Bacterial, fungal, and viral infections may involve the pericardium (pericarditis), although viral pericarditis is more common than bacterial pericarditis in both children and adults.[1] Awareness of this disease has increased because of the introduction of noninvasive diagnostic techniques, such as echocardiography, computed tomography (CT) scanning, and cardiac magnetic resonance imaging (CMRI).[2] The disease can be severe and even lethal, especially in children with immunosuppression. The infection may also involve the myocardium (myopericarditis).[2, 3, 4]
The infection involves the pericardium and leads to accumulation of pericardial effusion that, if untreated, can lead rapidly to hemodynamic collapse, tamponade, and death. The fluid is generally purulent but can be serosanguineous; however, the causative agent can usually be identified from the pericardial fluid by culture or more sensitive tests (eg, polymerase chain reaction (PCR) or from samples of pericardial biopsy in cases that require open drainage of the fluid.
Workup of the patient with infective pericarditis may include laboratory studies, imaging studies (CT scanning and CMRI), electrocardiography (ECG), pericardioscopy, and pericardial biopsy. (See Workup.)
Management of pediatric infective pericarditis is influenced by the cause of the pericarditis and the rapidity of pericardial fluid accumulation. It may involve supportive care, pain control, and antibiotic therapy if necessary, as well as pericardiocentesis (indicated in symptomatic patients or when the etiology is in doubt and essential in suspected tamponade), pericardial drainage, or pericardiectomy as required. (See Treatment.)
See Infective Endocarditis for more detailed information on this topic.
The pericardium consists of two layers: the visceral pericardium (epicardium) and the parietal pericardium. These layers enclose the pericardial cavity (a potential space) between them. In healthy adults, this cavity contains approximately 20 mL of serous fluid, which has the appearance of a plasma ultrafiltrate and serves to lubricate the space. The pericardium provides a membrane barrier that protects the heart from infection, limits acute myocardial distention, decreases friction, and modulates ventricular interdependence.
Inflammation of the pericardium secondary to an infection leads to an increase in permeability to proteins and inflammatory cells, and fluid accumulates between the visceral and parietal layers (pericardial effusion). Because the pericardium has a limited ability to stretch acutely, rapid accumulation of fluid leads to increased intrapericardial pressure and hemodynamic compromise. The capacity of the pericardial space is influenced by its natural stiffness, and infection is known to increase this stiffness, contributing to the symptomatology. Studies show that persistent pericarditis triggers an autoimmune reaction to the myopericardial cells. The clinical 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 distention.
Significant collection of fluid in the pericardial cavity is a potentially dangerous accompaniment of infective pericarditis. The normal 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. Rapid accumulation of fluid is poorly tolerated, whereas slow accumulation may allow large amounts of pericardial fluid to collect without producing symptoms. Tamponade occurs when pericardial fluid accumulates rapidly enough or in sufficient volume to impair diastolic filling. During tamponade, all four cardiac chambers compete for space within the pericardium, producing increased systemic venous, pulmonary venous, and atrial pressures. Therefore, the clinical features result from limitation of cardiac output and elevated venous pressures.
Initially, an increased ejection fraction and tachycardia maintain cardiac output. When these mechanisms fail, systemic vascular resistance rises to maintain the blood pressure (BP), and the pulse pressure narrows. Any further increase in pericardial volume compromises ventricular filling, producing systemic hypotension and cardiovascular collapse. Volume and rapidity of fluid accumulation both determine whether a pericardial effusion produces tamponade.
In healthy individuals, inspiration causes the systolic BP (SBP) 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 SBP falls further (>10 mm Hg). This exaggerated fall in SBP with inspiration is referred to as pulsus paradoxus. It is an important sign of cardiac tamponade; however, occasionally, severe respiratory distress of any cause (asthma, emphysema, pleural effusion) may give rise to this sign.
Infectious pericarditis may be caused by viruses, bacteria, and fungi, and parasites.[5]
Viral pericarditis
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
Chicken Pox (Varicella virus)
Epstein-Barr virus
[6]
Cytomegalovirus
[7]
Viral hepatitis B
Human immunodeficiency virus (HIV)
Human herpesvirus 6
Parvovirus B19
Postimmunization (vaccinia)
Hepatitis C virus[8]
Bacterial pericarditis
Primary infection of the pericardium is rare. Bacterial pericarditis most commonly occurs as a direct extension of an infection from an adjacent pneumonia or empyema, and rarely as a complication of infective endocarditis.[9, 10] Alternatively, a distant infection can hematogenously seed the pericardium. Patients recovering from thoracic, cardiac, or esophageal surgery are at risk for purulent pericarditis. Purulent pericarditis has been reported in patients recovering from traumatic injury.
The organisms that most commonly cause purulent pericarditis include the following:
S aureus is the most common cause of bacterial pericarditis in children, causing approximately 40%-80% of cases. Within the first 3 months after cardiac surgery, S aureus is the most common cause of purulent pericarditis.
