AUTHOR AND EDITOR INFORMATION

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INTRODUCTION

Section 2 of 11  

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Background

Pericardial involvement in patients with malignancy is common. Widespread use of noninvasive diagnostic techniques, such as echocardiography and CT scanning, has increased awareness of this diagnosis. The mere presence of pericardial effusion does not necessarily imply pericardial infiltration by malignant cells.

Pathophysiology

The pericardium consists of 2 layers, the visceral pericardium (epicardium) and the parietal pericardium, which enclose a potential space (ie, the pericardial cavity) between them. This cavity is normally lubricated by a very small amount of serous fluid (<30 mL in adults). Inflammation of the pericardium or obstruction of lymphatic drainage from the pericardium of any etiology causes an increase in fluid volume, referred to as a pericardial effusion.

Malignant involvement of the pericardium may be primary (less common) or secondary to spread from a nearby or distant focus of malignancy. Secondary neoplasms can involve the pericardium by contiguous extension from a mediastinal mass, nodular tumor deposits from hematogenous or lymphatic spread, and diffuse pericardial thickening from tumor infiltration (with or without effusion). In diffuse pericardial thickening, the heart may be encased by an effusive-constrictive pericarditis.

Other rare mechanisms include chronic myelomonocytic leukemia and intrapericardial extramedullary hematopoiesis with preleukemic conditions or during blast crisis in chronic myeloid leukemia. Obstruction of lymphatic drainage by mediastinal tumors, either benign or malignant, can also give rise to pericardial effusion, which can be chylous. The above mechanisms may act independently or jointly in any particular child with malignancy. The underlying myocardium is not involved in most patients.

Pathogenesis of clinical manifestations

In healthy individuals, 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

Pericardial effusion is a common cause of pericarditis, occurring in approximately 5-15% of patients with malignant neoplasms, according to autopsy data.

Most cardiac tumors in infants and children are benign (eg, rhabdomyoma, fibromas) and are rarely associated with pericardial involvement.¹

International

A study of 236 children in Poland reported cardiac involvement in 15% of children, including pericardial effusion in 7% of children.²

Mortality/Morbidity

Children with pericardial involvement due to malignancy have more extensive disease and, hence, a worse prognosis; pericardial tamponade may add to the mortality unless promptly detected and appropriately treated.^{2, 3}

Sex

Both sexes are affected. Medary et al reported higher incidence in males than in females at a ratio of 7:3.⁴

Age

All ages are affected, but pericardial effusion is more common in older children and adolescents. Medary et al reported a mean age of 14 y.⁴ This may be related to the longer survival of older children with malignancy.

CLINICAL

Section 3 of 11  

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History

• Pericardial malignancy is often asymptomatic. It is observed on chest radiography performed to evaluate the lungs or diagnosed as an incidental finding at autopsy.
• Although pericardial malignancy may be reported as an incidental finding, it may have contributed to the symptomatology and even death. A review of some cases leads to the conclusion that symptoms may be incorrectly attributed to the underlying neoplasm.
• Shortness of breath or dyspnea is the most common symptom (85%).
• Other manifestations may include chest pain, shoulder pain, and a hacking cough that varies with posture. Sitting up and leaning forward improves the cough. Orthopnea may be present.
• Primary cardiac malignancy presents as unresponsive heart failure.
• Cardiac tamponade may rarely be the initial manifestation of systemic malignancy.

