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A. Etiology [3,4] navigator

  1. Idiopathic (~85%) - often classified as viral, but etiology not clear
  2. Viral Pericarditis (~2%)
    1. Cocksackie B Virus
    2. Echovirus
    3. Adenovirus
    4. Other enteroviruses
    5. Smallpox live attenuated vaccine - myopericarditis 7.8 per 100,000 primary vaccinees []
  3. Malignancy (~7%)
    1. Lung and Breast Cancers and Lymphoma are most common
    2. Mesothelioma (usually associated with asbestosis)
    3. Metastatic Gastrointestinal Tumors
  4. Metabolic
    1. Uremia - up to 5% of severe renal failure
    2. Up to 15% of chronic hemodialysis patients
    3. Hypercholesterolemia
    4. Myxedema - only severe hypothyroidism
  5. Other infections
    1. Tuberculosis (~4%): subacute, usually followed by calcification, often with effusion [14]
    2. Acute Bacterial: Staphylococcus, Pneumococcus, Klebsiella, anaerobic species
    3. Helicobacter (previously Campylobacter) cinaedi (very rare) [4]
    4. Fungal: Candida, Aspergillus, Nocardia, Histoplasmosis (very uncommon)
    5. Lyme Disease
    6. Rickettsia - spotted fever (R. rickettsii), R. helvetica, R. typhis, others [7]
  6. Drug Induced
    1. Procainamide: up to 50% of patients
    2. Hydralazine
    3. Very uncommon: phenytoin (Dilantin®), minoxidil
    4. These agents induce pericarditis usually in setting of drug-induced lupus syndrome
    5. Serum eosinophilia is often present
  7. Idiopathic Inflammatory Disease
    1. Commonly associated with systemic lupus erythematosus (SLE) [5], rheumatoid arthritis, systemic sclerosis (scleroderma), mixed connective tissue disease (MCTD)
    2. Less commonly associated with acute rheumatic fever, polyarteritis nodosum (PAN), Wegener's granulomatosis
    3. Still's Disease
    4. Sarcoidosis
    5. Loffler's Endomyocarditis
  8. Following Myocardial Injury
    1. Dressler's (Post-Cardiotomy) Syndrome: usually 1-3 weeks after myocardial infarction (MI)
    2. Pericarditis occurs in 5-10% of patients with MI
    3. After open heart surgery, especially post-pericardiectomy, bypass surgery
    4. Post-infectious pericarditis
    5. Fever is very common
  9. Radiation Induced Injury
  10. Miscellaneous
    1. Trauma
    2. Pancreatitis
    3. Aortic Dissection
    4. Whipple's Disease
    5. Inflammatory Bowel Disease
  11. Calcific Constrictive Pericarditis [9]
    1. Previously relatively common, due to tuberculosis infection
    2. Now occurs in ~25% of patients with pericarditis, usually idiopathic
    3. Calcification associated with larger atrial size and atrial arrhythmias
    4. Independent predictor of increased perioperative mortality
  12. Effusive-Constrictive Pericarditis [13]
    1. Uncommon pericardial syndrome
    2. Tense pericardial effusion with constriction by visceral pericardium
    3. May progress to tamponade
    4. May be missed in some patients with tamponade
    5. Pericardiocentesis usually leads to only partial improvement (due to constriction)
    6. Extensive epicardiectomy is procedure of choice in most patients
  13. Pericarditis and myocarditis often coexist, particularly with infectious etiologies
  14. Nearly all entities which cause pericarditis can cause pericardial effusion

B. Characteristicsnavigator

  1. Symptoms depend largely on absence or presence and size of effusion
  2. Chest Pain
    1. Most common presentation
    2. Pain is usually either sharp ("pleuritic"), or dull and oppressive
    3. Typically retrosternal, radiating to L side of neck/back, trapezius or scapula
    4. Position dependent: worse lying back, best standing up or leaning forward
    5. Coughing or deep inspiration increases chest pain
    6. Radiation to L arm, or more squeezing quality of pain are concerning for MI
    7. Lasts hours to days
  3. Recent "viral syndrome" is quite common - fever, cough, myalgias
  4. Other Symptoms
    1. Cough due to bronchus irritation
    2. Hiccups due to phrenic nerve compression
  5. Effusion can cause ventricular dysfunction and failure [15]
    1. Symptoms of congestive heart failure (CHF) can occur
    2. Dyspnea and tachycardia early
    3. Slowly progressive frank CHF symptoms with normal ejection fraction
    4. May be due to effusion and/or progressive constrictive pericarditis
    5. Peripheral edema due to impedence of right heart filling
    6. Late progression to hypotension and cardiogenic shock