S aureus pericarditis occurs concomitantly in patients who have pneumonia with empyema and less often in patients with acute osteomyelitis or soft tissue abscess. Rarely, pericarditis is associated with S aureus endocarditis. Necrotizing infection and exotoxin production lead to increased incidence of shock and higher mortality risk.
H influenzae is the second most common organism listed in most reported pediatric case series of purulent pericarditis, although comprehensive data since the introduction of routine immunization is lacking. Infection of the upper or lower respiratory tract frequently precedes pericarditis caused by H influenzae. Purulent pericarditis may occur with H influenzae meningitis. H influenzae produces very thick fibrinopurulent exudate.
N meningitidis pericarditis occurs in approximately 5% of young adults with meningococcemia. The clinical course is often milder than with other causes of purulent pericarditis. Pericardial effusion can be detected at the onset of the illness or later in the course of the infection. Late-onset effusions have a purulent appearance but are usually sterile. Whether this late appearing effusion represents an infection, hypersensitivity to an antibiotic, or an immunologic response to the primary infection is unclear.
S pneumoniae was the leading cause of purulent pericarditis at one time, but it has become a much less common cause, perhaps because of widespread use of antibiotics. Like other causes, many cases are associated with respiratory infections but also with S pneumoniae with hemolytic uremic syndrome.
Other unusual organisms, such as gram-negative enteric bacilli and anaerobes or fungi, are rare but should be considered in patients who are immunocompromised, and in neonates.[12]
Aspergillus pericarditis arises as a result of pulmonary infection in patients who are immunocompromised. Patients have a very poor prognosis. Therapy includes long-term amphotericin B or itraconazole. Successful therapy relies on recovery of adequate immune function.
Mycoplasma pneumoniae pericarditis is associated with pulmonary disease. The effusion responds to erythromycin.
The frequency of viral pericarditis is not known; various studies have placed it between 12.5% and 60%. People of all ages are affected, and both sexes are affected. A recent report on acute pericarditis has indicated that the majority of cases (68%) are idiopathic; however, these could very well represent viral or postviral pericarditis.[13] Bacterial pericarditis is rare. Its incidence appears to be decreasing, perhaps because of earlier treatment of primary infections and the availability of H influenzae immunization, as well as the polyvalent pneumococcal vaccine. Bacterial pericarditis may occur more frequently in developing nations than in the United States. This increased incidence has been associated with delay in diagnosis and treatment of serious bacterial infections, malnutrition, and overcrowding.
Most cases of purulent pericarditis occur in children younger than 4 years. Infants may not present with typical or classic features. For example, in one study, no patient younger than 18 months had a friction rub. In infants, almost all cases of pericarditis have a bacterial etiology. Purulent pericarditis affects both sexes nearly equally.
Almost all patients with viral pericarditis without concomitant myocarditis recover completely if no serious acute complication occurs. 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. Constrictive pericarditis rarely occurs after viral pericarditis.
Bacterial pericarditis, on the other hand, is a life-threatening infection with a high mortality rate unless timely therapy with antibiotics and pericardial drainage is instituted. Without treatment, the mortality rate of bacterial pericarditis approaches 100%.[14] Even with optimal therapy with antibiotics and pericardial drainage, mortality rates of 2-20% have been reported in modern case series. Treatment without early and adequate pericardial drainage significantly increases the risk of death.
Other risk factors for increased mortality include tamponade or myocardial involvement, delays in diagnosis or delays in institution of therapy, infection with S aureus, and malnutrition.
Patients who survive the acute bacterial infection generally do well, without long-term sequelae. Infrequently, constrictive pericarditis may develop as a sequel of purulent pericarditis. Signs can develop as early as 15 days after onset of the acute illness. Pericardiectomy usually resolves the symptoms.
Complications
Cardiac tamponade may develop from any cause of acute fluid accumulation in the pericardial sac. Severe tamponade may cause cardiac arrest due to electromechanical dissociation. Less severe cases may cause tachycardia, tachypnea, decreased arterial blood pressure (BP), increased central venous pressure, and paradoxical pulse. Patients report dyspnea and chest pain.
Acutely, serial electrocardiograms (ECGs) may indicate the presence of occult arrhythmia, suggesting additional myocardial involvement.
Left ventricular pseudoaneurysm, which may require surgical resection may develop during the acute phase of the illness.[15, 16]
Rarely, purulent pericarditis may be associated with mycotic aneurysms. A case report has been published regarding an unusual combination of purulent pericarditis and false aneurysm of the ascending aorta.[17]
Constrictive pericarditis is a rare complication. Acute constriction has been reported as early as 8 days but generally develops within weeks of diagnosis. Patient symptoms include increased systemic venous pressure, weight gain, hepatomegaly, dyspnea, and decreased urine output. Presence of continued heart failure without a large cardiac silhouette suggests constriction.
See Neurological Sequelae of Infective Endocarditis for more detailed information on this topic.
