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## Complete Summary

#### GUIDELINE TITLE

Guidelines on the diagnosis and management of pericardial diseases. The
task force on the diagnosis and management of pericardial diseases of
the European Society of Cardiology.

#### BIBLIOGRAPHIC SOURCE(S)

Maisch B, Seferovic PM, Ristic AD, Erbel R, Rienmuller R, Adler Y,
Tomkowski WZ, Thiene G, Yacoub MH. Guidelines on the diagnosis and
management of pericardial diseases. Sophia Antipolis (FR): European
Society of Cardiology; 2004. 28 p. \[275 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

### \*\* REGULATORY ALERT \*\*

#### FDA WARNING/REGULATORY ALERT

**Note from the National Guideline Clearinghouse**: This guideline
references a drug(s)/intervention(s) for which important revised
regulatory information has been released.

-   [February 28, 2008, Heparin Sodium
    Injection](http://www.fda.gov/medwatch/safety/2008/safety08.htm#HeparinInj2):
    The U.S. Food and Drug Administration (FDA) informed the public that
    Baxter Healthcare Corporation has voluntarily recalled all of their
    multi-dose and single-use vials of heparin sodium for injection and
    their heparin lock flush solutions. Alternate heparin manufacturers
    are expected to be able to increase heparin production sufficiently
    to supply the U.S. market. There have been reports of serious
    adverse events including allergic or hypersensitivity-type
    reactions, with symptoms of oral swelling, nausea, vomiting,
    sweating, shortness of breath, and cases of severe hypotension.

-   [June 8, 2007, Troponin-I
    Immunoassay](http://www.fda.gov/medwatch/safety/2007/safety07.htm#Architect):
    Class I Recall of all lots of the Architect Stat Troponin-I
    Immunoassay. The assay may report falsely elevated or falsely
    decreased results at and near a low level, which may impact patient
    treatment.

-   [May 2, 2007,
    Colchicine](http://www.fda.gov/medwatch/safety/2007/safety07.htm#Colchicine):
    Immediate drug recall for all strengths, sizes and lots of
    ApothéCure compounded injectable Colchicine sold within the last
    year due to recent deaths associated with the use of the product.

### COMPLETE SUMMARY CONTENT

 \*\* REGULATORY ALERT \*\*\
 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 CONTRAINDICATIONS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Pericardial diseases, including congenital defects, pericarditis (dry,
effusive, effusive-constrictive, and constrictive), neoplasms, and cysts

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation\
Management\
Treatment

#### CLINICAL SPECIALTY

Cardiology\
Family Practice\
Internal Medicine\
Surgery

#### INTENDED USERS

Hospitals\
Physicians

#### GUIDELINE OBJECTIVE(S)

To provide recommendations on the diagnosis and management of patients
with pericardial diseases

#### TARGET POPULATION

Patients with pericardial diseases

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis**

1.  Auscultation

2.  Chest x-ray

3.  Echocardiography including transoesophageal and 2-D Doppler
    echocardiography

4.  Computed tomography (CT) scan

5.  Magnetic resonance imaging (MRI, including spin-echo and cine MRI)

6.  Electrocardiography

7.  Determination of pulsus paradoxus

8.  Blood analyses including erythrocyte sedimentation rate (ESR),
    C-reactive peptide (CRP), lactate dehydrogenase (LDH), leukocytes,
    troponin I, creatine kinase-muscle band (CK-MB)

9.  Pericardioscopy

10. Cardiac catheterisation

11. Angiography

12. Epicardial and pericardial biopsy (including polymerase chain
    reaction, immunocytochemistry, and immunohistochemistry)

13. Serum antibody levels

14. Gram, acid-fast, and fungal staining

15. Cultures of the pericardial and body fluids

**Treatment/Management**

1.  Nonsteroidal anti-inflammatory drugs (NSAID) including ibuprofen

2.  Aspirin

3.  Colchicine

4.  Corticosteroids (including prednisone and triamcinolone)

5.  Azathioprine

6.  Cyclophosphamide

7.  Fluid substitution

8.  Catecholamines

9.  Digitalis

10. Albendazole

11. Percutaneous aspiration and instillation of ethanol or silver
    nitrate

12. Hyperimmunoglobulin

13. Interferon alpha

14. Immunoglobulin

15. Antibiotics including antistaphylococcal antibiotics, gentamycin,
    tetracycline, doxycycline, minocycline, bleomycin, penicillin, and
    sulfonamides

16. Urokinase or streptokinase

17. Antituberculous drugs

18. Peritoneal dialysis

19. Haemodialysis

20. Radionuclides

21. Antifungal agents including fluconazole, ketoconasole, itraconasole,
    amphotericin B, liposomal amphotericin B, and amphotericin B lipid
    complex

22. Systemic antineoplastic treatment including cisplatin and thiotepa

23. Pericardiocentesis

24. Pleuropericardial fenestration

25. Percutaneous balloon pericardiotomy

26. Pericardiectomy including antero-lateral thoracotomy (fifth
    intercostals space) and median sternotomy (faster access to the
    aorta and right atrium for extra-corporeal circulation)

#### MAJOR OUTCOMES CONSIDERED

-   Signs and symptoms of pericardial diseases

-   Cardiac morbidity and mortality

-   Sensitivity and specificity of diagnostic tests

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Hand-searches of Published Literature (Primary Sources)\
Hand-searches of Published Literature (Secondary Sources)\
Searches of Electronic Databases\
Searches of Patient Registry Data\
Searches of Unpublished Data

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Not stated

#### NUMBER OF SOURCE DOCUMENTS

423

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**Levels of Evidence**

**A**: Data derived from multiple randomised clinical trials or
meta-analyses

**B**: Data derived from a single randomised trial or nonrandomised
studies

**C**: Consensus opinion of the experts and/or small studies

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review by a subgroup of the task force, then presentation to
the entire task force to reach consensus.

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

Formulation of drafts in specific areas by a subgroup of the task force,
then presentation to the entire task force to reach consensus.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Indications for various tests and procedures were ranked in three
classes:

**Class I**: Conditions for which there is evidence and/or general
agreement that a given procedure or treatment is useful and effective

**Class II**: Conditions for which there is conflicting evidence and/or
a divergence of opinion about the usefulness/efficacy of a procedure or
treatment

-   **Class IIa**: Weight of evidence/opinion is in favour of
    usefulness/efficacy.

-   **Class IIb**: Usefulness/efficacy is less well established by
    evidence/opinion.

**Class III**: Conditions for which there is evidence and/or general
agreement that the procedure/treatment is not useful/effective and in
some cases may be harmful

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

External Peer Review\
Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

A review coordinator is appointed with the Committee for Practice
Guidelines (CPG). The document is reviewed by the members of the CPG,
the European Society of Cardiology Board Members, and other experts in
the field chosen from joint societies, working groups, and other
sources.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

The class of recommendations (I-III) and level of evidence (A-C) are
defined at the end of the \"Major Recommendations\" field.

**Pericardial syndromes**

**Congenital defects of the pericardium**

Congenital defects of the pericardium (1/10,000 autopsies) comprise
partial left (70%), right (17%), or total bilateral (extremely rare)
pericardial absence. About 30% of patients have additional congenital
abnormalities. Most patients with a total absence of pericardium are
asymptomatic. However, homolateral cardiac displacement and augmented
heart mobility impose an increased risk for traumatic aortic type A
dissection. Partial left side defects can be complicated by cardiac
strangulation caused by herniation of the left atrial appendage, atrium,
or left ventricle through the defect (chest pain, shortness of breath,
syncope, or sudden death). The chest x-ray is typical, but the diagnosis
is confirmed by echocardiography and computed tomography (CT)/magnetic
resonance imaging (MRI). Excision of the atrial appendage and surgical
pericardioplasty (Dacron, Gore-tex, or bovine pericardium) is indicated
for imminent strangulation.

**Acute pericarditis**

Acute pericarditis is either dry, fibrinous, or effusive, independent
from its aetiology (please refer to table 1 of the original guideline
document for information on the aetiology, incidence, and pathogenesis
of pericarditis). A prodrome of fever (usually \<39 degrees C), malaise,
and myalgia is common, but elderly patients may not be febrile. Major
symptoms are retrosternal or left precordial chest pain (radiates to the
trapezius ridge, can be pleuritic or simulate ischaemia, and varies with
posture), nonproductive cough, and shortness of breath. The pericardial
friction rub can be transient, mono-, bi- or triphasic. Pleural effusion
may be present. Pericarditis is often accompanied by some degree of
myocarditis (evidenced by global or regional myocardial dysfunction,
myalgias, or rhabdomyolysis, elevations of troponins I and T, muscle
band \[MB\] creatine-kinase, serum myoglobin levels, and tumour necrosis
factor). Auscultation of a new S3 heart sound, convexly elevated J-ST
segment in the electrocardiogram (ECG), fixation of Indium-111-labelled
antimyosin antibodies, and structural changes in MRI are indicative.
However, only endomyocardial/epimyocardial biopsy findings are
diagnostic.

The diagnostic algorithm can be derived from Table 2 of the original
guideline document (see below). Heart rate is usually rapid and regular.
Microvoltage and electrical alternans are reversible after effusion
drainage. Findings by chest x-ray, CT, and MRI are shown in Table 3 of
the original guideline document. Echocardiography is essential to detect
pericardial effusion and to check for concomitant heart disease or
paracardial pathology.

