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Pericardial Effusion

Background: Pericardial effusion defines the presence of an abnormal amount and/or character of fluid in the pericardial space. It can be caused by a variety of local and systemic disorders, or it may be idiopathic. Pericardial effusions can be acute or chronic, and the time course of development has a great impact on the patient’s symptoms. Treatment varies, and is directed at both removal of the pericardial fluid and alleviation of the underlying cause, which usually is determined by a combination of fluid analysis and correlation with comorbid illnesses.

Pathophysiology: The pericardial space normally contains 15-50 cc of fluid, which serves as lubrication for the visceral and parietal layers of the pericardium. This fluid is thought to originate from the visceral pericardium and is essentially an ultrafiltrate of plasma. Total protein levels are generally low; however, the concentration of albumin is increased in pericardial fluids owing to its low molecular weight.
The cause of abnormal fluid production depends on the underlying etiology, but usually it is secondary to injury or insult to the pericardium (ie, pericarditis). Transudative fluids result from obstruction of fluid drainage, which occurs through lymphatic channels. Exudative fluids occur secondary to inflammatory, infectious, malignant, or autoimmune processes within the pericardium.
Clinical manifestations of pericardial effusion are highly dependent upon the rate of accumulation of fluid in the pericardial sac. Rapid accumulation of pericardial fluid may cause elevated intrapericardial pressures with as little as 80 cc of fluid, while slowly progressing effusions can grow to 2 liters without symptoms.