Physical

• Central venous pressure is increased.
• Jugular venous pressure is elevated and jugular veins are not pulsatile.
• The liver may be enlarged, and peripheral edema and ascites may be present.
• Encountering evidence of pulmonary edema is unusual because pericardial effusion limits the amount of blood that can enter the heart, and the left atrial pressure does not exceed the right atrial pressure.
• Heart sounds may be distant or faint.
• Pericardial friction rub may be observed.
• • A sign of pericardial inflammation is a grating, scratching sound caused by abrading of inflamed pericardial surfaces with cardiac motion.
  • Pericardial friction rub may have as many as 3 components.
  • • In the presence of a large effusion, heart sounds may be muffled, and the rub may disappear.
    • It is best heard in the second-fourth intercostal spaces along the left sternal border or along the mid clavicular line and is loudest in the upright position with the patient leaning forward.
    • It is often accentuated in inspiration.
  • The occasional persistence of a rub in pericardial tamponade is believed to represent a friction between the inflamed parietal pericardium and the pleura.
• Ewart sign may be observed.
• • This refers to subscapular dullness to percussion. It represents compression of the left lung by a massively enlarged heart and may be associated with abnormal breath sounds in that region.
  • No crepitations or rhonchi are heard.
• Cardiac tamponade features are as follows:
• • Low cardiac output
  • Elevated central venous pressures
  • Paradoxical pulse
  • Muffled or diminished heart sounds
  • Tachycardia
  • Jugular venous distension reflecting high central venous pressure
  • Low systolic blood pressure and low pulse pressure
• Pulsus alternans may be present.
• • This consists of a drop in systolic blood pressure in alternate beats, another ominous sign.
  • This sign is most reliably documented while observing intraarterial blood pressure tracing, rather than by palpating the pulse itself.
• Congenital intrapericardial tumors may be associated with fetal hydrops secondary to compression of fetal venous structures.

Causes

• Primary malignant neoplasms
• • Pericardial mesothelioma
  • Fibrosarcoma
  • Angiosarcoma
  • Liposarcoma
  • Lymphoma
  • Malignant pericardial teratoma
  • Rhabdomyosarcoma with tuberous sclerosis
  • Pheochromocytoma
  • Kaposi sarcoma and primary cardiac lymphoma in association with human immunodeficiency virus (HIV) infection
  • Intrapericardial teratoma in the fetus and neonate
• Metastatic or infiltrative disease
• • Non-Hodgkin lymphoma
  • Neuroblastoma
  • Ganglioneuroblastoma
  • Pheochromocytoma
  • Sarcomas
  • Wilms tumor
  • Hodgkin lymphoma
  • Primary mediastinal (thymic) B-cell lymphoma
  • Adenocarcinoma
  • Mesothelioma
  • Pulmonary lymphangiomatosis
  • Malignant fibrous histiocytomas
  • Leiomyosarcomas
  • Liposarcomas
  • High-grade sarcomas
  • Burkitt lymphoma

DIFFERENTIALS

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Other Problems to be Considered

Drug-induced hydralazine, isoniazid, procainamide
Chronic graft-versus-host disease (cGVHD)
Constrictive pericarditis
Purulent pericarditis
Radiation pericarditis
Tuberculous pericarditis
Uremic pericarditis
Hemolytic uremic syndrome after bone marrow transplantation
Posttransplant B-cell lymphoproliferative disorder (PTLD)
Doxorubicin- and daunorubicin-related pericarditis or myocardial dysfunction
Cystic lymphangioma
Effusion possibly related to pre–bone-marrow transplant drug conditioning
Malignant hepatic involvement with portal hypertension
Microvascular tumor spread in lungs with secondary pulmonary hypertension
Pericardial celomic cyst (unilocular)
Pericardial teratoma
Superior venacaval obstruction of any cause
Chylous or lymphatic pericardial effusions with (1) congenital thoracic cystic hygroma with pericardial involvement, (2) following surgery for congenital heart disease complicated by elevated venous pressures or trauma to the thoracic duct, or (3) secondary to obstruction of lymphatic drainage by mediastinal masses

WORKUP

Section 5 of 11  

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• Follow-up
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Lab Studies

• Blood investigations as dictated by the general condition
• • CBC count with platelet count and WBC differential
  • Blood film (smear)
  • Serum chemistry
  • Erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • Blood cultures
• Markers of specific malignancy may help in following disease progression
• • Serum alpha fetoprotein
  • Serum caner antigen (CA) 125
• Markers of malignancy in pericardial fluid cells
  • Human telomerase reverse transcriptase (hTERT) mRNA expression may be detected in abnormal cells in body fluids using in situ hybridization (ISH).
  • Molecular genetic studies can also be helpful in the analysis of lymphocyte-rich serous pericardial effusion.
  • Immunocytochemical panel of tests has also been suggested in selected cases.
• Electrocardiography
• • Changes are nonspecific and may represent the effect of pericardial inflammation on the underlying myocardium.
  • Low voltage QRS complexes (35%) are seen.
  • ST-segment elevation is found.
  • T-wave inversion is seen.
  • Development of arrhythmias (atrial tachycardia, atrial fibrillation, heart block) is observed.
  • Electrical alternans (17%), a beat-to-beat variation in QRS amplitude, occurs with excessive motion of the heart within the fluid-filled pericardial space.

