AUTHOR AND EDITOR INFORMATION

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INTRODUCTION

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Background

Both viral and bacterial infections may involve the pericardium (pericarditis), although viral pericarditis is more common than bacterial pericarditis in both children and adults. Awareness of this disease has increased because of the introduction of noninvasive diagnostic techniques, such as echocardiography and CT scanning. Although it is usually benign, the disease can be severe and even lethal, especially in children with immunosuppression. The infection may also involve the myocardium (myopericarditis).

Pathophysiology

The pericardium consists of 2 layers: the visceral pericardium (epicardium) and the parietal pericardium. These layers enclose the pericardial cavity (a potential space) between them. A small volume of serous fluid ( <30 mL in adults) lubricates this cavity. Inflammation of the pericardium secondary to a viral infection increases the volume of serous fluid (pericardial effusion). Studies show that persistent pericarditis triggers an autoimmune reaction to the myopericardial cells.

Pathogenesis of clinical manifestations

The manifestations may be due to the inflammatory process or the effusion or may be related to the underlying cause. Chest pain is related to pericardial inflammation and acute distension.

Significant collection of fluid in the pericardial cavity is a potentially dangerous accompaniment of viral pericarditis. The pericardium does not limit filling of the cardiac chambers either at rest or during exercise. When pericardial effusion occurs, chamber capacity may be reduced. Venous return may be severely limited and, therefore, cardiac output may be severely limited. Capacity of the pericardial space is influenced by its natural stiffness.

Rapid accumulation of fluid is poorly tolerated, whereas slow accumulation may allow large amounts of pericardial fluid to collect without producing symptoms. With increased pressure within the pericardial space, filling pressure in all chambers of the heart is elevated. In advanced stages, right and left atrial mean pressures and right and left ventricular end-diastolic pressures are virtually identical to the intrapericardial pressure. Therefore, the clinical features result from limitation of cardiac output and elevated venous pressures.

Pulsus paradoxus

In healthy individuals, inspiration causes the systolic blood pressure to fall slightly, as a result of the greater volume of blood accommodated by the pulmonary vascular bed. This occurs despite inspiratory increase in venous return to the right heart. In cardiac tamponade, right ventricular filling is maintained at the expense of restricted left ventricular filling, and the systolic blood pressure falls further (>10 mm Hg). This exaggerated fall in systolic blood pressure with inspiration is referred to as pulsus paradoxus. This is an important sign of cardiac tamponade, although, occasionally, severe respiratory distress of any cause (asthma, emphysema, pleural effusion) may give rise to this sign.

Frequency

United States

The frequency is unknown.

International

Various studies indicate that the frequency is 12.5-60%.

Mortality/Morbidity

• Most patients recover with no sequelae.
• Causes of death include cardiac tamponade and systemic involvement by the viral infection or the underlying disease.
• A small percentage of patients develop a chronic form of pericarditis that resembles chronic idiopathic pericarditis.

Sex

Both sexes are affected.

Age

People of all ages are affected.

CLINICAL

Section 3 of 11  

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History

• Fever and chest pain are usual presenting symptoms.
• • Low-to-moderate fever usually occurs but may be absent at the time of presentation.
  • Pain in the chest or left shoulder is often present and is aggravated or relieved by changes in physical position. Patients usually feel better in a sitting position.
  • The pain is usually substernal and accompanied by a sensation of chest constriction.
  • Any movement of the chest, including respiratory motion, increases the pain.
  • Sitting up and leaning forward may reduce the pain. Therefore, a child may refuse to lie down for examination.
  • Palm pressure applied to the sternum markedly increases the pain.
• A hacking cough is occasionally a presenting symptom.
• • The cough varies with position.
  • Sitting up and leaning forward improves the cough.
• Preceding symptoms of viral illness are present in 40-75% of patients and include the following:
• • Fever
  • Runny nose
  • Cough
  • Diarrhea
  • Rash