**Table 2: Diagnostic pathway and sequence of performance in acute
pericarditis (level of evidence B for all procedures)**

  -------------------------------------------------------------------------
  **Technique**         **Characteristic findings**    **Reference**
  --------------------- ------------------------------ --------------------
  *Obligatory (class                                   
  I)*                                                  

  Auscultation          Pericardial rub (mono-, bi-,   Spodick,1997
                        or triphasic)                  

  Electrocardiography   Stage I: anterior and inferior Spodick, 2001
  (ECG)^a^              concave ST segment elevation.  
                        PR segment deviations opposite 
                        to P polarity.\                
                        \                              
                        Early stage II: ST junctions   
                        return to the baseline, PR     
                        deviated.\                     
                        \                              
                        Late stage II: T waves         
                        progressively flatten and      
                        invert\                        
                        \                              
                        Stage III: generalised T wave  
                        inversions\                    
                        \                              
                        Stage IV: ECG returns to       
                        prepericarditis state.         

  Echocardiography      Effusion types B-D (Horowitz)  Levine et al., 1991;
                        (Fig. 1 in original guideline  Chuttani et al, 1991
                        document)\                     
                        \                              
                        Signs of tamponade (see        
                        Section 3.5)                   

  Blood analyses        \(a\) Erythrocyte              Meyers, Meyers, &
                        sedimentation rate (ESR),      Prendergast, 1997
                        c-reactive protein (CRP),      
                        lactate dehydrogenase (LDH),   
                        leukocytes (inflammation       
                        markers)\                      
                        \                              
                        (b) Troponin I, CK-MB (markers 
                        of myocardial lesion)^b^       

  Chest x-ray           Ranging from normal to \"water Eisenberg et al.,
                        bottle\" heart shadow.\        1993
                        \                              
                        Revealing additional           
                        pulmonary/mediastinal          
                        pathology.                     

  *Mandatory in                                        
  tamponade (class I),                                 
  optional in                                          
  large/recurrent                                      
  effusions or if                                      
  previous tests                                       
  inconclusive (class                                  
  IIa) in small                                        
  effusions (class                                     
  IIb)*                                                

  Pericardiocentesis    Polymerase chain reaction      Maisch, Ristić, &
  and drainage          (PCR) and histochemistry for   Pankuweit, 2002;
                        aetiopathogenetic              Maisch et al., 1994;
                        classification of infection or Tsang et al., 2002
                        neoplasia                      

  *Optional or if                                      
  previous tests                                       
  inconclusive (class                                  
  IIa)*                                                

  CT                    Effusions, peri-, and          Chiles et al., 2001
                        epicardium                     

  MRI                   Effusions, peri-, and          Chiles et al., 2001
                        epicardium                     

  Pericardioscopy,      Establishing the specific      Maisch, Ristić, &
  pericardial biopsy    aetiology                      Pankuweit, 2002;
                                                       Maisch et al., 1994;
                                                       Nugue et al., 1996;
                                                       Seferović et al.,
                                                       2003
  -------------------------------------------------------------------------

^a^Typical lead involvement: I, II, aVL, aVF, and V3-V6. The ST segment
is always depressed in aVR, frequently in V1, and occasionally in V2.
Occasionally, stage IV does not occur and there are permanent T wave
inversions and flattenings. If ECG is first recorded in stage III,
pericarditis cannot be differentiated by ECG from diffuse myocardial
injury, \"biventricular strain,\" or myocarditis. ECG in EARLY
REPOLARIZATION is very similar to stage I. Unlike stage I, this ECG does
not acutely evolve and J-point elevations are usually accompanied by a
slur, oscillation, or notch at the end of the QRS just before and
including the J point (best seen with tall R and T waves -- large in
early repolarisation pattern). Pericarditis is likely if in lead V6 the
J point is \>25% of the height of the T wave apex (using the PR segment
as a baseline).

^b^ Cardiac troponin I was detectable in 49% and \>1.5 ng/ml in 22% of
69 patients with acute pericarditis (only in those with ST elevation in
ECG) investigated by Bonnefoy et al. (2000) In another study (Brandt,
Filzmaier, & Hanrath, 2001), troponin I was detected in 10/14 patients
with a median peak concentration of 21.4 mg/mL (range 0.5 to \>50
ng/ml). CK-MB was elevated in 8/14 patients with the median peak of 21
U/L (range 13 to 43), corresponding to the relative index of 10.2% of
the total CK activity.

Hospitalisation is warranted for most patients to determine the
aetiology, observe for tamponade, and start anti-inflammatory and
symptomatic treatment. Nonsteroidal anti-inflammatory drugs (NSAID) are
the mainstay (level of evidence B, class I). Ibuprofen is preferred for
its rare side effects, favourable effect on the coronary flow, and the
large dose range. Depending on severity and response, 300 to 800 mg
every 6 to 8 h may be initially required and can be continued for days
or weeks, best until the effusion has disappeared. Gastrointestinal
protection must be provided in all patients. Colchicine (0.5 mg twice a
day \[bid\]) added to an NSAID or as monotherapy also appears to be
effective for the initial attack and the prevention of recurrences
(level of evidence B, class IIa). It is well tolerated with fewer side
effects than NSAIDs. Systemic corticosteroid therapy should be
restricted to connective tissue diseases, autoreactive or uremic
pericarditis. Intrapericardial application avoids systemic side effects
and is highly effective (level of evidence B, class IIa). For tapering
of prednisone, ibuprofen or colchicine should be introduced early (level
of evidence B, class IIa). Recovered patients should be observed for
recurrences or constriction. If patients require anticoagulants, heparin
is recommended under strict observation. Pericardiocentesis is indicated
for clinical tamponade, high suspicion of purulent or neoplastic
pericarditis (level of evidence B, class I), or for large or symptomatic
effusions despite the medical treatment for more than one week (Please
see below and refer to focus box 1 of the original guideline document
for information on pericardiocentesis).

[Focus box 1: pericardiocentesis]{.underline}

Pericardiocentesis is life saving in cardiac tamponade (level of
evidence B, class I). Aortic dissection is a major contraindication.
Relative contraindications include uncorrected coagulopathy;
anticoagulant therapy; thrombocytopenia \<50,000/mm^3^; and small,
posterior, and loculated effusions. Pericardiocentesis in acute
traumatic haemopericardium and purulent pericarditis is probably less
appropriate than surgical drainage. In effusions causing no haemodynamic
compromise, pericardiocentesis is indicated in effusions \>20 mm in
echocardiography in diastole or for diagnostic purposes if additional
procedures are available (e.g., pericardial fluid and tissue analyses,
pericardioscopy, and epicardial/pericardial biopsy) which could reveal
the etiology of the disease and permit further causative therapy (level
of evidence B, class IIa).

Pericardiocentesis guided by fluoroscopy is performed in the cardiac
catheterisation laboratory with ECG monitoring. Direct ECG monitoring
from the puncturing needle is not an adequate safeguard. Right-heart
catheterization can be performed simultaneously with pericardiocentesis,
allowing monitoring the improvement as the effusion is drained. The
subxiphoid approach has been used most commonly, with a long needle with
a mandrel (Tuohy or thin-walled 18-gauge) directed towards the left
shoulder at a 30 degree angle to the skin. This route is extrapleural
and avoids the coronary, pericardial, and internal mammary arteries. The
operator intermittently attempts to aspirate fluid and injects small
amounts of contrast. If haemorrhagic fluid is freely aspirated, a few
millilitres of contrast medium may be injected under fluoroscopic
observation. The appearance of sluggish layering of contrast medium
inferiorly indicates that the needle is correctly positioned. A soft
J-tip guidewire is introduced and, after dilatation, exchanged for a
multi-holed pigtail catheter. It is prudent to drain the fluid in steps
of less than 1L at a time to avoid the acute right-ventricular
dilatation (\"sudden decompression syndrome\"). It is essential to check
the position of the guidewire in at least two angiographic projections.
If the guidewire was erroneously placed intracardially, this should be
recognized before insertion of the dilator and drainage catheter. If,
despite the caution, the introducer set or the catheter have perforated
the heart and are laying intracardially, the catheter should be secured
and the patient promptly transferred to the cardiac surgery.
Alternatively, a second puncture can be attempted. If successful,
surgery may be avoided using autotransfusion of pericardial blood.

Echocardiographic guidance of pericardiocentesis is technically less
demanding and can be performed in the intensive care unit at the
bedside. Echocardiography should identify the shortest route where the
pericardium can be entered intercostally (usually in the sixth or
seventh rib space in the anterior axillary line). Prolonged pericardial
drainage is performed until the volume of effusion obtained by
intermittent pericardial aspiration (every 4 to 6 h) fall to \<25 mL per
day.

The feasibility is high (93%) in patients with anterior effusion
[\>]{.underline}10 mm, while the rate of success is only 58% with small,
posteriorly located effusions. Fluoroscopic and haemodynamic monitoring
improve feasibility (93.1% vs. 73.3%) in comparison to emergency
pericardial puncture with no imaging control. The tangential approach
using the epicardial halo phenomenon in the lateral view significantly
increased the feasibility of fluoroscopically guided pericardiocentesis
in patients with small effusions (200 to 300 mL) (92.6% vs. 84.9%) and
very small effusions (\<200 mL) (89.3% vs. 76.7%). Pericardiocentesis
with echocardiography guidance was feasible in 96% of loculated
pericardial effusions after cardiac surgery. A rescue pericardiocentesis
guided by echocardiography successfully relieved tamponade after cardiac
perforation in 99% of 88 patients and was the definitive therapy in 82%
of the cases. The most serious complications of pericardiocentesis are
laceration and perforation of the myocardium and the coronary vessels.
In addition, patients can experience air embolism, pneumothorax,
arrhythmias (usually vasovagal bradycardia), and puncture of the
peritoneal cavity or abdominal viscera. Internal mammary artery
fistulas, acute pulmonary oedema, and purulent pericarditis were rarely
reported. The safety was improved with echocardiographic or fluoroscopic
guidance. Recent large echocardiographic series reported an incidence of
major complications of 1.3 to 1.6%. In a large series of
fluoroscopy-guided percutaneous pericardiocenteses, cardiac perforations
occurred in 0.9%, serious arrhythmias in 0.6%, arterial bleeding in
1.1%, pneumothorax in 0.6%, infection in 0.3%, and a major vagal
reaction in 0.3%. Incidence of major complications was further
significantly reduced by utilizing the epicardial halo phenomenon for
fluoroscopic guidance.