C. Physical Examnavigator

  1. Friction Rub
    1. Present in ~85% of cases
    2. Often heard throughout heart beat, throughout in systole and diastole
    3. A scratchy, high-pitched sound - due to inflamed pericardial surfaces rubbing together
    4. May be triphasic
    5. Ventricular systole - loudest
      1. Early diastolic (V filling) - most difficult to hear
      2. Late diastolic (atrial systole) - present in ~70% of cases
    6. Typically, only one or two phases are heard
    7. Audible when patient holds their breath (contrast with pleural rub)
  2. Vital Signs
    1. Critical to complete evaluation - concern is evidence of tamponade
    2. Relative tachycardia (most sensitive)
    3. Hypotension - most concerning for tamponade
    4. Fever may be present
  3. Ewart's Sign: dullness beneath angle of L scapula, suggests pericardial effusion
  4. Effusion (see below)
    1. Pericardial friction rub often heard
    2. Tachycardia
    3. Larger effusions cause reduced venous return and lower cardiac output
    4. Severe: hypotension, distended neck veins, hepatomegaly, edema, ascites

D. Laboratorynavigator

  1. Standard Evaluation for all patients
    1. Blood counts - CBC
    2. Erythrocyte sedimentation rate
    3. Electrolyte (and renal) panel
    4. Evaluate cardiac muscle enzyme levels for myocarditis or infarction
  2. ECG
    1. Changes are typical for subepicardial involvement
    2. relies on segment depression or elevation
    3. ECG segment baseline is the TP segment
    4. Diffuse ST segment elevations, usually with classic J point elevations in nearly all leads
    5. PR interval depression in most leads are characteristic
    6. Leads aVR and V1 nearly always show reverse: ST depression and PR elevation
    7. Ratio of ST segment elevation to T wave amplitude in lead V6 >0.24 highly specific [4]
    8. Low voltage may indicate effusion
    9. Electrical alternans typically with pericardial effusion due to changing position of heart
    10. Q waves absent; T waves inverted even after ST segments normalized
    11. Atrial fibrillation or flutter are common
  3. Echocardiographic Changes
    1. Rarely occur with pure inflammation or small effusions (see below)
    2. Main concerns are septal deviation and chamber collapse
    3. Equalization of pressures indicates an emergency
    4. Fluid surrounding heart should be drained and analyzed
  4. Specific Tests
    1. Rheumatologic Diseases: ANA, RF, consider anti-Scl70 Abs (scleroderma)
    2. Lyme Titers
    3. Pericardial fluid analysis: Gram Stain, AFB, Culture, Chemistry, Cell Counts
    4. All patients should have PPD and controls placed to rule out tuberculosis
  5. Enlargement of Pericardium
    1. Normal parietal and visceral pericardium are 1-2mm thick
    2. MRI or CT can detect thickened (inflamed and/or fibrotic) pericardium
  6. Poor Prognostic Signs
    1. Leukocytosis with left shift
    2. Exposure to systemic anticoagulation
    3. Fever
    4. Signs of myonecrosis / myocarditis

E. Treatment [1,3]navigator

  1. Pain and inflammation
    1. Usually controlled well with NSAIDs (such as ibuprofen 400-800mg po qid)
    2. Aspirin 650mg po tid-qid may be used in patients with history of MI
    3. Indomethacin 25-50mg po tid is effective but poorly tolerated
    4. Colchicine has also been used effectively and is safer than glucocorticoids
    5. Colchicine 0.6mg po bid adjunct to NSAIDs or prednisone can be used
    6. Colchicine 1.0-2.0mg on day 1 then maintenance 0.5-1.0mg/d for 6 months reduced risk of recurrent pericarditis >50% and was well tolerated when added to standard care [10]
    7. Prednisone short course (40mg qd-bid initially with taper) may be required (second step)
    8. Note that tuberculosis should be ruled out if possible before starting glucocorticoids
    9. Glucocorticoids should be reserved for recurrent or severe disease
    10. Intrapericardial instillation of non-absorbable glucocorticoid has been used with good effect
  2. Treat underlying cause
  3. Urokinase may be useful in exudative fibrinous pericarditis
  4. Fibrotic pericardium may best be treated by surgical resection (pericardiectomy)
  5. Recurrence occurs in 15-30% of idiopathic disease

OTHER PERICARDIAL DISEASES

A. Pericardial Effusions [1]navigator
  1. Accumulation of Fluid
    1. Normal pericardial fluid volume 15-35mL
    2. Up to 2 liters can accumulate slowly in normal pericardium without problems
    3. About 40% of patients with symptomatic pericarditis develop effusions [8]
    4. Large, chronic pericardial effusions can be tolerated for years in many patients [8]
  2. Determinants of cardiac compromise with effusion
    1. Total Volume
    2. Rate of accumulation
    3. Pericardial distensibility
  3. Causes of Effusions (overall)
    1. Biopsy of pericardium and culture of fluid are highly diagnostic
    2. Malignancy (23%), Viral Infection (14%), Radiation (14%), CVD (12%), Uremia (12%)
    3. Other infections: Mycobacterial (7%), Mycoplasma (4%), Other Bacterial (2%); ? in 7%
    4. Drug induced effusions should strongly be considered
  4. Hemopericardium
    1. Neoplasm most common - malignant metastases or primary cardiac tumor
    2. Myocardial and great vessel rupture
    3. Tuberculosis [14]
    4. Surgery or Trauma
    5. Drugs
    6. Underlying cause in setting of anti-coagulation
    7. Uremia - coagulopathy may predispose to bleeding
  5. Symptoms of Pericardial Effusion
    1. Shortness of Breath
    2. Dizziness - Hypotension
    3. Fatigue
    4. Pedal Edema
    5. Cough
    6. Symptoms of Tamponade should be evaluated (see below)
  6. Electrocardiographic Changes
    1. Diffuse low voltage
    2. Electrical alternans
    3. PR Depression and ST elevation may be present (as for pericarditis)
  7. Evaluation of Pericardial Fluid
    1. Complete fluid count with cell differential
    2. Lactate dehydrogenase, pH, glucose, protein, consider amylase (pancreatitis)
    3. Viral Titers / Culture - usually in serum
    4. Stains for AFB, bacteria, fungus
    5. Cultures, including mycobacteria and fungus
  8. Routine drainage of large pericardial effusions in asymptomatic patients not useful
  9. Recurrance after therapeutic pericardiocentesis should prompt pericardiectomy [8]