Imaging Studies

• Chest radiography
• • Chest radiography reveals a varying degree of cardiomegaly, depending on the amount of pericardial fluid and its rate of accumulation.
  • Rapidly accumulating effusion is associated with relatively minimal cardiomegaly.
  • Massive effusions produce a large cardiac shadow, causing the characteristic water-bottle heart or triangular heart with smoothed-out cardiac borders.
  • Pleural effusion, mediastinal widening, hilar mass, or, less commonly, irregular nodular contour of the cardiac silhouette or a bony or parenchymal metastatic deposit may indicate underlying disease.
  • Rarely, pericardial calcification may be evident.
• Echocardiography
• • Echocardiography is the primary study performed for diagnosis and quantification of the effusion, as well as for guiding needle pericardiocentesis. Echocardiography has a 96% diagnostic accuracy in pericardial effusion.
  • Pericardial fluid gives the appearance of an echo-free space between the epicardial and pericardial reflections. This is evident in both 2-dimensional images and M-mode images.
  • When effusion is minimal, it accumulates posterior to the left ventricle and is more apparent in systole.
  • When effusion is massive, fluid is observed all around the heart and throughout the cardiac cycle.
  • Diastolic ventricular filling is abnormal secondary to cardiac compression.
  • A swinging motion of the heart may be observed within the pericardial cavity, along with abnormalities of septal motion, dilation of the inferior vena cava, and loss of respiratory caval motion.
  • Irregular undulating masses that protrude into the pericardial space, atria, ventricles, or even into the pulmonary arteries are reported.
  • Fetal echocardiography can identify fetal pericardial effusion secondary to fetal pericardial malignancy (most commonly teratoma).
• CT scanning
• • CT scanning reveals the thickness and density of the pericardium and content of the pericardial space.
  • CT scanning aids in identification of constrictive pericarditis by providing additional information on the status of the vena cava, atria, ventricles, and pleural changes.
  • The minimum amount of pericardial fluid that can be detected by CT scanning is estimated to be 10 mL.
  • CT scanning provides added information regarding the presence and location of space-occupying masses within the pericardium and adjacent mediastinum and lungs.
• Transesophageal echocardiography (TEE): This provides information regarding the presence and location of space-occupying masses within the pericardium.
• MRI: MRI provides added information regarding the presence and location of space-occupying masses within the pericardium and adjacent mediastinum and lungs. In addition, this technique is more sensitive in differentiating malignant lesions from benign ones.
• Radionuclide imaging: This can demonstrate a pericardial effusion in previously undiagnosed pericardial disease.