Frequency:
  • In the US: Few large studies have characterized the epidemiology of pericardial effusion. Pericardial effusion has been found in 3.4% of subjects in general autopsy studies. Small pericardial effusions often are asymptomatic.
A higher incidence of pericardial effusion is associated with certain diseases. Twenty-one percent of cancer patients have metastases to the pericardium. The most common are lung (37% of malignant effusions), breast (22%), and leukemia/lymphoma (17%). Patients with HIV, with or without AIDS, are found to have increased prevalence, with 41-87% having asymptomatic effusion and 13% having moderate-to-severe effusion.
Mortality/Morbidity: Dependent upon etiology and comorbid conditions
  • Idiopathic effusions are well tolerated in most patients. As many as 50% of patients with large, chronic effusions were asymptomatic during long-term follow-up.
  • Pericardial effusion is the primary or contributory cause of death in 86% of cancer patients with symptomatic effusions.
  • Survival rate for patients with HIV and symptomatic pericardial effusion is 36% at 6 months, 19% at 1 year.
Race:
  • No consistent difference among races is reported in the literature.
  • AIDS patients with pericardial effusion are more likely to be white.
Sex:
  • No sexual predilection exists.
Age:
  • Observed in all age groups
  • Mean occurrence in fourth or fifth decades; earlier in patients with HIV
History:
  • Cardiovascular
  • Chest pain, pressure, discomfort
  • Light-headedness, syncope
  • Palpitations
  • Respiratory - Cough, dyspnea, hoarseness
  • Gastrointestinal - Hiccoughs
  • Neurologic - Anxiety, confusion
Physical:
  • Cardiovascular
    • Classic Beck triad of pericardial tamponade - Hypotension, muffled heart sounds, jugular venous distension
    • Pulsus paradoxus - Exaggeration of physiologic respiratory variation in systemic blood pressure, defined as a decrease in systolic blood pressure of more than 10 mm Hg with inspiration, signaling falling cardiac output during inspiration
    • Pericardial friction rub - Best heard in the supine position, at end exhalation, with diaphragm of stethoscope
    • Widened pulse pressure
    • Tachycardia
    • Hepatojugular reflux - Can be observed by applying pressure to the periumbilical region. A rise in the jugular venous pressure (JVP) of greater than 3 cm H 2 O for greater than 30 seconds suggests elevated central venous pressure. Transient elevation in JVP may be normal.
  • Respiratory
    • Tachypnea
    • Decreased breath sounds (secondary to pleural effusions)
    • Ewart sign - Dullness to percussion beneath the angle of left scapula from compression of the left lung by pericardial fluid
  • Gastrointestinal - Hepatosplenomegaly
  • Extremities
    • Weakened peripheral pulses
    • Edema
  • Cyanosis
Causes:
  • Idiopathic
  • Hydropericardium - Congestive heart failure, valvular disease
  • Neoplastic - Malignant, nonmalignant. Not all cancer-associated effusions are malignant. Mediastinal lymphoma, Hodgkin disease, and metastatic breast cancer have been found to cause transient effusions, likely due to impaired lymphatic drainage, which do not cause long-term sequelae.
  • Infectious - Bacterial, viral, fungal, parasitic, tuberculosis, HIV related
  • Autoimmune or connective tissue disorders - Systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, vasculitides
  • Trauma
  • Uremia
  • Drugs
  • Postpericardiotomy syndrome
  • Chylopericardium
  • Myxedema
  • Radiation
Lab Studies:
  • Electrolytes - Metabolic abnormalities (eg, renal failure)
  • CBC count with differential - Leukocytosis for evidence of infection, as well as cytopenias, as signs of underlying chronic disease (eg, cancer, HIV)
  • Cardiac enzymes - To rule out myocardial ischemia
  • Thyroid-stimulating hormone - Thyroid-stimulating hormone screen for hypothyroidism
  • Rickettsial antibodies - If high index of suspicion of tick-borne disease
  • Rheumatoid factor, immunoglobulin complexes, antinuclear antibody test (ANA), and complement levels (which would be diminished) - In suspected rheumatologic causes
  • Pericardial fluid analysis - Routine tests
    • Lactic (acid) dehydrogenase (LDH), total protein - The Light criteria (for exudative pleural effusion) found to be as reliable in distinguishing between exudative and transudative effusions
      • Total protein fluid-to-serum ratio >0.5
      • LDH fluid-to-serum ratio >0.6
      • LDH fluid level exceeds two thirds of upper-limit of normal serum level
    • Other indicators suggestive of exudate - Specific gravity >1.015, total protein >3.0 mg/dL, LDH >300 U/dL, glucose fluid-to-serum ratio
    • Cell count - Elevated leukocytes (ie, >10,000) with neutrophil predominance suggests bacterial or rheumatic cause, although unreliable
    • Gram stain - Specific but insensitive indicator of bacterial infection
    • Cultures - Signals and identifies infectious etiology
    • Fluid hematocrit for bloody aspirates - Hemorrhagic fluid hematocrits usually significantly less than simultaneous peripheral blood hematocrits
  • Pericardial fluid - Special tests
    • Viral cultures
    • Adenosine deaminase; polymerase chain reaction (PCR); culture for tuberculosis; smear for acid-fast bacilli in suspected tuberculosis infection, especially in patients with HIV
Imaging Studies:
  • Chest radiography
    • Findings include enlarged cardiac silhouette (so-called water-bottle heart), pericardial fat stripe.
    • A third of patients have a coexisting pleural effusion.
    • Radiography is unreliable in establishing or refuting diagnosis of pericardial effusion.
  • Echocardiogram
    • This is the criterion standard for noninvasive diagnosis.
    • Pericardial effusion appears as an "echo-free" space between the visceral and parietal pericardium. Early effusions tend to accumulate posteriorly owing to expandable posterior/lateral pericardium. Large effusions are characterized by excessive motion within the pericardial sac. Severe cases may be accompanied by diastolic collapse of the right atrium and right ventricle (and in hypovolemic patients the left atrium and left ventricle), signaling the onset of pericardial tamponade.
    • Large pericardial effusions are defined as greater than 1 cm thick on echocardiography, completely surrounding the heart. Small effusions are less than 1 cm and often localized, usually posteriorly.
    • False-positive echocardiograms can occur in pleural effusions, pericardial thickening, increased pericardial fat (especially the anterior epicardial fat pad), atelectasis, and mediastinal lesions.
  • Transesophageal echocardiography: Transesophageal echocardiography (TEE) is useful in characterizing loculated effusions.
  • CT scan
    • Potentially can determine composition of fluid
    • May detect as little as 50 cc of fluid
    • Fewer false-positives than with echocardiography
    • Problem: Patients must be transported to the CT scanner, which may not be possible if the patient’s condition is unstable.
  • MRI
    • Can detect as little as 30 cc of pericardial fluid
    • May be able to distinguish hemorrhagic and nonhemorrhagic effusions