**Chronic pericarditis**

Chronic pericardial inflammation (\>3 months) includes effusive,
adhesive, and constrictive forms. It is important to differentiate
chronic inflammatory effusions from noninflammatory hydropericardium
(congestive heart failure). Symptoms are usually mild (chest pain,
palpitations, fatigue), related to the degree of chronic cardiac
compression and residual pericardial inflammation.

The diagnostic algorithm is similar as in acute pericarditis (see above
and Table 2 of the original guideline document). The detection of the
curable causes (e.g., tuberculosis, toxoplasmosis, myxedema, autoimmune,
and systemic diseases) allows specific therapy with high success rate.
Symptomatic treatment is as in acute pericarditis. Intrapericardial
instillation of crystalloid nonabsorbable corticosteroids is highly
efficient in autoreactive forms. Pericardiocentesis is indicated as a
diagnostic and therapeutic procedure. If the recurrences are frequent,
pleuropericardial fenestration and percutaneous balloon pericardiotomy
may be appropriate (level of evidence B, class IIb). For chronic
persistent/recurrent large effusions despite intrapericardial therapy or
balloon pericardiotomy, pericardiectomy should be considered.

**Recurrent pericarditis**

The term recurrent pericarditis encompasses (1) the intermittent type
(widely varying symptom-free interval without therapy) and (2) the
incessant type (discontinuation of anti-inflammatory therapy always
ensures a relapse). Mechanisms suggested to explain recurrence include
(1) insufficient dose or/and insufficient treatment duration of
antiphlogistics or corticoids in an autoimmune pericardial disease, (2)
early corticosteroids treatment causing augmented viral deoxyribonucleic
acid/ribonucleic acid (DNA/RNA) replication in pericardial tissue
leading to increased viral antigen exposure, (3) reinfection, and (4)
exacerbation of the connective tissue disease. Evidence for an
immunopathological process includes (1) the latent period lasting for
months; (2) the presence of anti-heart antibodies; and (3) the quick
response to steroid treatment and the similarity and coexistence of
recurrent pericarditis with other autoimmune conditions (lupus, serum
sickness, polyserositis, postpericardiotomy/postmyocardial infarction
syndrome, celiac disease, dermatitis herpetiformis, frequent
arthralgias, eosinophilia, allergic drug reaction, and history of
allergy). Evidence of a potential underlying genetic disorder in
recurrent pericarditis is rare familial clustering with autosomal
dominant inheritance with incomplete penetrance and sex-linked
inheritance (chronic recurrent pericarditis associated with ocular
hypertension) suggested in two families. Precordial pain, often with a
pleuritic component, is characteristic. Fever, pericardial rub,
dyspnoea, elevated erythrocyte sedimentation rate, and
electrocardiographic changes may also occur. Massive pericardial
effusion, cardiac tamponade, and pericardial constriction are rare.

Symptomatic management relies on exercise restriction and the regimen
used in acute pericarditis. Indomethacin should be avoided in elderly
patients due to its flow reduction in the coronaries. Colchicine
inhibits mitoses in the cell nucleus, binds to tubulin, inhibits various
polymorphonuclear functions, and interferes with transcellular movement
of collagen. It was effective for recurrent pericarditis when NSAIDs and
corticosteroids failed to prevent relapses. During 1,004 months of
colchicine treatment, only 13.7% new recurrences occurred. During the
2,333 months of follow-up, 60.7% of the patients remained
recurrence-free. The recommended dose is two mg/day for one or two days,
followed by one mg/day (level of evidence B, class I). Corticosteroids
should be used only in patients with poor general condition or in
frequent crises (level of evidence C, class IIa). A common mistake is to
use a dose too low to be effective or to taper the dose too rapidly. The
recommended regimen is prednisone 1 to 1.5 mg/kg, for at least one
month. If patients do not respond adequately, azathioprine (75 to 100
mg/day) or cyclophosphamide can be added. Tapering of corticoids should
occur over a three-month period. If symptoms recur during the taper,
return to the last dose that suppressed the manifestations, maintain
that dose for 2 to 3 weeks, and then recommence tapering. Towards the
end of the taper, introduce anti-inflammatory treatment with colchicines
or NSAIDs. Renewed treatment should continue for at least three months.
Pericardiectomy is indicated only in frequent and highly symptomatic
recurrences resistant to medical treatment (level of evidence B, class
IIa). Before pericardiectomy, the patient should be on a steroid-free
regimen for several weeks. Post pericardiectomy recurrences were also
demonstrated, possibly due to incomplete resection of the pericardium.

**Pericardial effusion and cardiac tamponade**

Pericardial effusion may appear as transudate (hydropericardium),
exudate, pyopericardium, or haemopericardium. Large effusions are common
with neoplastic, tuberculous, cholesterol, uremic pericarditis,
myxedema, and parasitoses. Effusions that develop slowly can be
remarkably asymptomatic, while rapidly accumulating smaller effusions
can present with tamponade. Loculated effusions are more common when
scarring has supervened (e.g., postsurgical, posttrauma, postpurulent
pericarditis). Massive chronic pericardial effusions are rare (2 to 3.5%
of all large effusions). Cardiac tamponade is the decompensated phase of
cardiac compression caused by effusion accumulation and the increased
intrapericardial pressure. In \"surgical\" tamponade, intrapericardial
pressure is rising rapidly, in the matter of minutes to hours (i.e.,
haemorrhage), whereas a low-intensity inflammatory process is developing
in days to weeks before cardiac compression occurs (\"medical\"
tamponade). The volume of fluid causing tamponade varies inversely with
both parietal pericardial stiffness and thickness (150 to 2,000 mL). In
local compression, dyspnoea, dysphagia, hoarseness (recurrent laryngeal
nerve), hiccups (phrenic nerve), or nausea (diaphragm) can occur. Heart
sounds are distant. Compression of the base of the lung results in a
dullness under the left scapula (Bamberger--Pins--Ewart\'s sign). In
tamponade, chest discomfort, tachypnea, and dyspnoea on exertion
progress to orthopnoea, cough, and dysphagia, occasionally also with
episodes of unconsciousness. Insidiously developing tamponade may
present with the signs of its complications (renal failure, abdominal
plethora, shock liver and mesenteric ischaemia). In 60% of the patients,
the cause of pericardial effusion may be a known medical condition.
Tamponade without two or more inflammatory signs (typical pain,
pericardial friction rub, fever, diffuse ST segment elevation) is
usually associated with a malignant effusion (likelihood ratio 2.9).
Electrocardiography may demonstrate diminished QRS and T-wave voltages,
PR-segment depression, ST-T changes, bundle branch block, and electrical
alternans (rarely seen in the absence of tamponade). In chest
radiography large effusions are depicted as globular cardiomegaly with
sharp margins (\"water bottle\" silhouette). On well-penetrated lateral
radiographies, or better on cine films, pericardial fluid is suggested
by lucent lines within the cardiopericardial shadow (epicardial halo
sign or various other terms for this phenomenon). Recently, it was
suggested that this sign might be useful for fluoroscopic guidance of
pericardiocentesis. The separation of pericardial layers can be detected
in echocardiography, when the pericardial fluid exceeds 15 to 35 mL
(Please refer to figure 1 of the original guideline document for
information on the Horowitz classification of pericardial effusions).
The size of effusions can be graded as (1) small (echo-free space in
diastole \<10 mm), (2) moderate (at least [\>]{.underline}10 mm
posteriorly), (3) large ([\>]{.underline}20 mm), or (4) very large
([\>]{.underline}20 mm and compression of the heart). In large
pericardial effusions, the heart may move freely within the pericardial
cavity (\"swinging heart\"). This exaggerated motion of the heart
induces \"pseudo\" motions like pseudomitral valve prolapse,
pseudosystolic anterior motion of the mitral valve, paradoxical motion
of the interventricular septum, and midsystolic aortic valve closure.
Importantly, effusion size is an overall predictor of prognosis: large
effusions generally indicate more serious disease. Two-dimensional
echocardiography also provides information about the nature of the
fluid, suggesting the presence of fibrin, clot, tumour, air, and
calcium. Intrapericardial bands, often found after radiation of the
chest, are frequently combined with a thick visceral or parietal
pericardium. Rarely tumour masses, sometimes cauliflower-like, are found
within or adjacent to the pericardium. In parasternal long-axis view,
pericardial fluid reflects at the posterior atrioventricular groove,
while pleural fluid continues under the left atrium, posterior to the
descending aorta. Diagnostic pitfalls are small loculated effusions,
haematoma, cysts, tumours, foramen of Morgagni hernia, hiatus hernia,
lipodystrophia with paracardial fat, inferior left pulmonary vein, left
pleural effusion, mitral annulus calcification, giant left atrium,
epicardial fat (best differentiated in CT), and left ventricular
pseudoaneurysm. Metastatic infiltration of the pericardium may
masquerade pericardial tamponade in echocardiography in patients with no
pericardial effusion. After open-heart surgery, localized effusion at
the posterior wall can be found with complete compression of the right
atrium leading to cardiac tamponade. This may be misinterpreted as
atrial myxoma or other cardiac tumour. When bleeding into the
pericardium occurs and thrombosis develops, the typical echolucent areas
may disappear, so that development of cardiac tamponade may be
overlooked. Transesophageal echocardiography is particularly useful in
postoperative loculated pericardial effusion or intrapericardial clot as
well as in identifying metastases and pericardial thickening. CT,
spin-echo, and cine MRI can also be used to assess the size and extent
of simple and complex pericardial effusions. The effusions measured by
CT or by MRI may tend to be larger than by echocardiography. Up to
one-third of patients with asymptomatic large pericardial chronic
effusion developed unexpected cardiac tamponade. Triggers for tamponade
include hypovolemia, paroxysmal tachyarrhythmia, and intercurrent acute
pericarditis; often no trigger is identifiable. Major diagnostic
findings in cardiac tamponade are noted in Table 4 and Focus box 2 of
the original guideline document.