Educate the patient on the likelihood of recurrence and about the need to continue medications and for follow-up, as appropriate. In particular, the following items should be discussed with patients and/or their families in children with bacterial pericarditis:
Bacterial pericarditis is a life-threatening disease.
Proper treatment includes a prolonged course of antibiotics and drainage of the pericardium.
If the patient survives, prognosis is good.
If symptoms suggestive of congestive heart failure develop after treatment (eg, dyspnea, fatigue, weight gain), the patient should seek evaluation for possible constrictive pericarditis.
For patient education information, see the Heart Health Center, as well as Chest Pain.
Fever and chest pain are usual presenting symptoms. Low to moderate fever usually occurs but may be absent at the time of presentation. Patients generally appear less toxic than those with bacterial pericarditis but may shows signs of shock if significant myocarditis is present.
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
Bacterial pericarditis
Patients are acutely ill and exhibit symptoms of sepsis. Acute purulent pericarditis in an infant is a medical emergency. Rapid evaluation, diagnosis, and treatment are essential.
Symptoms are often nonspecific and include fever, respiratory distress, and tachycardia out of proportion to the degree of fever. Children may complain of abdominal discomfort. Precordial chest pain is not a frequent symptom, especially in young children. The pain, if present, may be sharp or dull. Supine position, chest wall motion, or coughing may worsen the pain. Sitting forward may relieve the pain.
Most patients have preceding or concurrent infection that is the source of pericarditis. These infections include the following:
Pneumonia
Meningitis
Acute osteomyelitis
Acute arthritis
Soft tissue infections
Neonatal sepsis
Pericarditis may rarely complicate neonatal sepsis.
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. Sounds may occur in three 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.
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 two leather surfaces rubbing together or like hair being rubbed between the fingers. With a large effusion, the loudness of a rub may decrease or even disappear because the pericardial surfaces separate.
Cardiac tamponade is seen in infants but can also occur in older children.[18] Cardiac tamponade is a unique form of obstructive shock with symptoms of venous congestion and low cardiac output. Signs of low cardiac output include weak peripheral pulses, cool and clammy extremities, low blood pressure, and tachycardia. With cardiac tamponade, heart sounds seem distant. The pulse pressure is also narrow. Signs of venous congestion include distend neck veins, pulmonary edema, or hepatomegaly. The patient may report abdominal pain due to acute hepatic distension.
Physical signs of venous congestion, such as elevated nonpulsatile jugular venous pulse and peripheral or pulmonary edema, 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 cardiac tamponade. Likewise, avoid administering diuretics because intravascular depletion can increase cardiac compression.
Pulsus paradoxus may be documented by observing an inspiratory decrease in blood pressure (BP) of more than 10 mm Hg. In healthy individuals, inspiration causes systolic BP (SBP) 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 SBP decreases further (>10 mm Hg). This exaggerated decrease in SBP 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.
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.
Bacterial pericarditis
Infants with bacterial pericarditis are generally very ill and can present with signs of severe sepsis and shock. A high index of suspicion is required. Purulent pericarditis should be suspected in any infant who appears to have sepsis and has an abnormal cardiovascular examination. Tachypnea and tachycardia out of proportion to fever is characteristic of both purulent pericarditis and acute myocarditis.
Classic cardiac findings of pericarditis include muffled heart sounds and a friction rub. A friction rub can occur in any combination of systole, mid diastole, and late diastole. It may vary with patient position or the respiratory cycle and may be transient. A friction rub is unlikely in the presence of a large pericardial effusion or in an infant with purulent pericarditis. Diastolic filling sounds may be heard.
Signs of venous congestion may be present, including hepatomegaly and jugular venous distention. Jugular veins are difficult to assess in infants.
Tamponade can present rapidly with hypotension, soft heart tones of poor quality, signs of venous congestion, and signs of low cardiac output, indicating acute cardiac decompensation. Alternatively, tamponade can develop more insidiously, presenting a picture of right heart failure. Signs of tamponade include dyspnea, tachycardia, narrow pulse pressure, pulsus paradoxus, and venous congestion. Any significant pericardial constriction produces pulsus paradoxus. Other causes of pulsus paradoxus include hypovolemia and either a large or small airway obstruction like epiglottitis or asthma.
Workup of the patient with infective pericarditis may include laboratory studies, imaging studies, electrocardiography (ECG), pericardioscopy, and epicardial biopsy. Echocardiography and cardiac magnetic resonance imaging have key roles in the diagnosis, management, and prognostic outlook of pediatric inflammatory and/or infectious cardiac conditions.[2]
See Infective Endocarditis for more detailed information on this topic.
A complete blood cell (CBC) count is performed. In cases of viral pericarditis, the CBC is usually within the reference range but may show relative lymphocytosis. In cases of bacterial pericarditis, the CBC usually reveals leukocytosis with predominance of immature polymorphonuclear leukocytes. Lymphocytosis may also reflect an idiopathic etiology.
Nasopharyngeal aspirate and stool samples should be obtained for viral isolation, and blood samples should be obtained for acute and convalescent viral titers.
