expect from the disease or treatment of the disease?

Constrictive pericarditis is a potential chronic complication that may require pericardiectomy. In rare cases of complications resulting from pericardiocentesis (i.e. cardiac puncture), the patient may require immediate surgical management.

Are additional laboratory studies available; even some that are not widely available?

N/A

How can pericarditis be prevented?

Since approximately 40-85% of pericarditis cases are idiopathic, prevention is difficult in many cases. For non-idiopathic cases, avoidance of predisposing events and timely treatment of illnesses may be of help.

What is the evidence?

Maisch, B. "Guidelines on the Diagnosis and Management of Pericardiac Diseases". European Heart Journal. vol. 25. 2004. pp. 587-610.

(Formal guidelines with evidence based medicine on the diagnosis and management of pericarditis for adults. Useful resources for pediatric caretakers. References with formal guidelines as well as evidence based medicine for pericarditis in the pediatric population are difficult to find.)

Imazio, M, Spodick, DH, Brucato, A, Trinchero, R, Adler, Y. "Controversial issues in the management of pericardial diseases". Circulation. vol. 121. 2010 Feb 23. pp. 916-28.

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