Procedures

• Pericardiocentesis
• • This is used to diagnose cause, assess cytology, and treat hemodynamic compromise in the presence of cardiac tamponade.
  • Malignancy may be first suspected from pericardial fluid analysis in as many as 5% of such patients.
  • Catheter drainage technique is commonly used.
  • Ultrasonographic guidance adds to the safety of the procedure but is not a requisite with emergency drainage.
  • Insert a beveled, sharp needle beneath the xiphoid process and angle upward and leftward toward the left shoulder. Sometimes, a pop is felt as the needle is passed into the pericardium.
  • Attempts to withdraw fluid are made with each advance of the needle.
  • If fluid is obtained, remove enough to alleviate tamponade. Even a small amount often provides significant benefit.
  • Process the pericardial fluid for cytology, biochemistry, and culture and sensitivity, including viral and fungal cultures in relevant cases.
  • Exudates differ from transudates by demonstrating higher leukocyte counts, lower glucose, higher protein contents, and higher specific gravity.
  • Cytospin preparations can be stained with Wright-Giemsa stain to identify cellular morphology that, in turn, can be used to test for immunologic markers and for electron microscopy.
  • Complications are myocardial puncture, coronary artery or vein laceration, hemopericardium, laceration of the internal mammary artery, pneumothorax, and liver and aortic injury.
  • In the presence of significant effusion, maintain good hydration and effective filling pressures to help maintain perfusion until pericardiocentesis can be performed.
• Closed drainage technique
• • Continuous drainage of the pericardial space is accomplished by advancement of a pigtail catheter over a guide wire.
  • This is necessary for patients in whom the effusion reaccumulates rapidly.
• Pericardioscopy: This can reveal neoplastic effusions by direct observation and by obtaining a biopsy of the pericardium for further analysis.
• Video-assisted thoracoscopic pericardial fenestration: This is safe and effective for loculated pericardial effusions previously treated by percutaneous drainage maneuvers and patients with concomitant pleural disease.
• Fetal pericardiocentesis: This has been used in the treatment of pericardial effusion secondary to fetal teratoma.
• Pericardial biopsy
• • Specimens can be obtained by open pericardiotomy or during thoracotomy (rare). Pericardial biopsy has a sensitivity of approximately 55% for diagnosing malignant involvement; however, together with cytology of the pericardial fluid, pericardial biopsy provides nearly 100% sensitivity.
  • Open pericardial biopsy may be required if initial cytology is negative. Obtaining a larger biopsy specimen by open biopsy should provide a histologic diagnosis in up to 90% of cases. The procedure carries significant risk in patients who are critically ill, but a false-negative diagnosis may occur if the tissue sample is too small.
  • Biopsy specimens are subjected to histologic and immunohistologic evaluations, polymerase chain reaction, or in situ hybridization analysis for microbial DNA and ribonucleic acid.
• Cardiac catheterization: This is not required for diagnosis of pericardial effusion. Potential indications for cardiac catheterization include the following:
• • Suspected superior venacaval obstruction and pulmonary microvascular tumor (lymphangitic tumor) may occur with malignant cardiac tamponade and contribute to the development of facial edema and jugular venous distension.
  • Cyanosis, hypoxemia, and elevated pulmonary vascular resistance may indicate pulmonary microvascular tumor (lymphangitic tumor). The diagnosis can be established by obtaining a blood sample for cytologic analysis from the pulmonary capillary wedge position using the right heart catheter.

Histologic Findings

• Malignant pericardial effusion is often hemorrhagic or serosanguineous, but this alone does not differentiate among neoplastic, radiation, or idiopathic causes. Because treatment strategies differ, carrying out a meticulous cytologic examination of the fluid is essential in an attempt to differentiate malignant pericarditis from other causes. False-negative cytology is uncommon in carcinomatous pericarditis but, when it occurs, it may be due to scant cellularity or the presence of obscuring blood. False-negative results are more common with lymphoma and mesothelioma. Chylothorax is most often reported with mediastinal lymphangioma.
• Detection of malignant cells in effusions is facilitated by the use of immunocytochemistry, using a wide panel of antibodies. BerEP4 and B72.3 appear to be the best markers when both sensitivity and specificity are considered, followed by BG8. Carcinoembryonic antigen (CEA) and CA-125 have a limited role in detection of metastases from gynecologic tumors because of the low sensitivity of CEA and the low specificity of the CA-125.
• Flow cytometry can also be used to detect DNA diploidy (benign) and aneuploidy (malignant), but the results have not been uniformly convincing. The low sensitivity of flow cytometric DNA analysis does not favor routine use.

TREATMENT

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Medical Care

• Medical care is dictated mainly by the general condition of the patient and the underlying malignancy.
• Remember that almost 50% of patients with symptomatic pericardial effusion and neoplastic disease have a nonmalignant cause, such as radiation-related, idiopathic, infectious (including tuberculous and fungal), and lymphatic obstruction.
• Intrapericardial administration of drugs, such as cisplatin, has been attempted and can be of significant value.