Blood culture should be performed to help confirm or exclude bacterial infection. Cultures are positive in more than half of patients with bacterial pericarditis. Erythrocyte sedimentation rate (ESR) and other acute phase reactant levels are elevated in purulent pericarditis.
Other blood tests (eg, blood gas analysis and electrolyte, blood urea nitrogen, and glucose tests) may be required for acute-stage management.
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; however, it may not be prognostic.[19]
Laboratory examination of pericardial fluid should include cell count, Gram stain, and culture. Cytologic examination should be considered for patients with suspected malignancy or systemic lupus erythematosus. Polymerase chain reaction (PCR) tests for virus and Mycoplasma allows for more rapid identification in some cases.
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 (see the image below).
View Image
Plain chest radiograph in a 2-year-old boy with viral pericarditis and massive pericardial effusion.
Chamber enlargement is not observed.
A rapidly enlarging cardiac silhouette with a water-bottle appearance without accompanying increase in pulmonary vascular markings strongly suggests a pericardial effusion (see image below).
View Image
Left: Chest radiograph in a patient with bacterial pericarditis reveals cardiomegaly and left lower lobe infiltrate with marked increase in pulmonary ....
Consider purulent pericarditis or myocarditis in an infant or child with sepsis and cardiomegaly.
Echocardiography is the imaging modality of choice for rapid, accurate identification of a pericardial effusion and determination of its size and distribution (see the image below).[2, 20] The thickness of the pericardium and the amount of fluid can be quantified, and the effect of fluid on cardiac hemodynamics can be assessed. However, limitations of this imaging modality include a limited ability to characterize myocardial tissue anomalies (eg, inflammation, fibrosis), and limited quantitative evaluation of cardiac output and valve function.[2]
View Image
Two-dimensional echocardiograph shows a large pericardial effusion.
On two-dimensional echocardiography, pericardial fluid appears as an “echo-free” space. With small effusions, fluid first appears posteriorly in the dependent portion of the pericardial sack. With larger effusions, fluid is found both anterior and posterior to the heart. Echogenic material may be observed in the pericardial fluid and may represent adhesions, clots, or fibrinous material.
Multiple echocardiographic indicators of cardiac tamponade are recognized, but none is completely sensitive or specific. Echocardiography findings must be considered in relation to the clinical picture when making the diagnosis of tamponade.
Common criteria of tamponade on echocardiography include right atrium collapse at end diastole that continues into systole (see the image below), right ventricle compression during diastole (especially with expiration), and marked respiratory variation in transvalvular flow velocities by Doppler echocardiogram. Reversal of systemic venous return flow can occur during diastole.
View Image
Apical four-chamber view from a patient with bacterial pericarditis. The large pericardial effusion (EF) appears as an echo clear space in this view s....
Echocardiography is useful in guiding pericardiocentesis. (See Treatment.) Visualizing the tip of the needle is helpful because echographic artifacts arising from the shaft of the needle may mislead the operator to the actual location of the needle tip. If needle position is uncertain, 5 mL (or less) of agitated saline may be injected for a contrast echocardiography.
Echocardiography is also helpful to assess for coexisting congenital heart defects and vegetations from infective endocarditis.[9, 12]
Computed tomography (CT) scanning 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.
Cardiac magnetic resonance imaging (CMRI) reveals similar findings and helps to assess cardiac function (especially right ventricular function) in the presence of coexisting myocarditis. Thus, CMRI is superior to echocardiography for characterizing myocardial tissue anomalies (eg, inflammation, fibrosis) quantitatively evaluating cardiac output and valve function.[2]
Most patients exhibit at least one electrocardiographic (ECG) abnormality. ECG abnormalities depend on the amount of effusion and the presence of superficial myocardial injury. Normal ECG findings do not exclude the diagnosis of acute pericarditis. Typical findings include ST-segment elevation and low-voltage QRS complexes.
Generalized elevation of the ST segment in leads with an upright T wave is the typical pattern of pericarditis, although this finding may not be apparent (see the image below).
View Image
A 12-lead ECG from a patient with bacterial pericarditis demonstrating marked ST elevation in multiple leads.
Pericardial fluid can produce low-voltage QRS complexes because of a dampening effect of the fluid between the chest wall and myocardium. Such complexes may occur in the presence of a large effusion. However, an adult study showed greater frequency of low P-wave and T-wave voltages than low QRS voltages.[21]
Similarly, differentiation of ST-segment elevation on ECG from acute pericarditis could prove difficult from the normal variant early repolarization and early repolarization of left ventricular hypertrophy,[22] but clinical features could help.
Electrical alternans (ie, changes in the P, QRS, and T wave voltages) is very specific, although not sensitive, for large pericardial effusions. Electrical alternans results from the heart swinging in a large effusion (see the image below).
View Image
This ECG shows markedly decreased QRS voltage and electrical alternans (especially in lead V1)
Four classic stages of ECG changes are described in acute pericarditis; however, many patients do not exhibit all four stages. The stages are as follows:
ST-segment elevation and PR segment may be depressed.