Pericardiocentesis may not be necessary when the diagnosis can be made
based on other systemic features or the effusions are very small or
resolving under anti-inflammatory treatment. Where doubt remains,
pericardiocentesis, pericardioscopy, and epicardial and pericardial
biopsy (including PCR, immunocytochemistry, and immunohistochemistry)
may be valuable (level of evidence B, class IIa). Please see above and
refer to focus box 1 of the original guideline document for information
on pericardiocentesis. Haemodynamic compromise and cardiac tamponade is
an absolute indication for drainage (class I). Patients with dehydration
and hypovolemia may temporarily improve with intravenous fluids
enhancing ventricular filling. Pericardiocentesis is not applicable in
wounds, ruptured ventricular aneurysm, or dissecting aortic haematoma,
when clotting makes needle evacuation impossible so that surgical
drainage with suppression of bleeding sources is mandatory. Loculated
effusions may require thoracoscopic drainage, subxyphoid window, or open
surgery. All patients should be monitored for postdrainage
decompensation. Whenever possible, treatment should be aimed at the
underlying aetiology rather than the effusion itself. However, even in
idiopathic effusions extended pericardial catheter drainage (3
[+]{.underline} 2 days, range 1 to 13 days) was associated with a trend
to lower recurrence rates (6% vs. 23%) than in those without catheter
drainage during the follow-up of 3.8 [+]{.underline} 4.3 years.
Resistant neoplastic processes require intrapericardial treatment,
percutaneous balloon pericardiotomy, or rarely pericardiectomy. Surgical
approach is recommended only in patients with very large chronic
effusion (with or without symptoms) in whom repeated pericardiocentesis
and/or intrapericardial therapy were not successful.

**Constrictive pericarditis**

Constrictive pericarditis is a rare but severely disabling consequence
of the chronic inflammation of the pericardium, leading to an impaired
filling of the ventricles and reduced ventricular function.
Tuberculosis, mediastinal irradiation, and previous cardiac surgical
procedures are frequent causes of the disease, which can present in
several pathoanatomical forms (please refer to figure 2 of the original
guideline document for information on the pathoanatomical forms of
constrictive pericarditis vs. restrictive cardiomyopathy). Constrictive
pericarditis may rarely develop only in the epicardial layer in patients
with previously removed parietal pericardium. Transient constrictive
pericarditis is rare entity, distinguished by its self-limiting nature.
Patients complain about fatigue, peripheral oedema, breathlessness, and
abdominal swelling, which may be aggravated by a protein-loosing
enteropathy. Typically, there is a long delay between the initial
pericardial inflammation and the onset of constriction. In decompensated
patients, venous congestion, hepatomegaly, pleural effusions, and
ascites may occur. Haemodynamic impairment of the patient can be
additionally aggravated by a systolic dysfunction due to myocardial
fibrosis or atrophy. Clinical, echocardiographic, and haemodynamic
parameters can be derived from Table 5 of the original guideline
document. Differential diagnosis has to include acute dilatation of the
heart, pulmonary embolism, right ventricular infarction, pleural
effusion, chronic obstructive lung diseases, and restrictive
cardiomyopathy. The best way to distinguish constrictive pericarditis
from restrictive cardiomyopathy is the analysis of respiratory changes
with or without changes of preload by Doppler and/or tissue Doppler
echocardiography, but physical findings, ECG, chest radiography, CT and
MRI, haemodynamics, and endomyocardial biopsy may be helpful as well
(Please refer to table 6 of the original guideline document for
information on differential diagnosis: constrictive pericarditis vs.
restrictive cardiomyopathy).

Pericardiectomy is the only treatment for permanent constriction. The
indications are based upon clinical symptoms, echocardiography findings,
CT/MRI, and heart catheterisation. There are two standard approaches,
both aiming at resecting the diseased pericardium as far as possible:
(1) the antero-lateral thoracotomy (fifth intercostal space) and (2)
median sternotomy (faster access to the aorta and right atrium for
extracorporeal circulation). A primary installation of cardiopulmonary
bypass is not recommended, due to the enhanced diffuse bleeding during
dissection of the pericardium, following systemic heparinisation. If
severe calcified adhesions between peri- and epicardium or a general
affection of the epicardium (\"outer porcelain heart\") are present,
surgery carries a high risk of either incomplete success or severe
myocardial damage. An alternative approach in such cases may be a
\"laser shaving\" using an Excimer laser. Areas of strong calcification
or dense scarring may be left as islands to avoid major bleeding.
Pericardiectomy for constrictive pericarditis has a mortality rate of 6
to 12% in the current series. The complete normalization of cardiac
haemodynamics is reported in only 60% of the patients. The deceleration
time (DT) may remain prolonged, and postoperative respiratory variations
of mitral/tricuspid flow are found in 9 to 25%. Left ventricular
ejection fraction increases due to a better ventricular filling, but
consistent changes of the left and right atrial sizes were not reported.
Major complications include acute perioperative cardiac insufficiency
and ventricular wall rupture. Cardiac mortality and morbidity at
pericardiectomy is mainly caused by the presurgically unrecognised
presence of myocardial atrophy or myocardial fibrosis (Please refer to
figure 2 of the original guideline document for information on the
pathoanatomical forms of constrictive pericarditis vs. restrictive
cardiomyopathy). Myocardial atrophy in CT is characterized by (1)
thinning of the interventricular septum and posterolateral wall (\<1
cm), (2) reduction of the wall thickening during the cardiac cycle
(\<40%), and (3) reduction of the left-ventricular muscle mass
(LVMM)/end-diastolic volume (EDV) ratio (\<1). Myocardial fibrosis
should be considered whenever the thickened/calcified pericardium is not
separated from the myocardium by subepicardial fat and when the
myocardial wall is showing wave-like appearance with wall thinning.
Exclusion of patients with extensive myocardial fibrosis and/or atrophy
led to a reduction of the mortality rate for pericardiectomy to 5%.
Postoperative low cardiac output should be treated by fluid substitution
and catecholamines, high doses of digitalis, and intra-aortic balloon
pump in most severe cases. If indication for surgery was established
early, long-term survival after pericardiectomy corresponds to that of
the general population. However, if severe clinical symptoms were
present for a longer period before surgery, even a complete
pericardiectomy may not achieve a total restitution.

**Pericardial cysts**

Congenital pericardial cysts are uncommon and may have both unilocular
and multilocular forms, with the diameter from 1 to 5 cm. Inflammatory
cysts comprise pseudocysts as well as encapsulated and loculated
pericardial effusions, caused by rheumatic pericarditis, bacterial
infection, particularly tuberculosis, trauma, and cardiac surgery.
Echinococcal cysts usually originate from ruptured hydatid cysts in the
liver and lungs. Most patients are asymptomatic and cysts are detected
incidentally on chest roentgenograms as an oval, homogeneous radiodense
lesion, usually at the right cardiophrenic angle. However, the patients
can also present with chest discomfort, dyspnoea, cough, or
palpitations, due to the compression of the heart. Echocardiography is
useful, but additional imaging by CT (density readings) or MRI is often
needed.

The treatment for congenital and inflammatory cysts is percutaneous
aspiration and ethanol sclerosis. If this is not feasible, video
assisted thoracotomy or surgical resection may be necessary. The
surgical excision of echinococcal cysts is not recommended. Percutaneous
aspiration and instillation of ethanol or silver nitrate after
pretreatment with Albendazole (800 mg/day 4 weeks) is safe and
effective.

**Specific forms of pericarditis**

**Viral pericarditis**

Viral pericarditis is the most common infection of the pericardium.
Inflammatory abnormalities are due to direct viral attack, the immune
response (antiviral or anticardiac), or both. Early viral replication in
pericardial and epimyocardial tissue elicits cellular and humoral immune
responses against the virus and/or cardiac tissue. Viral genomic
fragments in pericardial tissue may not necessarily replicate, yet they
serve as a source of antigen to stimulate immune responses. Deposits of
immunoglobulin M (IgM), immunoglobulin G (IgG), and occasionally
immunoglobulin A (IgA), can be found in the pericardium and myocardium
for years. Various viruses cause pericarditis (entero-, echo-, adeno-,
cytomegalo-, Ebstein Barr-, herpes simplex-, influenza, parvo B19,
hepatitis C, human immunodeficiency virus (HIV), etc.). Attacks of
enteroviral pericarditis follow the seasonal epidemics of Coxsackie
virus A+B and Echovirus infections. Cytomegalovirus pericarditis has an
increased incidence in immunocompromised and HIV infected hosts.
Infectious mononucleosis with sore throat, adenopathy, and positive
mumps or Epstein Barr virus (EBV) serology may also present with
pericarditis.

The diagnosis of viral pericarditis is not possible without the
evaluation of pericardial effusion and/or pericardial/epicardial tissue,
preferably by PCR or in-situ hybridisation (level of evidence B, class
IIa) (please refer to focus boxes 3 to 5 of the original guideline
document for information on analyses of pericardial effusion,
pericardioscopy and epicardial/pericardial biopsy, and
epicardial/pericardial tissue analyses). A fourfold rise in serum
antibody levels is suggestive but not diagnostic for viral pericarditis
(level of evidence B, class IIb).