Surgical Care

• Open surgical drainage (pericardiotomy)
• • The safety and effectiveness of surgical drainage of pericardial fluid via pericardiectomy (compete or partial) or the creation of a pericardial window are well recognized.
  • This procedure removes fluid that is excessively thick. Perform open surgical drainage if purulent pericarditis is present. Obtain biopsy specimens from the pericardium and epicardium.
  • Total pericardiectomy may be required, especially in the presence of a thickened pericardium that has a constricting effect.
• Thoracotomy may be required to arrive at a complete diagnosis.

Consultations

• Pediatrician
• Pediatric cardiologist
• Pediatric oncologist
• Radiologist
• Nuclear medicine specialist
• Cardiothoracic surgeon
• Physiotherapist
• Occupational therapist
• Specialist nurse
• Family physician

Diet

• No special diet requirements are necessary.

Activity

• Restrict activity only to the limit of intolerance.

MEDICATION

Section 7 of 11  

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Hemodynamic support is of some value until drainage of pericardial fluid can be accomplished. Pericardiocentesis and intrapericardial sclerosis are effective therapies for malignant pericardial effusions that recur. Intrapericardial administration of drugs, such as cisplatin, can be important. Use anti-inflammatory drugs for viral pericarditis.

Drug Category: Nonsteroidal anti-inflammatory drugs

These agents are analgesics that offer anti-inflammatory action. They 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.

Drug Category: Corticosteroids

These agents elicit 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

• Further inpatient care is determined by the underlying condition.

Further Outpatient Care

• Further outpatient care is often required to look for evidence of constrictive pericarditis.

In/Out Patient Meds

• Medication is dictated by the underlying malignancy.

Transfer

• Transfer pediatric patients with a pericardial effusion to a facility that provides pediatric cardiology and cardiovascular services. If a diagnosis of malignancy is suspected, immediate availability of pediatric oncologists is necessary.

Deterrence/Prevention

• Although reported as an incidental finding in some patients, malignant pericardial effusion can contribute to symptomatology and even death.
• Symptoms may be wrongly attributed to the underlying neoplasm.

Complications

• Cardiac tamponade
• Sudden death
• Retinoic acid syndrome
• • Retinoic acid syndrome is characterized by fever and respiratory distress, along with weight gain, pleural or pericardial effusions, peripheral edema, thromboembolic events, and intermittent hypotension.
  • Relate to all trans-retinoic acid therapy for underlying malignancy.
• Leukemic coronary artery occlusion

Prognosis

• Detection of malignant cells in pleural, peritoneal, and pericardial fluids of patients with cancer marks the presence of metastatic disease, usually establishing a grave prognosis; however, all patients with malignancy and pericardial effusion do not have metastatic involvement.
• Immediate relief of large effusions is essential to prolong survival.

Patient Education

• Educate patients about cardiac symptoms of tamponade and the need to follow up with regular examination.

MISCELLANEOUS

Section 9 of 11  

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Medical/Legal Pitfalls

• Failure to diagnose and appropriately react to presence of a pericardial effusion

MULTIMEDIA

Section 10 of 11  

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Media file 1:  Plain chest radiograph in a 3-month-old infant with pneumonia and malignant pericardial effusion showing cardiomegaly and bilateral pneumonic patches.
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Media type:  Radiograph

Media file 2:  Two-dimensional echocardiograph from a subcostal window showing a large pericardial effusion.
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Media type:  Echo

Media file 3:  M-mode echocardiograph in a child with pericardial effusion.
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Media type:  Echo

Media file 4:  Cytologic features of malignant pericardial effusion. Smear of centrifuged pericardial fluid in a patient with malignant pericardial involvement from lymphoma. Low-power view showing numerous mononuclear cells along with large atypical malignant cells.
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Media type:  Photo

Media file 5:  Cytologic features of malignant pericardial effusion. Smear of centrifuged pericardial fluid in a patient with malignant pericardial involvement from lymphoma. High-power view showing morphologic details of the malignant cells. These cells are large and show oval hyperchromatic nuclei, some of them having nucleoli. The cytoplasm is reduced to a thin rim.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 11 of 11

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Article Last Updated: Jul 24, 2008