ST segment is still elevated, but the PR segment returns to baseline with decreased T-wave amplitude. PR segment is depressed.
ST segment returns to normal with T-wave inversion (may be incomplete in some cases).
ECG normalization occurs. T-wave changes may persist and do not necessarily indicate active disease.
Pericardiocentesis 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. The volume of fluid present should be sufficient to allow for the removal of a reasonable portion for diagnostic purposes.
However, note the procedure is associated with morbidity and therefore should be performed by or supervised by an experienced physician. Pericardiocentesis is safest when performed in a controlled environment, such as the catheterization laboratory or intensive care unit (ICU). Electrocardiography (ECG), blood pressure, and oximetry monitoring is necessary. (See Pericardiocentesis for a description of the procedure.)
Laboratory examination of pericardial fluid should include a cell count, Gram stain, and culture. Cytologic examination should be considered for patients with suspected malignancy or systemic lupus erythematosus. Polymerase chain reaction (PCR) tests for virus and Mycoplasma allows for more rapid identification in some cases. Lactate dehydrogenase (LDH) and protein levels are often sent but are generally elevated in most types of pericarditis and do not add much diagnostic utility. Many times, bloody fluid is obtained and raises a concern for intracardiac puncture. Several findings can help exclude cardiac puncture, including echocardiographic images, a hematocrit value of the effusion lower than peripheral blood, and failure of the fluid to clot.
Pericardioscopy with epicardial biopsy is rarely required by may yield diagnostic information in tuberculous 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. 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.
Fibrinous strands or increased vascular injection can be observed in viral, autoimmune, or idiopathic pericarditis or in idiopathic perimyocarditis. In the last three forms of pericardial effusion, only inflammatory cells are observed when the pericardial fluid is analyzed. Pericardial biopsy findings may reveal a viral etiology using antigen detection techniques. Definitive diagnosis of purulent pericarditis requires direct examination of pericardial fluid.
Management of pediatric infective pericarditis is influenced by the cause of the pericarditis. It may involve supportive care, pain control, and antibiotic therapy if necessary, as well as pericardiocentesis (indicated when the etiology is in doubt and essential in suspected tamponade), open pericardial drainage, or pericardiectomy as required.
2015 European Society of Cardiology (ESC) recommendations
The 2015 ESC update of their 2004 guidelines on the diagnosis and management of pericardial diseases recommends the following for treatment of pediatric acute and recurrent pericarditis (all level C evidence)[23] :
Administer high-dose NSAIDs as first-line therapy for acute pericarditis until complete symptom resolution (class I).
Consider colchicine as an adjunct to anti-inflammatory therapy for acute recurrent pericarditis (< 5 years: 0.5 mg/day; >5 years, 1.0-1.5 mg/day in two or three divided doses) (class IIa).
Consider anti-interleukin 1 agents in the setting of recurrent pericarditis, particularly in corticosteroid-dependent children (class IIb).
Avoid aspirin (because of the associated risk of Reye syndrome and hepatotoxicity), as well as corticosteroids (due to severity of side effects in this population) unless there are specific indications (eg, autoimmune disease) (both class III).
See Infective Endocarditis and Antibiotic Prophylactic Regimens for Endocarditis for more information on these topics.
In general, management of viral pericarditis is conservative (expectant) and symptom specific. Bed rest and the use of anti-inflammatory agents (eg, nonsteroidal anti-inflammatory drugs [NSAIDs]) are mainstays of initial therapy. Aggressive pain control may be necessary in some patients, although most cases respond to salicylates or NSAIDs.
Corticosteroid therapy is rarely indicated. Consider this option only when NSAIDs are unsuccessful and a bacterial or fungal etiology has clearly been excluded by culture of the pericardial fluid. Although corticosteroid therapy might dramatically reduce symptoms, no convincing evidence of any long-term benefits has been reported. Moreover, corticosteroid therapy may increase the risk of recurrence.
A prospective randomized trial reported a significant reduction in the rate of recurrence with colchicine therapy in patients with a first episode of acute pericarditis.[24] However, this study involved mainly adult patients with a wide spectrum of etiologies, and diarrhea necessitated discontinuation of colchicine in numerous 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.
Therapy for cardiac tamponade consists of removing the pericardial fluid by means of pericardiocentesis, pericardiotomy, or pericardiectomy. A pericardial drainage catheter may be indicated. (See the Pericardiocentesis and Surgical Pericardial Drainage sections.)
Caution: Do not confuse tamponade with congestive heart fialure (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, certain anesthetic agents, or afterload-reducing agents can also lead to cardiovascular collapse.
Occasionally, intravenous immunoglobulin has been used to treat patients who develop chronic pericarditis with satisfactory results.
Viral pericarditis requires no special diet. The patient’s activity should be reduced to the level that he or she can tolerate.
Bacterial pericarditis
Proper treatment of life-threatening illness requires proper antimicrobial therapy, pericardial decompression and drainage, and intensive supportive care.