Treatment of viral pericarditis is directed to resolve symptoms (see
acute pericarditis), prevent complications, and eradicate the virus. In
patients with chronic or recurrent symptomatic pericardial effusion and
confirmed viral infection, the following specific treatment is under
investigation: (1) cytomegalovirus (CMV) pericarditis:
hyperimmunoglobulin -- 1 time per day 4 mL/kg on day 0, 4, and 8; 2
mL/kg on day 12 and 16; (2) Coxsackie B pericarditis: Interferon alpha:
2.5 Mio. IU/m^2^ surface area subcutaneously 3x per week; (3) adenovirus
and parvovirus B19 perimyocarditis: immunoglobulin treatment: 10 g
intravenously at day 1 and 3 for 6 to 8 h.

Pericardial manifestation of HIV infection can be due to infective,
noninfective, and neoplastic diseases (Kaposi sarcoma and/or lymphoma).
Infective (myo)pericarditis results from the local HIV infection and/or
from the other viral (cytomegalovirus, herpes simplex), bacterial
(*Staphylococcus aureus*, *Klebsiella pneumoniae*, *Mycobacterium
avium*, and tuberculosis) and fungal coinfections (Cryptococcus
neoformans). In progressive disease the incidence of
echocardiographically detected pericardial effusion is up to 40%.
Cardiac tamponade is rare. During the treatment with retroviral
compounds, lipodystrophy can develop (best demonstrated by MRI) with
intense paracardial fat deposition leading to heart failure. Treatment
is symptomatic, while in large effusions and cardiac tamponade
pericardiocentesis is necessary. The use of corticoid therapy is
contraindicated except in patients with secondary tuberculous
pericarditis, as an adjunct to tuberculostatic treatment (level of
evidence A, class I).

**Bacterial pericarditis**

Purulent pericarditis in adults is rare (please refer to table 7 of the
original guideline document for information on the differential
diagnosis of the specific forms of pericarditis) but always fatal if
untreated. Mortality rate in treated patients is 40%, mostly due to
cardiac tamponade, toxicity, and constriction. It is usually a
complication of an infection originating elsewhere in the body, arising
by contiguous spread or haematogenous dissemination. Predisposing
conditions are preexisting pericardial effusion, immunosuppression,
chronic diseases (alcohol abuse, rheumatoid arthritis, etc), cardiac
surgery, and chest trauma. Rarely, left ventricular pseudoaneurysm may
complicate bacterial pericarditis.

The disease appears as an acute, fulminant infectious illness with short
duration. Percutaneous pericardiocentesis must be promptly performed.
Obtained pericardial fluid should undergo urgent Gram, acid-fast, and
fungal staining, followed by cultures of the pericardial and body fluids
(level of evidence B, class I).

Rinsing of the pericardial cavity, combined with effective systemic
antibiotic therapy, is mandatory (combination of antistaphylococcal
antibiotic and aminoglycoside, followed by tailored antibiotic therapy
according to the results of pericardial fluid and blood cultures).
Intrapericardial instillation of antibiotics (e.g., gentamycin) is
useful but not sufficient. Frequent irrigation of the pericardial cavity
with urokinase or streptokinase using large catheters may liquefy the
purulent exudate, but open surgical drainage through subxiphoid
pericardiotomy is preferable. Pericardiectomy is required in patients
with dense adhesions, loculated and thick purulent effusion, recurrence
of tamponade, persistent infection, and progression to constriction.
Surgical mortality up to 8% was reported for pericardiectomy combined
with antibiotic treatment, but the total mortality is higher.

[Tuberculous (TBC) pericarditis]{.underline}

In the last decade TBC pericarditis in the developed countries has been
primarily seen in immunocompromised patients (acquired immunodeficiency
syndrome \[AIDS\]). The mortality rate in untreated acute effusive TBC
pericarditis approaches 85%. Pericardial constriction occurs in 30 to
50%. The clinical presentation is variable: acute pericarditis with or
without effusion; cardiac tamponade; silent, often large, pericardial
effusion with a relapsing course; toxic symptoms with persistent fever;
acute constrictive pericarditis; subacute constriction;
effusive-constrictive or chronic constrictive pericarditis; and
pericardial calcifications. The diagnosis is made by the identification
of *Mycobacterium tuberculosis* in the pericardial fluid or tissue
and/or the presence of caseous granulomas in the pericardium.
Pericarditis in a patient with proven extracardiac tuberculosis is
strongly suggestive of TBC aetiology (several sputum cultures should be
taken). The tuberculin skin test may be false negative in 25 to 33% of
patients and false positive in 30 to 40% (elderly patients). A more
accurate enzyme-linked immunospot (ELISPOT) test was recently developed,
detecting T-cells specific for *Mycobacterium tuberculosis* antigen.
Perimyocardial TBC involvement is also associated with high titres of
antimyolemmal and antimyosin antibodies in the sera. The diagnostic
yield of pericardiocentesis in TBC pericarditis ranges from 30 to 76%
according to the methods applied for the analyses of pericardial
effusion. Pericardial fluid demonstrates high specific gravity, high
protein levels, and high white-cell count (from 0.7 to 54 x 10^9^/L).
Importantly, PCR can identify DNA of *Mycobacterium tuberculosis*
rapidly from only 1 microliter of pericardial fluid. High adenosine
deaminase activity and interferon gamma concentration in pericardial
effusion are also diagnostic, with a high sensitivity and specificity
(please refer to Focus box 4 of the original guideline document for
information on pericardioscopy and epicardial/pericardial biopsy): Both
pericardioscopy and pericardial biopsy have also improved the diagnostic
accuracy for TBC pericarditis. Pericardial biopsy enables rapid
diagnosis with better sensitivity than pericardiocentesis (100 vs. 33%).

Various antituberculous drug combinations of different lengths (6, 9, 12
months) have been applied. However, only patients with proven or very
likely TBC pericarditis should be treated. Prevention of constriction in
chronic pericardial effusion of undetermined aetiology by \"ex
iuvantibus\" antitubercular treatment was not successful. The use of
steroids remains controversial. A meta- analysis of patients with
effusive and constrictive TBC pericarditis suggested that
tuberculostatic treatment combined with steroids might be associated
with fewer deaths, less frequent need for pericardiocentesis or
pericardiectomy (level of evidence A, class IIa). If given, prednisone
should be administered in relatively high doses (1 to 2 mg/kg per day)
since rifampicin induces its liver metabolism. This dose is maintained
for 5 to 7 days and is progressively reduced to discontinuation in 6 to
8 weeks. If, in spite of combination therapy, constriction develops
pericardiectomy is indicated (level of evidence B, class I).

**Pericarditis in renal failure**

Renal failure is a common cause of pericardial disease, producing large
pericardial effusions in up to 20% of patients. Two forms have been
described: (1) Uremic pericarditis -- in 6 to 10% of patients with
advanced renal failure (acute or chronic) before dialysis has been
instituted or shortly thereafter. It results from inflammation of the
visceral and parietal pericardium and correlates with the degree of
azotemia (the blood urea nitrogen \[BUN\] is usually \>60 mg/dL). (2)
Dialysis-associated pericarditis -- in up to 13% of patients on
maintenance haemodialysis, and occasionally with chronic peritoneal
dialysis due to inadequate dialysis and/or fluid overload. Pathologic
examination of the pericardium shows adhesions between the thickened
pericardial membranes (\"bread and butter\" appearance). The clinical
features may include fever and pleuritic chest pain, but many patients
are asymptomatic. Pericardial rubs may persist even in large effusions
or may be transient. Due to autonomic impairment in uremic patients,
heart rate may remain slow (60 to 80 beats/min) during tamponade,
despite fever and hypotension. Anaemia, due to induced resistance to
erythropoetin may worsen the clinical picture. The ECG does not show the
typical diffuse ST/T wave elevations observed with other causes of acute
pericarditis due to the lack of the myocardial inflammation. If the ECG
is typical of acute pericarditis, intercurrent infection must be
suspected.

Most patients with uremic pericarditis respond rapidly to haemo- or
peritoneal dialysis with resolution of chest pain and pericardial
effusion. To avoid haemopericardium, heparin-free haemodialysis should
be used. Care should be taken since acute fluid removal with
haemodialysis can lead to cardiovascular collapse in patients with
tamponade or pre-tamponade. Hypokalemia and hypophosphatemia should be
prevented by supplementing the dialysis solution when appropriate.
Intensified dialysis usually leads to resolution of the pericarditis
within 1 to 2 weeks. Peritoneal dialysis, which does not require
heparinisation, may be therapeutic in pericarditis resistant to
haemodialysis, or if heparin-free haemodialysis cannot be performed.
NSAIDs and systemic corticosteroids have limited success when intensive
dialysis is ineffective. Cardiac tamponade and large chronic effusions
resistant to dialysis must be treated with pericardiocentesis (level of
evidence B, class IIa). Large, non-resolving symptomatic effusions
should be treated with instillation of intrapericardial corticosteroids
after pericardiocentesis or subxiphoid pericardiotomy (triamcinolone
hexacetonide 50 mg every 6 h for 2 to 3 days). Pericardiectomy is
indicated only in refractory, severely symptomatic patients due to its
potential morbidity and mortality. Within two months after renal
transplantation pericarditis has been reported in 2.4% of patients.
Uraemia or infection (CMV) may be the causes.