Physical

• Patients with viral pericarditis generally appear less toxemic than those with bacterial pericarditis. However, some patients appear ill, especially if the accompanying myocarditis is clinically significant.
• Physical signs in the absence of tamponade or clinically significant myocarditis are minimal and may be limited to a pericardial friction rub, which is audible in 80% of patients.
• • Rub sounds are best heard with the diaphragm of the stethoscope. The sounds may seem close to the ear. They are caused by the inflamed pericardial surfaces rubbing against each other. This rubbing sound may be misinterpreted as movement of the stethoscope on the chest surface.
  • Pericardial friction rub may sound like 2 leather surfaces rubbing together or like hair being rubbed between the fingers.
  • Sounds may occur in 3 phases of the cardiac cycle. Ventricular contraction is the first phase and occurs during systole. Ventricular filling and atrial contraction are the second and third phases and occur during diastole.
  • The loudness of a rub may decrease or even disappear with a large effusion because the pericardial surfaces separate.
• Cardiac tamponade refers to sudden accumulation of pericardial fluid sufficient to inhibit cardiac output. Rapidly accumulating effusion may cause cardiovascular collapse and death.
• • Physical signs, such as elevated nonpulsatile jugular venous pulse and tachycardia, mimic those of congestive heart failure (CHF). However, do not misinterpret this event as heart failure because drugs commonly used to treat heart failure may cause vascular collapse in a patient with pericarditis. Likewise, avoid administering diuretics because intravascular depletion can increase cardiac compression.
  • With cardiac tamponade, heart sounds seem distant, peripheral pulses are weak, and BP is low. The pulse is pressure also low. Extremities may be cool and clammy.
  • Pulsus paradoxus may be documented by observing an inspiratory decrease in BP of more than 10 mm Hg.
  • In healthy individuals, inspiration causes systolic BP to decrease slightly as a result of the increased volume of blood accommodated by the pulmonary vascular bed. This occurs despite inspiratory increase in venous return to the right heart.
  • In cardiac tamponade, right ventricular filling is maintained at the expense of restricted left ventricular filling, and systolic BP decreases further (>10 mm Hg). This exaggerated decrease in systolic BP with inspiration is referred to as pulsus paradoxus, an important sign of cardiac tamponade. On occasion, severe respiratory distress of any cause (eg, asthma, emphysema, pleural effusion) may cause this sign.
  • Neck veins may be distended.
  • Hepatomegaly can develop, and the patient may report abdominal pain due to acute hepatic distension.
• A chronic, large pericardial effusion may cause ascites and peripheral edema.
• Pulmonary edema is unusual because pericardial effusion limits the amount of blood that can enter the heart. No rales are heard in the lungs, and the patient does not have dyspnea or tachypnea. With myocarditis, evidence of left-heart failure may be observed.
• Signs and symptoms of viral pericarditis can mimic those of systemic lupus erythematosus.

Causes

Often, a specific etiologic agent is not identified. These cases are often considered viral in origin. Common viral causes include the following:

• Coxsackievirus B (most common cause)
• Echovirus
• Adenovirus
• Influenza virus
• Mumps virus
• Varicella virus
• Epstein-Barr virus
• Cytomegalovirus
• Viral hepatitis B
• Human immunodeficiency virus (HIV)
• Human herpesvirus 6
• Parvovirus B19
• Postimmunization (vaccinia)

DIFFERENTIALS

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Other Problems to be Considered

Effusion, pleurodynia
Erythematosus
Esophageal, foreign body
Lymphedema
Other causes of chest pain
Other collagen vascular disease
Pulmonary causes, pleural
Postpericardiotomy effusion
Radiation pericarditis
Renal failure, chronic, with dialysis
Trauma, hemopericardium, cocaine use
Tuberculous pericarditis
Tumors, invasive and noninvasive
Uremia

WORKUP

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Lab Studies

• The CBC count is usually within the reference range but may show relative lymphocytosis.
• Obtain a blood culture to help exclude bacterial infection.
• Obtain nasopharyngeal aspirate and stool samples for viral isolation, and obtain blood samples for acute and convalescent viral titers.
• Other blood tests (eg, blood gas analysis and electrolyte, BUN, and glucose tests) may be required for acute-stage management.
• Recently, elevated troponin I levels have been reported in young adults with pericarditis, especially with effusion. This may indicate the coexistence of varying degrees of underlying myocarditis.

Imaging Studies

• Chest radiography
• • Radiography demonstrates cardiomegaly, depending on the amount of fluid and presence of underlying myocarditis.
  • A typical saclike appearance of the cardiac silhouette is characteristic of large effusions.
  • Chamber enlargement is not observed.
  • Pulmonary venous congestion is not a feature of pericarditis, although coexistent myocarditis and heart failure may cause such congestion.
• Two-dimensional echocardiography
• • Echocardiography is the definitive test to demonstrate pericardial fluid and to establish the diagnosis.
  • The thickness of the pericardium and the amount of fluid can be quantified, and the effect of fluid on cardiac hemodynamics can be assessed.
• CT and MRI
• • CT scan of the chest can help confirm the thickness of the pericardial effusion, assess pericardial thickening, and also help to identify other abnormalities, such as mediastinal lymph nodes, that may point to an etiology.
  • MRI reveals similar findings.