Until a definitive agent is identified, empiric therapy includes antibiotics to treat both S aureus and gram-negative bacilli. Initial antibiotics should include a combination of penicillinase-resistant penicillin and third-generation cephalosporin. In areas of high antibiotic resistance, consider the use of vancomycin and a third-generation cephalosporin. Include an aminoglycoside if the patient is postoperative from cardiac surgery, is immunocompromised, or has a genitourinary coinfection. Relatively recently, tigecycline has been used to treat methicillin-resistant S aureus pericarditis, where vancomycin is ineffective.[9]
Duration of antibiotic therapy is empiric but generally continues for 3-4 weeks with an antibiotic specific to the organism isolated.
Although needle pericardiocentesis may be life saving in tamponade and may confirm the diagnosis, it rarely provides complete and long-lasting resolution of the effusion. Drainage with a percutaneous pigtail catheter or open surgical drainage is required in most cases of purulent pericarditis to adequately drain the space. (See the Pericardiocentesis and Surgical Pericardial Drainage, sections.)
Almost all patients require intensive supportive care. In patients with tamponade, supportive therapies are of little or no benefit until emergent pericardial drainage is performed.
In patients with tamponade prior to drainage, plasma volume expansion is helpful in maintaining cardiac output. Drugs that depress the heart rate, produce vasodilatation, or decrease intravascular volume are contraindicated because they further compromise cardiac output. Do not administer digoxin to an infant with purulent pericarditis who shows signs of congestive heart failure. Although systolic function of the heart may not be depressed, inotropic agents may be required to treat hypotension that persists after pericardial drainage.
Application of positive pressure mechanical ventilation and positive end-expiratory pressure (PEEP) must be performed carefully because the increased intrathoracic pressure can lead to lethal falls in ventricular preload and worsened shock and /or pulseless electrical activity.
Bacterial pericarditis is often preceded by other severe bacterial infections such as pneumonia with empyema. Proper treatment of those infections prevents some cases of pericarditis. Immunization against H influenzae has led to dramatic decreases in the incidence of invasive H influenzae disease, including pericarditis.
Pericardiocentesis is required for all patients who have clinical evidence of cardiac tamponade or suspected bacterial pericarditis and for some immunocompromised patients. Pericardiocentesis is also used as a diagnostic test in patients with a pericardial effusion of unknown cause. The volume of fluid present should be sufficient to allow for the removal of a reasonable portion for diagnostic purposes.
The procedure is associated with morbidity and should be performed or supervised by an experienced physician. Pericardiocentesis is safest when performed in a controlled environment, such as the catheterization laboratory or intensive care unit (ICU). Electrocardiography (ECG), blood pressure, and oximetry monitoring is necessary.
Place the child in a half-sitting position (roughly 45°), with sedation or anesthesia as needed. Sedation is desirable unless the patient is unconscious or extremely unstable. Pericardiocentesis in a struggling patient is dangerous. If anesthesia is used, avoid agents that precipitously decrease systemic vascular resistance because circulatory collapse and cardiac arrest can ensue.
Echocardiography should be available to monitor the position of the needle. Some operators prefer to use ECG monitoring of needle advancement by clipping the V1 lead to the needle. This technique is cumbersome and is not often used. If visualization is desired, echocardiography or fluoroscopy guidance is preferred.
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.
During pericardiocentesis, bloody fluid is often obtained that may be blood from a myocardial puncture or bloody pericardial fluid. 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.
Potential complications of pericardiocentesis include arrhythmias, laceration of coronary arteries with subsequent hemopericardium and tamponade, pneumothorax, and myocardial perforation. Avoid repeated attempts at needle pericardiocentesis because they are associated with increased morbidity rates.
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 using a modified Seldinger technique over the guidewire into the pericardium to serve as a drain. The use of a pigtail catheter reduces the risk of dislodgment and myocardial puncture. Additionally, the pigtail catheter may be left in place to provide continuous and potentially definitive pericardial drainage (see image below).
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Left: Chest radiograph in a patient with bacterial pericarditis reveals cardiomegaly and left lower lobe infiltrate with marked increase in pulmonary ....
Careful handling of pericardial fluid is required to properly identify etiologic agents for pericardial effusion. Definitive therapy can begin when a precise diagnosis is established, often only after detailed examination of the fluid is completed.
The effusion of purulent pericarditis usually has a high white blood cell (WBC) count with predominately polymorphonuclear cells. Viral pericarditis produces a lymphocytic picture.
Fluid should be cultured for aerobic and anaerobic organisms, fungi, miliary tuberculosis, and viruses. Approximately 50%-60% of patients with purulent pericarditis have positive pericardial fluid cultures. Antigen detection tests can be helpful in patients who have received antibiotics.
In cases of suspected viral pericarditis, 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.[25]
If malignancy is suspected, other studies include cell count and differential, Gram stain, and cytology. Protein and lactate dehydrogenase (LDH) levels are often obtained, although both are usually elevated in most types of pericarditis.