**Autoreactive pericarditis and pericardial involvement in systemic
autoimmune diseases**

The diagnosis of autoreactive pericarditis is established using the
following criteria: (1) increased number of lymphocytes and mononuclear
cells \>5,000/mm^3^ (autoreactive lymphocytic), or the presence of
antibodies against heart muscle tissue (antisarcolemmal) in the
pericardial fluid (autoreactive antibody-mediated); (2) signs of
myocarditis on epicardial/endomyocardial biopsies by [\>]{.underline}14
cells/mm^2^; (3) exclusion of active viral infection both in pericardial
effusion and endomyocardial/epimyocardial biopsies (no virus isolation,
no IgM-titer against cardiotropic viruses in pericardial effusion, and
negative PCR for major cardiotropic viruses); (4) tuberculosis,
*Borrelia burgdorferi*, *Chlamydia pneumoniae*, and other bacterial
infection excluded by PCR and/or cultures; (5) neoplastic infiltration
absent in pericardial effusion and biopsy samples; (6) exclusion of
systemic, metabolic disorders, and uraemia. Intrapericardial treatment
with triamcinolone is highly efficient with low incidence of side
effects.

Pericarditis, with or without effusion, is also a component of a
multiserositis in systemic autoimmune diseases: rheumatoid arthritis,
systemic lupus erythematosus (SLE), progressive systemic sclerosis,
polymyositis/ dermatomyositis, mixed connective tissue disease,
seronegative spondyloarthropathies, systemic and hypersensitivity
vasculitides, Behcet syndrome, Wegener granulomatosis, and sarcoidosis.
Intensified treatment of the underlying disease and symptomatic
management are indicated (level of evidence B, class I). Treatment
should focus on pericardial symptoms, management of the pericardial
effusion, and the underlying systemic disease.

**The post-cardiac injury syndrome: postpericardiotomy syndrome**

Post-cardiac injury syndrome develops within days to months after
cardiac, pericardial injury or both. It resembles the post-myocardial
infarction syndrome, both appearing to be variants of a common
immunopathic process. Unlike post-myocardial infarction syndrome,
post-cardiac injury syndrome acutely provokes a greater antiheart
antibody response (antisarcolemmal and antifibrillary), probably related
to more extensive release of antigenic material. Pericardial effusion
also occurs after orthotopic heart transplantation (21%). It is more
frequent in patients receiving aminocaproic acid during the operation.
Cardiac tamponade after open heart surgery is more common following
valve surgery (73%) than coronary artery bypass grafting (CABG) alone
(24%) and may be related to the preoperative use of anticoagulants.
Constrictive pericarditis may also occur after cardiac surgery. Warfarin
administration in patients with early postoperative pericardial effusion
imposes the greatest risk, particularly in those who did not undergo
pericardiocentesis and drainage of the effusion. Symptomatic treatment
is as in acute pericarditis (NSAIDs or colchicine for several weeks or
months, even after disappearance of effusion). Long-term (3 to 6 months)
oral corticoids or preferably pericardiocentesis and intrapericardial
instillation of triamcinolone (300 mg/m^2^) are therapeutic options in
refractory forms. Redo surgery and pericardiectomy are very rarely
needed. Primary prevention of postpericardiotomy syndrome using
short-term perioperative steroid treatment or colchicine is under
investigation.

**Postinfarction pericarditis**

Two forms of postinfarction pericarditis can be distinguished: an
\"early\" form (pericarditis epistenocardica) and a \"delayed\" form
(Dressler\'s syndrome). Epistenocardiac pericarditis, caused by direct
exudation, occurs in 5 to 20% of transmural myocardial infarctions but
is clinically discovered rarely. Dressler\'s syndrome occurs from one
week to several months after clinical onset of myocardial infarction,
with symptoms and manifestations similar to the post-cardiac injury
syndrome. It does not require transmural infarction and can also appear
as an extension of epistenocardiac pericarditis. Its incidence is 0.5 to
5% and is still lower in patients treated with thrombolytics (\<0.5%),
but was more frequent in cases of pericardial bleeding after
antithrombotic treatment. Of note, ECG changes are often overshadowed by
myocardial infarction changes. Stage I ECG changes are uncommon and
suggest \"early\" post-myocardial infarction syndrome whereas failure to
evolve or \"resurrection\" of previously inverted T waves strongly
suggest myocardial infarction pericarditis. Postinfarction pericardial
effusion \>10 mm is most frequently associated with haemopericardium,
and two thirds of these patients may develop tamponade/free wall
rupture. Urgent surgical treatment is life saving. However, if the
immediate surgery is not available or contraindicated pericardiocentesis
and intrapericardial fibrin-glue instillation could be an alternative in
subacute tamponade.

Hospitalisation to observe for tamponade, differential diagnosis, and
adjustments of treatment is needed. Ibuprofen, which increases coronary
flow, is the agent of choice. Aspirin, up to 650 mg every 4 h for 2 to 5
days has also been successfully applied. Other nonsteroidal agents risk
thinning the infarction zone. Corticosteroid therapy can be used for
refractory symptoms only but could delay myocardial infarction healing
(level of evidence B, class IIa).

**Traumatic pericardial effusion and haemopericardium in aortic
dissection**

Direct pericardial injury can be induced by accidents or iatrogenic
wounds. Blood loss, vasoconstriction, and haematothorax leading to
severe hypotension and shock may mask pulses paradoxus. Thoracotomy and
surgical repair should be performed to stabilize the haemodynamics.

Iatrogenic tamponade occurs most frequently in percutaneous mitral
valvuloplasty, during or after transseptal puncture, particularly if no
biplane catheterisation laboratory is available and a small left atrium
is present. Whereas the puncture of the interatrial septum is
asymptomatic, the passage of the free wall induces chest-pain
immediately. If high-pressure containing structures are punctured, rapid
deterioration occurs. However, if only the atrial wall is passed, the
onset of symptoms and the tamponade may be delayed for 4 to 6 h. Rescue
pericardiocentesis is successful in 95 to 100% with a mortality of less
than 1% (please refer to table 8 of the original guideline document for
information on traumatic pericardial effusion).

Dissection of the coronary artery and acute or subacute cardiac
tamponade may occur during percutaneous coronary interventions. A
breakthrough in the treatment of coronary perforation is
membrane-covered graft stents. Perforation of the coronary artery by a
guidewire is not infrequent and causes very rarely a relevant
pericardial haemorrhage.

During right ventricular endomyocardial biopsy due to the low stiffness
of the myocardium, the catheter may pass the myocardium, particularly,
when the bioptome has not been opened before reaching the endocardial
border. The rate of perforation is reported to be in the range of 0.3 to
5%, leading to tamponade and circulatory collapse in less than half of
the cases. The incidence of pericardial haemorrhage in left ventricular
endomyocardial biopsy is lower (0.1 to 3.3%). Frank cardiac perforations
seem to be accompanied by sudden bradycardia and hypotension. Severe
complications, leading to procedure related mortality were reported in
only 0.05% in a worldwide survey of more than 6,000 cases and in none of
the 2,537 patients from the registry of an experienced reference centre.

Pacemaker leads penetrating the right ventricle or epicardial fixed
electrodes may cause pericarditis with tamponade, adhesions, or
constriction. A right bundle brand block instead of a usually induced
left bundle branch block can be a first clue.

Blunt chest trauma is the major risk of car accidents. The deceleration
force can lead to myocardial contusion with intrapericardial
haemorrhage, cardiac rupture, pericardial rupture, or herniation.
Transesophageal echocardiography in the emergency room or immediate CT
should be performed. Indirect injury of the pericardium is more
difficult to detect. Pericardial laceration and partial extrusion of the
heart into the mediastinum and pleural space may also occur after
injury.

In dissection of the ascending aorta, pericardial effusion can be found
in 17 to 45% of the patients and in 48% of the autopsy cases (Please
refer to table 8 of the original guideline document for information on
traumatic pericardial effusion). In a clinical series of aortic
dissection, pericardial tamponade was found by CT, MRI, or
echocardiography in 17 to 33% of patients with type I dissection and 18
to 45% in type II dissection and 6% in type III dissection.
Pericardiocentesis is contraindicated, due to the risk of intensified
bleeding and extension of the dissection. Surgery should be performed
immediately after the diagnosis is established by echocardiography
and/or CT/MRI, even if no coronary angiography or aortography is
available (level of evidence B, class I).

**Neoplastic pericarditis**

Primary tumours of the pericardium are 40 times less common than the
metastatic ones. Mesothelioma, the most common of the primary tumours,
is almost always incurable. Metastatic tumours of the pericardium are
found in 15 to 30% of all autopsies performed in patients with malignant
disease and in 4% in general autopsies. The most common secondary
malignant tumours are lung cancer, breast cancer, malignant melanoma,
lymphomas, and leukemias. Effusions may be small or large with an
imminent tamponade (frequent recurrences) or constriction. It even may
be the initial sign of malignant disease. The majority of patients with
malignant pericardial effusion are asymptomatic, particularly if the
accumulation of pericardial effusion is gradual. The onset of dyspnoea,
cough, chest pain, tachycardia, and jugular venous distension is
observed when the volume of fluid exceeds 500 mL. Orthopnea, weakness,
dysphagia, syncope, palpitations, pericardial friction rub, hiccups,
distant heart sounds, pleural effusion, hepatomegaly, oliguria, and
oedema can also be present. Pulsus paradoxus, hypotension, cardiogenic
shock, and paradoxical movement of the jugular venous pulse are
important signs of cardiac tamponade.

The diagnosis is based on the confirmation of the malignant infiltration
within pericardial sac. Of note, in almost two thirds of the patients
with documented malignancy, pericardial effusion is caused by
non-malignant diseases (e.g., radiation pericarditis) or opportunistic
infections. The chest roentgenogram, CT, and MRI may reveal mediastinal
widening, hilar masses, and pleural effusion. The analyses of
pericardial fluid and pericardial or epicardial biopsy are essential for
the confirmation of malignant pericardial disease (level of evidence B,
class I) (please refer to Focus boxes 3 to 5 of the original guideline
document for information on analyses of pericardial effusion,
pericardioscopy and epicardial/pericardial biopsy, and
epicardial/pericardial tissue analyses).