B. Pericardial (Cardiac) Tamponade [6,12] navigator

  1. Life-threatening compression of heart due to pericardial fluid accumulation
  2. May be rapid (acute) or slow (chronic)
  3. Classic Triad of Symptoms
    1. Elevated Jugular Venous Pressure
    2. Low Blood Pressure
    3. Pulsus paradoxus: exaggerated ( >10mm Hg ) fall in systolic blood pressure with inspiration
    4. In practice, these are unreliable indicators of impending cardiac arrest
    5. Note normal systolic pressure decreases slightly with inspiration
  4. Pathophysiology
    1. Stiffness of pericardium and rate of fluid accumulation determine symptoms
    2. Under chronic inflammatory conditions with flexible pericardium, over 2 liters of fluid may accumulate prior to development of life-threatening tamponade
    3. Tamponade leads to inability to fill the cardiac chambers in diastole
    4. Rising intracardiac pressures
    5. Reduction in stroke volume and cardiac output leads to hypotension
  5. Diagnostic Evaluation [6]
    1. Nearly all patients have at least dyspnea, tachycardia, or chest fullness
    2. Pulsus paradoxus (not a paradox, but an exaggeration) is also usually present
    3. Chest Radiograph: increased cardiac silhouette only with >250cc fluid accumulation
    4. Certainty of tamponade diagnosis requires echocardiography or catheterization
    5. Once effusion is documented, follow progression with heart rate and echocardiography
    6. Echocardiographic Changes
    7. RV collapse: >30% free wall inversion during diastole (respiratory flow variation)
      1. LA collapse: LA wall inversion
    8. Catheterization: equalization of chamber pressures, loss of Y descent
  6. Treatment
    1. Drainage of fluid - pericardiocentesis
    2. Treat symptoms as for pericarditis
    3. Pericardiectomy or Window Placement may be required
  7. Pericardiocentsis [12]
    1. Needle drainage of pericardial fluid
    2. Best done with aide of imaging
    3. Paraxiphoid area (just to patient's left of xiphoid process) usually chosen if not imaged
    4. Angle needle 15° bypassing costal margin and aim toward left shoulder
    5. Slowly advance needle until pericardium is reached
    6. Sheethed 16-18 gauge PTFE needle is recommended since core can be removed
    7. Prolonged drainage facilitated by passing guide wire through sheath and then introducing pigtail angiographic catheter

References navigator

  1. Troughton RW, Asher CR, Klein AL. 2004. Lancet. 363(9410):717 abstract
  2. Spodick DH. 2003. JAMA. 289(9):1150 abstract
  3. Lange RA and Hillis LD. 2004. NEJM. 351(21):2195 abstract
  4. Lewis GD, Holmes CB, Holmvang G, Butterton JR. 2007. NEJM. 356(11):1153 (Case Record) abstract
  5. Caro I and Zembowicz A. 2003. NEJM. 348(7):630 (Case Record) abstract
  6. Roy CL, Minor MA, Brookhart MA, Choudhry NK. 2007. JAMA. 297(16):1810 abstract
  7. Nilsson K, Lindquist O, Pahlson C. 1999. Lancet. 354(9185):1169 abstract
  8. Sagrista-Sauleda J, Angel J, Permanyer-Miralda G, Soler-Soler J. 1999. NEJM. 341(27):2054 abstract
  9. Ling LH, Oh JK, Breen JF, et al. 2000. Ann Intern Med. 132(6):444 abstract
  10. Imazio M, Bobbio M, Cecchi E, et al. 2005. Arch Intern Med. 165(17):1987 abstract
  11. Halsell JS, Riddle JR, Atwood JE, et al. 2003. JAMA. 289(24):3283 abstract
  12. Spodick DH. 2003. NEJM. 349(7):684 abstract
  13. Sagrista-Sauleda J, Angel J, Sanchez A, et al. 2004. NEJM. 350(5):469 abstract
  14. Nardell EA, Fan D, Shepard JO, Mark EJ. 2004. NEJM. 351(3):279 (Case Record) abstract
  15. Wang A and Bashore TM. 2004. NEJM. 351(10):1014 (Case Discussion) abstract