Other Tests

• Electrocardiography
• • Generalized elevation of the ST segment in leads with an upright T wave is the typical pattern of pericarditis, though this finding may not be apparent.
  • Low-voltage QRS complexes may occur in the presence of a large effusion. However, a recently published adult study showed greater frequency of low P-wave and T-wave voltages than low QRS voltages

Procedures

• Pericardiocentesis: This procedure is required for all patients who have clinical evidence of cardiac tamponade or suspected bacterial pericarditis and for some patients with immunocompromise. Pericardiocentesis is also used as a diagnostic test in patients with a pericardial effusion of unknown cause. Caution: The volume of fluid present should be sufficient to allow for the removal of a reasonable portion for diagnostic purposes.
• • Place the child in a half-sitting position (roughly 45°), with sedation or anesthesia as needed. If anesthesia is used, avoid agents that precipitously decrease systemic pressure because circulatory collapse can ensue.
  • Echocardiography should be available to monitor the position of the needle.
  • Insert a beveled, sharp needle beneath the xiphoid, angling up and left toward the left shoulder. Sometimes, a pop is felt as the needle passes into the pericardium.
  • Attempt to withdraw fluid with each advance of the needle. If fluid is obtained, remove enough to alleviate the tamponade. A small amount (as little as 20 mL in an adult) provides considerable benefit.
  • If the fluid is grossly bloody, consider the possibility that the needle is in a cardiac chamber. Placement of a few drops of the fluid on a towel sometimes immediately proves whether the problem is bloodstained fluid and not pure blood. If the result is debatable, centrifuge the fluid because it may have a hematocrit lower than that of blood.
  • After the fluid obtained is confirmed to be from the pericardium, drain all easily removable fluid. Patients may report relief from symptoms at this point.
  • The decision whether to leave a drain in the pericardium depends on the probable diagnosis. If evidence of bacterial infection is found, pass a guidewire into the pericardium and confirm its position with echocardiography or fluoroscopy. Then, pass a catheter over the guidewire into the pericardium to serve as a drain. Leave the drain in place.
  • Definitive therapy can begin when a precise diagnosis is established, often only after detailed examination of the fluid is completed.
  • Attempt to identify the virus using antigen detection techniques, such as immunohistochemistry and indirect immunofluorescence assay (IFA), amplification of viral genomes by nested reverse transcription polymerase chain reaction (RT-PCR), and sequence analysis
• Pericardioscopy and epicardial biopsy: This new diagnostic procedure seldom applies to an episode of viral pericarditis. Instead, it is used to macroscopically visualize alterations of both the epicardium and the pericardium. The macropathology of the epicarditis and pericarditis can be observed in vivo.
• • The prerequisite is documentation of a large pericardial effusion (>150 mL by the cubic model) with an echocardiographically documented effusion of type C. This procedure requires a separation of at least 5 mm between the epicardial and pericardial layers in diastole at the anterior side of the heart when echocardiographic imaging is performed from the subxiphoidal or third intercostal space.
  • After the pericardial effusion is punctured, introduce a 9-ft sheath using a guidewire under echocardiographic or fluoroscopic control.
  • Aspirate the fluid and infuse 100-150 mL of saline warmed to body temperature into the pericardial sac.
  • Use a flexible 8-ft fiberglass instrument (eg, Vantec, Baxter, Storz) and a rigid 110°, 8-ft endoscope (Storz) to visualize the pericardium and epicardium and to document the findings on videotape.
  • The macropathology is endoscopically inspected. Fibrinous strands or increased vascular injection can be observed in viral, autoimmune, or idiopathic pericarditis or in idiopathic perimyocarditis. In the last 3 forms of pericardial effusion, only inflammatory cells are observed when the pericardial fluid is analyzed.
• Cardiac catheterization: This is not indicated for diagnosis of viral pericarditis.

Histologic Findings

Pericardial fluid is either serous or serosanguineous, with a predominance of lymphocytes, although neutrophils may also be present. Pericardial biopsy findings may reveal a viral etiology using antigen detection techniques.

TREATMENT

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Medical Care

Management is conservative (expectant) and symptom specific.

• Bed rest and the use of anti-inflammatory agents are mainstays of initial therapy.
• Aggressive pain control may be necessary in some patients, although most cases respond to salicylates or nonsteroidal anti-inflammatory drugs (NSAIDs).
• Corticosteroid therapy is rarely indicated. Consider this option only when NSAIDs are unsuccessful and a bacterial etiology has clearly been excluded. While corticosteroid therapy might reduce symptoms dramatically, no convincing evidence of any long-term benefits has been reported. Moreover, corticosteroid therapy may increase the risk of recurrence.
• A recently published prospective randomized trial reported a significant reduction in the rate of recurrence with colchicine therapy in patients with a first episode of acute pericarditis. However, this study involved mainly adult patients with a wide spectrum of etiologies, and diarrhea necessitated discontinuation of colchicine in a number of patients.
• Resolution of effusion may occur within several days to weeks after initiating anti-inflammatory drugs; However, patients should be closely observed for the development of pericardial tamponade as a part of their initial care.
• Pericardiocentesis is indicated when the etiology is in doubt and is essential in suspected tamponade.
• Occasionally, intravenous immunoglobulin has been used to treat patients who develop chronic pericarditis with satisfactory results.