Treatment of cardiac tamponade is a class I indication for
pericardiocentesis. The following steps are recommended in suspected
neoplastic pericardial effusion without tamponade: (1) systemic
antineoplastic treatment as baseline therapy which can prevent
recurrences in up to 67% of cases (level of evidence B, class I); (2)
pericardiocentesis to relieve symptoms and establish diagnosis (level of
evidence B, class IIa); (3) intrapericardial instillation of
cytostatic/sclerosing agent (level of evidence B, class IIa).
Pericardial drainage is recommended, when technically possible, in all
patients with large effusions because of the high recurrence rate (40 to
70%) (level of evidence B, class I). Prevention of recurrences, may be
achieved by intrapericardial instillation of: sclerosing, cytotoxic
agents, or immunomodulators. Intrapericardial treatment tailored to the
type of the tumour indicate that administration of cisplatin is most
effective in secondary lung cancer and intrapericardial instillation of
thiotepa was more effective in breast cancer pericardial metastases. No
patient showed signs of constrictive pericarditis (for both agents,
level of evidence B, class IIa). Tetracycline as sclerosing agents also
control the malignant pericardial effusion in around 85% of cases, but
side effects and complications are quite frequent: fever (19%), chest
pain (20%), and atrial arrhythmias (10%) (level of evidence B, class
IIb). Although classic sclerotherapy after intrapericardial instillation
of tetracycline, doxycycline, minocycline and bleomycin is an effective
procedure, constrictive pericarditis secondary to fibrosis remains a
severe problem in long-term survivors. Although intrapericardial
administration of radionuclides has yielded very good results, it is not
widely accepted because of the logistic problems connected with their
radioactivity (level of evidence B, class IIa). Radiation therapy is
very effective (93%) in controlling malignant pericardial effusion
(level of evidence B, class IIa) in patients with radiosensitive tumours
such as lymphomas and leukemias. However, radiotherapy of the heart can
cause myocarditis and pericarditis by itself. Subxyphoid pericardiotomy
is indicated when pericardiocentesis cannot be performed (level of
evidence B, class IIb). The procedure can be carried out in local
anaesthesia, but complications include myocardial laceration,
pneumothorax, and mortality. Pleuropericardiotomy allows drainage of
malignant pericardial fluid into the pleural space (level of evidence C,
class IIb). It is associated with a higher complications rate and offers
no advantage over pericardiocentesis or subxyphoid pericardiotomy.
Pericardiectomy is rarely indicated, mainly for pericardial constriction
or complications of previous procedures.

Percutaneous balloon pericardiotomy creates a pleuro-pericardial direct
communication, which allows fluid drainage into the pleural space (level
of evidence B, class IIa). In large malignant pericardial effusions and
recurrent tamponade, it seems to be effective (90 to 97%) and safe but
has the potential risk of dissemination of neoplastic cells.

**Rare forms of pericardial disease**

**Fungal pericarditis**

Fungal pericarditis occurs mainly in immunocompromised patients or in
the course of endemic-acquired fungal infections. The clinical picture
comprises the full spectrum of pericardial diseases, including fungal
myocarditis. Fungal pericarditis is mainly due to endemic fungi
(Histoplasma, Coccidioides), or nonendemic opportunistic fungi (Candida,
Aspergillus, Blastomyces) and semifungi (Nocardia, Actinomyces).
Diagnosis is obtained by staining and culturing pericardial fluid or
tissue. Antifungal antibodies in serum are also helpful in establishing
the diagnosis of fungal infection. Antifungal treatment with
fluconazole, ketoconasole, itraconasole, amphotericin B, liposomal
amphotericin B, or amphotericin B lipid complex is indicated in patients
with documented fungal pericarditis (level of evidence B, class I).
Corticosteroids and NSAIDs can support the treatment with antifungal
drugs (level of evidence C, class IIa). Patients with pericarditis in
the course of histoplasmosis do not need antifungal therapy, but respond
to NSAIDs given during 2 to 12 weeks. Sulfonamides are the drugs of
choice for a nocardiosis infection. Combination of three antibiotics
including penicillin should be given for actinomycosis (level of
evidence C, class I). Pericardiocentesis or surgical treatment is
indicated for haemodynamic impairment. Pericardiectomy is indicated in
fungal constrictive pericarditis (level of evidence C, class I).

**Radiation pericarditis**

The probability to develop radiation-induced pericarditis is influenced
by the applied source, dose, its fractionation, duration, radiation
exposed volume, form of mantel field therapy, and the age of the
patients. Radiation-induced pericarditis may occur already during the
therapy or months and years later -- with latency of up to 15 to 20
years. The effusion may be serous or haemorrhagic, later on with
fibrinous adhesions or constriction, typically without tissue
calcification. The symptoms may be masked by the underlying disease or
the applied chemotherapy. Imaging should start with echocardiography,
followed by cardiac CT or MRI if necessary. Pericarditis without
tamponade may be treated conservatively or by pericardiocentesis for
diagnostic purposes or if haemodynamic compromise/tamponade occurs.
Pericardial constriction may happen in up to 20% of patients, requiring
pericardiectomy. The operative mortality is high (21%) and the
postoperative five years survival rate is very low (1%) mostly due to
myocardial fibrosis.

**Chylopericardium**

Chylopericardium refers to a communication between the pericardial sac
and the thoracic duct, as a result of trauma, congenital anomalies, or
as a complication of open-heart surgery, mediastinal lymphangiomas,
lymphangiomatous hamartomas, lymphangiectasis, and obstruction or
anomalies of the thoracic duct. Infection, tamponade, or constriction
may aggravate the prognosis. The pericardial fluid is sterile,
odourless, and opalescent with a milky white appearance and the
microscopic finding of fat droplets. The chylous nature of the fluid is
confirmed by its alkaline reaction, specific gravity between 1010 and
1021, Sudan III stain for fat, the high concentrations of triglycerides
(5 to 50 g/L) and protein (22 to 60 g/L). Enhanced CT, alone or combined
with lymphography, can identify not only the location of the thoracic
duct but also its lymphatic connection to the pericardium. Treatment
depends on the aetiology and the amount of chylous accumulation.
Chylopericardium after thoracic or cardiac operation without signs of
tamponade is preferably treated by pericardiocentesis and diet (medium
chain triglycerides). If further production of chylous effusion
continues, surgical treatment is mandatory (level of evidence B, class
I). When conservative treatment and pericardiocentesis fail,
pericardio-peritoneal shunting by a pericardial window is a reasonable
option. Alternatively, when the course of the thoracic duct was
precisely identified, its ligation and resection just above the
diaphragm is the most effective treatment. In secondary chylopericardium
the underlying disease (e.g., mediastinal tumour) should be treated.

**Drug- and toxin-related pericarditis**

Pericardial reactions to drugs are rare. However, several medications
and toxic substances can induce pericarditis, tamponade, adhesions,
fibrosis, or constriction (Please refer to table 9 of the original
guideline document for information on drug- and toxin-related
pericardial diseases). Mechanisms include drug induced lupus reactions,
idiosyncrasy, \"serum sickness,\" foreign substance reactions, and
immunopathy. Management is based on discontinuation of the causative
agent and symptomatic treatment.

**Pericardial effusion in thyroid disorders**

Pericardial effusion occurs in 5 to 30% of patients with hypothyroidism.
Fluid accumulates slowly and tamponade occurs rarely. In some cases
cholesterol pericarditis may be observed. The diagnosis of
hypothyroidism is based on serum levels of thyroxin and thyroid
stimulating hormone. Bradycardia, low-voltage of the QRS and T-wave
inversion or flattening in the ECG, cardiomegaly in the roentgenogram,
and pericardial effusion in echocardiography, as well as a history of
radiation-induced thyroid dysfunction, myopathy, ascites, pleural
effusion, and uveal oedema may be observed. Therapy with thyroid hormone
decreases pericardial effusion (level of evidence B, class I).

**Pericardial effusion in pregnancy**

There is no evidence that pregnancy affects susceptibility to
pericardial disease. However, many pregnant women develop a minimal to
moderate clinically silent hydropericardium by the third trimester.
Cardiac compression is rare. ECG changes of acute pericarditis in
pregnancy should be distinguished from the slight ST-segment depressions
and T-wave changes seen in normal pregnancy (please refer to Table 10 of
the original guideline document for information on the diagnostic
approach to pericardial effusion in pregnancy). Occult constriction
becomes manifest in pregnancy due to the increased blood volume. Most
pericardial disorders are managed as in nonpregnant. Caution is
necessary while high-dose aspirin may prematurely close the ductus
arteriosus, and colchicine is contraindicated in pregnancy.
Pericardiotomy and pericardiectomy can be safely performed if necessary
and do not impose a risk for subsequent pregnancies. Foetal pericardial
fluid can be detected by echocardiography after 20 weeks´ gestation and
is normally 2 mm or less in depth. More fluid should raise questions of
hydrops foetalis, Rh disease, hypoalbuminemia, and immunopathy or
maternally transmitted mycoplasmal or other infections, and neoplasia.

**Definitions**

**Class of Recommendations**

**Class I**: Conditions for which there is evidence and/or general
agreement that a given procedure or treatment is useful and effective

**Class II**: Conditions for which there is conflicting evidence and/or
a divergence of opinion about the usefulness/efficacy of a procedure or
treatment

-   **Class IIa**: Weight of evidence/opinion is in favour of
    usefulness/efficacy.

-   **Class IIb**: Usefulness/efficacy is less well established by
    evidence/opinion.