If pericardiocentesis is unsuccessful in resolving tamponade, emergent surgical drainage is indicated. Surgical drainage is indicated in patients with fungal or bacterial pericarditis and continued effusion. Purulent pericarditis is rare and may require surgical drainage.[26]
Continuous drainage using specialized pericardial catheters and echographic monitoring has had reported success in treating bacterial pericarditis. However, the pericardial fluid may be too thick or loculated to be drained adequately by a catheter. Delay in adequate pericardial drainage is associated with increased mortality rates. Thus, a surgical approach to pericardial drainage may be indicated.
Various surgical procedures have been used to provide adequate pericardial drainage, and the optimal approach is controversial. Techniques for drainage include placement of a large-bore subxiphoid drainage tube (with or without irrigation), creation of a pericardial window and placement of a drain, or pericardiectomy. Proponents of pericardiectomy argue that thick clots and fibrin are not removed through a tube and that it prevents the possibility of late pericardial constriction and recurrent tamponade.
Pericardiectomy is rarely required to manage chronic recurrent cases. Video-assisted thoracoscopic window has been used in adult patients for diagnosis and management of pericardial effusions. Late pericardiectomy may be required in the rare patient who develops constrictive pericarditis as a complication of the infection.
In viral pericarditis, the following consultations are appropriate:
Pediatric cardiologist
Radiologist
Family physician
Psychologist
School teacher
Specialist nurse
Pharmacist
Dietitian
In bacterial pericarditis, the following consultations are appropriate:
Critical care specialist - Most patients with bacterial pericarditis present with severe hemodynamic compromise. Patients should be referred to specialists proficient in the use of vasoactive agents and mechanical ventilation.
Cardiologist - Patients with cardiomegaly and cardiovascular compromise require cardiology consultation and echocardiography to rule out myocardial or pericardial disease.
Cardiovascular surgeon - Emergent consultation with a cardiovascular surgeon is warranted in patients who have incomplete resolution of pericardial tamponade despite pericardiocentesis. Consultation should be considered in patients with recurrent effusions or constrictive pericarditis.
Long-term care for viral pericarditis may include the following:
Reevaluation of recurrent cases
Pericardial biopsy
Pericardiectomy
Continue anti-inflammatory therapy, such as aspirin or indomethacin, for at least several months to monitor the patient’s progress.
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.[27, 28]
Bacterial pericarditis
Once the patient has recovered from the acute infection, follow-up with a cardiologist is recommended to monitor for the development of constrictive pericarditis. Once identified, pericardiectomy is indicated.
Critically ill patients with suspected purulent pericarditis require transfer to a tertiary pediatric center with cardiac, cardiac surgical, and critical care medicine expertise. Bacterial pericarditis is a life-threatening disease that requires a full complement of pediatric subspecialty care.
Do not delay treatment of a critically ill infant in shock. Every hospital with echocardiographic capability should have someone who can perform an emergency pericardiocentesis.
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 nonsteroidal anti-inflammatory drugs (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. Management includes reinstitution of NSAIDs or corticosteroids. The use of immunosuppressive agents has been reported, and pericardiectomy should be reserved for patients with frequent recurrences. Colchicine has also been used in some patients, with a good response.
Clinical Context:
Diclofenac sodium possesses properties similar to propionic acid derivatives and reduces formation of inflammatory mediators by enzyme inhibition.
Clinical Context:
Indomethacin behaves like propionic acid derivatives and inhibits formation of inflammatory mediators. It is rapidly absorbed; metabolism occurs in the liver by demethylation, deacetylation, and glucuronide conjugation; it inhibits prostaglandin synthesis.
NSAIDs have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action inhibits cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may also occur, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.
Clinical Context:
The use of prednisolone is restricted to resistant cases that do not respond to nonsteroidal medications. This agent decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Clinical Context:
Prednisone is a corticosteroid that may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
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.
Clinical Context:
Oxacillin is a bactericidal penicillin antibiotic that inhibits cell wall synthesis. It is used in the treatment of infections caused by penicillinase-producing staphylococci. It may be used to initiate therapy when a staphylococcal infection is suspected.
Clinical Context:
Nafcillin is a bactericidal penicillin antibiotic that inhibits cell wall synthesis. It is used in the treatment of infections caused by penicillinase-producing staphylococci. It may be used to initiate therapy when a staphylococcal infection is suspected.
Clinical Context:
Vancomycin is indicated for patients with suspected or known infection with resistant organisms. To avoid toxicity, assay vancomycin trough levels 30 min before the fourth dose. Use creatinine clearance to adjust the dose in patients diagnosed with renal impairment.
Clinical Context:
Cefotaxime arrests bacterial cell wall synthesis, which, in turn, inhibits bacterial growth. It is a third-generation cephalosporin with a gram-negative spectrum. It has lower efficacy against gram-positive organisms.