    • More difficult to perform than CT scan acutely, given the length of time the patient must remain in the scanner
Other Tests:
  • ECG
    • Classic findings include low voltage, sinus tachycardia, electrical alternans, and PR-segment depression and diffuse ST elevation if pericarditis is present (see Image 4 ).
    • Two studies found that these ECG findings are not particularly accurate in identifying pericardial effusion.
Procedures:
  • Pericardiocentesis
    • This procedure is used for diagnostic as well as therapeutic purposes. Support for the use of echocardiographic guidance is increasing, unless emergent treatment is required.
    • Indications include impending hemodynamic compromise (ie, pericardial tamponade), suspected infectious etiology, and uncertain etiology.
    • Use of a needle that is at least 5 cm long, 16-gauge in diameter, and has a short bevel can minimize the risk of complications and should allow for adequate pericardial drainage. A system allowing placement of a catheter over the needle is preferred.
    • Contrast echocardiography using agitated saline is useful in cases when bloody fluid is aspirated to determine if the needle is in the ventricular cavity.
    • Attaching an ECG electrode to the pericardiocentesis needle is also useful for avoiding myocardial puncture. Electrical activity will be seen on the monitor when the needle comes into contact with atrial or ventricular myocardium. These changes may be delayed, however, and instill a false sense of security in needle placement; sense of touch and the findings on aspiration should guide the procedure, with the clinician ultimately relying on good clinical sense.
    • Complications of pericardiocentesis include ventricular rupture, dysrhythmias, pneumothorax, myocardial and/or coronary artery laceration, and infection.
    • Recurrence rates within 90 days may be as high as 90% in patients with cancer.
  • Balloon pericardotomy
    • A catheter is placed in the pericardial space under fluoroscopy, which, after inflation of the balloon, creates a channel for passage of fluid into the pleural space, where reabsorption occurs more readily.
    • This may be useful for recurrent effusions.
  • Pericardial sclerosis
    • Several pericardial sclerosing agents have been used with varying success rates (eg, tetracycline, doxycycline, cisplatin, 5-fluorouracil).
    • The pericardial catheter may be left in place for repeat instillation if necessary until the effusion resolves.
    • Complications include intense pain, atrial dysrhythmias, fever, and infection.
    • Success rates are reported as high as 91% at 30 days.
  • Pericardioscopy
    • This procedure is not universally available.
    • It may increase diagnostic sensitivity in cases of unexplained pericardial effusions. It allows for visualization of pericardium and for pericardial biopsies.
Medical Care:
  • Initially, medical care is focused on determination of the underlying etiology.
  • Intravenous fluid resuscitation may be helpful in cases of hemodynamic compromise.
  • Antineoplastic therapy (eg, systemic chemotherapy, radiation) in conjunction with pericardiocentesis has been shown to be effective in reducing recurrences of malignant effusions.
  • Corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) are helpful in patients with autoimmune conditions.
Surgical Care:
  • Subxiphoid pericardial window with pericardiostomy
    • This procedure is associated with low morbidity, mortality, and recurrence rates.
    • It can be performed under local anesthesia.
    • It may be less effective when effusion is loculated.
    • It may replace pericardiocentesis as initial treatment for stable pericardial effusions.
    • A recent study suggests that this may be safer and more effective at reducing recurrence rates than pericardiocentesis. However, only patients who were hemodynamically unstable underwent pericardiocentesis, and no change in overall survival rate was observed.
  • Thoracotomy
    • This should be reserved for patients in whom conservative approaches have failed.
    • Thoracotomy allows for creation of a pleuropericardial window, which provides greater visualization of pericardium.
    • Thoracotomy requires general anesthesia and thus has higher morbidity and mortality rates than the subxiphoid approach.
  • Video-assisted thoracic surgery
    • Video-assisted thoracic surgery (VATS) enables resection of a wider area of pericardium than the subxiphoid approach without the morbidity of thoracotomy.
    • The surgeon is able to create a pleuropericardial window and address concomitant pleural pathology, which is especially common in patients with malignant effusions.
    • One disadvantage of VATS is that it requires general anesthesia with single lung ventilation, which may be difficult in otherwise seriously ill patients.
  • Median sternotomy
    • This procedure is reserved for patients with constrictive pericarditis.
    • Operative mortality rate is high (5-15%).
Consultations:
  • A cardiologist should be involved in the care of patients with pericardial effusion.
  • Cardiothoracic surgery may be required for recurrent or complicated cases.
Further Inpatient Care:
  • Patients who present with significant symptoms or cardiac tamponade require emergent treatment and admission to ICU.
  • The pericardial catheter (if placed) should be removed within 24-48 hours to avoid infection.
  • Symptomatic patients should remain hospitalized until definitive treatment is accomplished and/or symptoms have resolved.
Further Outpatient Care:
  • Patients should be educated on symptoms of increasing pericardial effusion and should be evaluated whenever these symptoms begin to occur.
  • Indications for echocardiography after diagnosis include the following:
    • A follow-up imaging study to evaluate for recurrence/constriction: Repeat studies may be performed to answer specific clinical questions.
    • The presence of large or rapidly accumulating effusions (to detect early signs of tamponade)
Transfer:
  • Symptomatic patients requiring treatment (who are surgical candidates) should receive care at an institution with cardiothoracic surgery capabilities.
Complications:
  • Pericardial tamponade
    • Can lead to severe hemodynamic compromise and death
    • Heralded by equalization of diastolic filling pressures
    • Treat with expansion of intravascular volume (small amounts of crystalloids or colloids may lead to improvement, especially in hypovolemic patients) and urgent pericardial drainage. Avoid positive-pressure ventilation if possible, as this decreases venous return and cardiac output. Vasopressor agents are of little clinical benefit.
  • Chronic pericardial effusion
    • Effusions lasting longer than 6 months
    • Usually well tolerated
Prognosis:
  • Patients with symptomatic pericardial effusions from HIV/AIDS or cancer have high short-term mortality rates.
  • Patients with idiopathic effusions generally have a good prognosis.
  • The prognosis of patients with most other types of pericardial effusions depends on the treatment and on control of the underlying condition that precipitated the effusion.

 

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