Surgical Care

• Place a pericardial drainage catheter.
• Pericardiectomy, the surgical creation of a pericardial window needed in refractory cases to facilitate open drainage, is rarely required to manage chronic recurrent cases. Recently, video-assisted thoracoscopic window has been used in adult patients for diagnosis and management of pericardial effusions.

Consultations

• Pediatric cardiologist
• Radiologist
• Family physician
• Psychologist
• School teacher
• Specialist nurse
• Pharmacist
• Dietitian

Diet

Viral pericarditis requires no special diet.

Activity

Reduce the patient's activity to the level he or she can tolerate.

MEDICATION

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Bed rest and use of anti-inflammatory agents are the mainstays of initial therapy. Aggressive pain control may be necessary in some patients; however, most cases respond to salicylates or NSAIDs. Although corticosteroid therapy is rarely indicated, consider this course when NSAIDs are unsuccessful and when a bacterial etiology is clearly excluded.

Corticosteroids may dramatically reduce symptoms, but no convincing evidence suggests any long-term benefit. Anti-inflammatory therapy (eg, with aspirin, indomethacin) may continue for several months. After therapy is discontinued, 15-30% of patients may have a relapse. The optimal method for prevention is not fully established, but accepted modalities include NSAIDs, corticosteroids, immunosuppressive agents, and pericardiectomy. Colchicine has also been used in some patients, with a good response.

Drug Category: NSAIDs

These drugs have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action inhibits cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may also exist, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.

Drug Category: Corticosteroids

These drugs have anti-inflammatory and immunosuppressive properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

FOLLOW-UP

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Further Inpatient Care

• Reevaluate recurrent cases
• Pericardial biopsy
• Pericardiectomy

Further Outpatient Care

• Continue anti-inflammatory therapy, such as aspirin or indomethacin, for at least several months to monitor the patient's progress.

Complications

• Severe tamponade may cause cardiac arrest due to electromechanical dissociation.
• • Less severe cases may cause tachycardia, tachypnea, decreased arterial BP, increased central venous pressure, and paradoxical pulse. Patients report dyspnea and chest pain.
  • Cardiac tamponade may develop from any cause of acute fluid accumulation in the pericardial sac.
  • Therapy for cardiac tamponade consists of removing the pericardial fluid by means of pericardiocentesis, pericardiotomy, or pericardiectomy.
  • Caution: Do not confuse tamponade with CHF. Medications used to treat heart failure (eg, digoxin) may slow the heart rate. Because tachycardia represents the only effective compensatory mechanism available to the patient for maintaining cardiac output, slowing the rate may cause acute cardiovascular collapse. Administration of diuretics or afterload-reducing agents can also lead to cardiovascular collapse. Administration of diuretics, certain anesthetic agents that relax the peripheral vascular bed, or afterload-reducing agents can also lead to cardiovascular collapse.
• After therapy is discontinued, 15-30% of patients have a relapse.
• • The optimal method for prevention is not fully established.
  • Accepted modalities include NSAIDs, corticosteroids, immunosuppressive agents, and pericardiectomy.
  • Colchicine has also been tried in some patients, with a good response.

Prognosis

• Almost all patients recover completely if no serious acute complication occurs.
• Constrictive pericarditis rarely occurs after viral pericarditis.

Patient Education

• Educate the patient on the likelihood of recurrence and about the need to continue medications and for follow-up, as appropriate.
• For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Chest Pain.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Failure to diagnose bacterial pericarditis
• Failure to diagnose tamponade
• Failure to suspect effusion in chest infections or other systemic diseases
• Failure to attribute symptoms to tamponade, especially in patients with underlying malignancy or in posttransplant patients
• Failure to follow up for recurrence
• Failure to distinguish between tamponade and CHF

Special Concerns

• Early identification of bacterial effusion is important because it requires antibiotic therapy and open drainage.
• Any underlying collagen vascular disease must be recognized.
• Discontinue aspirin administration to patients who have had contact with chicken pox.

MULTIMEDIA

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Media file 1:  Two-dimensional echocardiogram shows a large pericardial effusion.
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Media file 2:  M-mode echocardiogram shows moderate pericardial effusion.
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Media file 3:  Plain chest radiograph in a 2-year-old boy with viral pericarditis and massive pericardial effusion.
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Media type:  X-RAY

REFERENCES

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Article Last Updated: Dec 7, 2006