Clinical Context:
Ceftriaxone is a third-generation cephalosporin with broad-spectrum gram-negative activity; it has lower efficacy against gram-positive organisms; it has higher efficacy against resistant organisms. It arrests bacterial growth by binding to one or more penicillin binding proteins.
Clinical Context:
Gentamicin is an aminoglycoside antibiotic used to provide gram-negative coverage. Dosing regimens are numerous; adjust the dose on the basis of creatinine clearance and changes in the volume of distribution. To avoid toxicity, assay trough levels 30 min before the fourth dose and peak levels 30-60 min after.
Clinical Context:
Ceftazidime is a third-generation cephalosporin with broad-spectrum, gram-negative activity, including pseudomonas. It has lower efficacy against gram-positive organisms and higher efficacy against resistant organisms. It arrests bacterial growth by binding to one or more penicillin-binding proteins, which, in turn, inhibit the final transpeptidation step of peptidoglycan synthesis in bacterial cell wall synthesis, thus inhibiting cell wall biosynthesis. The condition of the patient, severity of the infection, and susceptibility of the microorganism should determine the proper dose and route of administration.
Clinical Context:
Tigecycline is a glycycline antibiotic with properties and side effects similar to tetracyclines. Efficacy and safety in children younger than 18 years has not been established, and its use is restricted to highly selected patients for whom there is no other option.[15]
Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting. Until a definitive agent is identified, empiric therapy includes antibiotics to treat both S aureus and gram-negative bacilli. Initial empiric coverage requires a combination of a penicillinase-resistant penicillin and third-generation cephalosporin. In areas of high antibiotic resistance, substitute vancomycin for the penicillin antibiotic
Clinical Context:
Aspirin irreversibly inhibits platelet aggregation by inhibiting platelet cyclooxygenase. This, in turn, inhibits the conversion of arachidonic acid to PGI2 (potent vasodilator and inhibitor of platelet activation) and thromboxane A2 (potent vasoconstrictor and platelet aggregate).
Clinical Context:
Colchicine is an alkaloid extract that inhibits microtubule formation. It has unique anti-inflammatory properties. It concentrates well in leukocytes and reduces neutrophilic chemotaxis and motility. It reduces release of lactic acid and proinflammatory enzymes. It inhibits release of histamine-containing granules from mast cells, which may be important in pathogenesis of elastic tissue changes found in anetoderma.
Poothirikovil Venugopalan, MBBS, MD, FRCPCH, Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK
Disclosure: Nothing to disclose.
Coauthor(s)
John Berger, MD, Associate Professor, Department of Pediatrics, George Washington University School of Medicine, Director, Cardiac Intensive Care and Pulmonary Hypertension Program, Children's National Medical Center
Disclosure: Nothing to disclose.
Specialty Editors
Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Hugh D Allen, MD, Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine
Disclosure: Nothing to disclose.
Chief Editor
Stuart Berger, MD, Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine
Disclosure: Nothing to disclose.
Additional Contributors
Ira H Gessner, MD, Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine
Plain chest radiograph in a 2-year-old boy with viral pericarditis and massive pericardial effusion.
Left: Chest radiograph in a patient with bacterial pericarditis reveals cardiomegaly and left lower lobe infiltrate with marked increase in pulmonary vascular markings. Right: The same patient after placement of a pigtail pericardial catheter and pulmonary artery catheter.
Two-dimensional echocardiograph shows a large pericardial effusion.
Apical four-chamber view from a patient with bacterial pericarditis. The large pericardial effusion (EF) appears as an echo clear space in this view surrounding the right atrium (RA) and left ventricle (LV). The RA wall is collapsed, indicating tamponade. The longer the duration of RA inversion into systole correlates with increasing hemodynamic severity.
A 12-lead ECG from a patient with bacterial pericarditis demonstrating marked ST elevation in multiple leads.
This ECG shows markedly decreased QRS voltage and electrical alternans (especially in lead V1)
Left: Chest radiograph in a patient with bacterial pericarditis reveals cardiomegaly and left lower lobe infiltrate with marked increase in pulmonary vascular markings. Right: The same patient after placement of a pigtail pericardial catheter and pulmonary artery catheter.
Two-dimensional echocardiograph shows a large pericardial effusion.
Plain chest radiograph in a 2-year-old boy with viral pericarditis and massive pericardial effusion.
Left: Chest radiograph in a patient with bacterial pericarditis reveals cardiomegaly and left lower lobe infiltrate with marked increase in pulmonary vascular markings. Right: The same patient after placement of a pigtail pericardial catheter and pulmonary artery catheter.
Apical four-chamber view from a patient with bacterial pericarditis. The large pericardial effusion (EF) appears as an echo clear space in this view surrounding the right atrium (RA) and left ventricle (LV). The RA wall is collapsed, indicating tamponade. The longer the duration of RA inversion into systole correlates with increasing hemodynamic severity.
A 12-lead ECG from a patient with bacterial pericarditis demonstrating marked ST elevation in multiple leads.
This ECG shows markedly decreased QRS voltage and electrical alternans (especially in lead V1)
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