**Class III**: Conditions for which there is evidence and/or general
agreement that the procedure/treatment is not useful/effective and in
some cases may be harmful

**Level of Evidence**

**A**: Multiple randomised clinical trials or meta-analyses

**B**: A single randomised trial or nonrandomised studies

**C**: Consensus opinion of the experts

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### REFERENCES SUPPORTING THE RECOMMENDATIONS

[References open in a new
window](http://www.guideline.gov/summary/select_ref.aspx?doc_id=4960)

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is identified and graded for selected
recommendations (see \"Major Recommendations\").

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Appropriate diagnosis and management of pericardial diseases

#### POTENTIAL HARMS

-   Indomethacin should be avoided in elderly patients due to its flow
    reduction in coronaries.

-   The most serious complications of pericardiocentesis are laceration
    and perforation of the myocardium and the coronary vessels. In
    addition, patients can experience air embolism, pneumothorax,
    arrhythmias (usually vasovagal bradycardia), and puncture of the
    peritoneal cavity or abdominal viscera.

-   If severe calcified adhesions between peri- and epicardium or a
    general affection of the epicardium (\"outer porcelain heart\") are
    present, surgery carries a high risk of either incomplete success or
    severe myocardial damage.

-   Tetracycline as sclerosing agents also control the malignant
    pericardial effusion in around 85% of cases, but side effects and
    complications are quite frequent: fever (19%), chest pain (20%), and
    atrial arrhythmias (10%).

-   Radiation therapy is very effective (93%) in controlling malignant
    pericardial effusion in patients with radiosensitive tumours such as
    lymphomas and leukemias. However, radiotherapy of the heart can
    cause myocarditis and pericarditis by itself.

-   Subxyphoid pericardiotomy is indicated when pericardiocentesis
    cannot be performed. The procedure can be carried out in local
    anaesthesia, but complications include myocardial laceration,
    pneumothorax, and mortality.

-   Occult constriction becomes manifest in pregnancy due to the
    increased blood volume. Most pericardial disorders are managed as in
    nonpregnant. Caution is necessary while high-dose aspirin may
    prematurely close the ductus arteriosus.

### CONTRAINDICATIONS

#### CONTRAINDICATIONS

-   Pericardiocentesis is life saving in cardiac tamponade. Aortic
    dissection is a major contraindication. Relative contraindications
    include uncorrected coagulopathy, anticoagulant therapy,
    thrombocytopenia \<50,000/mm^3^, small, posterior, and loculated
    effusions.

-   Pericardiectomy is contraindicated for myocardial atrophy and global
    form of pericardial constriction with bilateral thickening of the
    pericardium along both ventricles separated from the right
    myocardial wall by a thin layer of subepicardial fat (tube-like
    configuration of both ventricles and enlargement of both atria with
    thinning of the interventricular septum and posterolareral wall of
    the left ventricle below 1 cm suggests myocardial atrophy).

-   Pericardiectomy is contraindicated for perimyocardial fibrosis and
    global form of pericardial constriction with bilateral thickening of
    the pericardium along both ventricles (the right sided thickened
    pericardium cannot be separated from the wave-like thin form of
    right sided ventricular wall suggesting perimyocardial fibrosis).

-   Pericardial manifestation of human immunodeficiency virus (HIV)
    infection can be due to infective, non-infective, and neoplastic
    diseases (Kaposi sarcoma and/or lymphoma). The use of corticoid
    therapy is contraindicated except in patients with secondary
    tuberculous pericarditis, as an adjunct to tuberculostatic
    treatment.

-   In a clinical series of aortic dissection, pericardial tamponade was
    found by computed tomography (CT), magnetic resonance imaging (MRI),
    or echocardiography in 17 to 33% of patients with type I dissection
    and 18 to 45% in type II dissection and 6% in type III dissection.
    Pericardiocentesis is contraindicated, due to the risk of
    intensified bleeding and extension of the dissection.

-   Colchicine is contraindicated in pregnancy.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Personal Digital Assistant (PDA) Downloads\
Pocket Guide/Reference Cards\
Slide Presentation

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Maisch B, Seferovic PM, Ristic AD, Erbel R, Rienmuller R, Adler Y,
Tomkowski WZ, Thiene G, Yacoub MH. Guidelines on the diagnosis and
management of pericardial diseases. Sophia Antipolis (FR): European
Society of Cardiology; 2004. 28 p. \[275 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2004 Jan

#### GUIDELINE DEVELOPER(S)

European Society of Cardiology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

European Society of Cardiology (ESC). The Task Force on the Diagnosis
and Management of Pericardial Diseases was supported financially in its
entirety by The European Society of Cardiology and was developed without
any involvement of the pharmaceutical industry.

#### GUIDELINE COMMITTEE

Task Force on the Diagnosis and Management of Pericardial Diseases of
the European Society of Cardiology (ESC)

ESC Committee for Practice Guidelines

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Task Force Members*: Bernhard Maisch, (Chairperson) (Germany); Petar M.
Seferović (Serbia and Montenegro); Arsen D. Ristić (Serbia and
Montenegro); Raimund Erbel (Germany); Reiner Rienmuller (Austria);
Yehuda Adler (Israel); Witold Z. Tomkowski (Poland); Gaetano Thiene
(Italy); Magdi H. Yacoub (UK)

*European Society of Cardiology Committee for Practice Guidelines*:
Silvia G. Priori (Chairperson) (Italy); Maria Angeles Alonso Garcia
(Spain); Jean-Jacques Blanc (France); Andrzej Budaj (Poland); Martin
Cowie (UK); Veronica Dean (France); Jaap Deckers (The Netherlands);
Enrique Fernandez Burgos (Spain); John Lekakis (Greece); Bertil Lindahl
(Sweden); Gianfranco Mazzotta (Italy); Joao Morais (Portugal); Ali Oto
(Turkey); Otto A. Smiseth (Norway)

*Document Reviewers*: Gianfranco Mazzotta, CPG Review Coordinator
(Italy); Jean Acar (France); Eloisa Arbustini (Italy); Anton E. Becker
(The Netherlands); Giacomo Chiaranda (Italy); Yonathan Hasin (Israel);
Rolf Jenni (Switzerland); Werner Klein (Austria); Irene Lang (Austria);
Thomas F. Luscher (Switzerland); Fausto J. Pinto (Portugal); Ralph
Shabetai (USA); Maarten L. Simoons (The Netherlands); Jordi Soler Soler
(Spain); David H. Spodick (USA)

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

The Task Force makes every effort to avoid any actual or potential
conflicts of interest that might arise as a result of an outside
relationship or personal interest of a member of the writing panel.
Specifically, all members of the writing panel, before final approval by
the Committee for Practice Guidelines (CPG), are asked to provide
disclosure statements of all such relationships that might be perceived
as real or potential conflicts of interest. Once they have verbally
accepted to become members of the Task Force, a written consent form is
signed as well as this \"Disclosure form\" and this for all Guidelines.
The disclosure form must be updated if any changes occur during the
elaboration to the document.

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available from the [European Society of Cardiology
(ESC) Web
site](http://www.escardio.org/guidelines-surveys/esc-guidelines/Pages/pericardial-diseases.aspx).

Print copies: Available from Elsevier Publishers Ltd., 32 Jamestown
Road, London, NW1 7BY, United Kingdom. Tel: +44 207 424 4422; Fax: +44
207 424 4433; E-mail: <gr.davies@elsevier.com>

#### AVAILABILITY OF COMPANION DOCUMENTS

The following is available:

-   Guidelines on the diagnosis and management of pericardial diseases.
    Executive summary. 2004. Electronic copies: Available in Portable
    Document Format (PDF) from the [European Society of Cardiology (ESC)
    Web
    site](http://www.escardio.org/guidelines-surveys/esc-guidelines/GuidelinesDocuments/guidelines-pericardial-ES.pdf).

-   Diagnosis and management of pericardial disease. Pocket guidelines.
    Available from the [ESC Web
    site](http://www.escardio.org/guidelines-surveys/esc-guidelines/GuidelinesDocuments/PERICA-pocket.aspx).
    Also available for PDA download from the [ESC Web
    site](http://pocketgram.net/escardio/).

-   Diagnosis and management of pericardial disease. Slide set.
    Available from the [ESC Web
    site](http://www.escardio.org/guidelines-surveys/esc-guidelines/GuidelinesDocuments/guidelines-pericardial-slides.pdf).

Print copies: Available from Elsevier Publishers Ltd., 32 Jamestown
Road, London, NW1 7BY, United Kingdom. Tel: +44 207 424 4422; Fax: +44
207 424 4433; E-mail: <gr.davies@elsevier.com>

#### PATIENT RESOURCES

None available

#### NGC STATUS

This NGC summary was completed by ECRI on July 26, 2004. The information
was verified by the guideline developer on September 24, 2004. This
summary was updated on May 3, 2005 following the withdrawal of Bextra
(valdecoxib) from the market and the release of heightened warnings for
Celebrex (celecoxib) and other nonselective nonsteroidal
anti-inflammatory drugs (NSAIDs). This summary was updated by ECRI on
June 16, 2005, following the U.S. Food and Drug Administration advisory
on COX-2 selective and non-selective non-steroidal anti-inflammatory
drugs (NSAIDs). This summary was updated by ECRI Institute on May 17,
2007 following the U.S. Food and Drug advisory on Colchicine. This
summary was updated by ECRI Institute on June 22, 2007 following the
U.S. Food and Drug Administration (FDA) advisory on heparin sodium
injection. This summary was updated by ECRI Institute on July 12, 2007
following the U.S. Food and Drug Administration (FDA) advisory on
Troponin-1 Immunoassay. This summary was updated by ECRI Institute on
March 13, 2008 following the updated FDA advisory on heparin sodium
injection.

#### COPYRIGHT STATEMENT

This summary is based on the original guideline, which is subject to the
guideline developer\'s copyright restrictions.

### DISCLAIMER

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