ESC Guidline 2015

D IA G N O S IS A N D
M A N A G EM EN T O F
P ER IC A R D IA L D IS EA S E

1.Introduction
Th pericardium is a double walled sac containing

the heart and the root of the great vessel

The pericardial sac has two layers:
a. a serous visceral layer
b. a fibrous parietal layer
It encloses the pericardial cavity which contains
pericardial fluid
Pericardial disease may be either isolated disease
or apart of systemic disease.
The main pericardial syndromes that are
encountered in clinical practice include pericarditis,
pericardial effusion, cardiac tamponade,
constrictive pericarditis and pericardial masses.

2.1 Epidem iology

Dispite the relative high frecuency of pericardial

disease, there are few epidemiological data, especially

frome primary care.
Pericarditis is the most common disease of the
pericardium encountered in clinical practice.
The incidence of acute pericarditis has been reported as
277 cases per 100000 population per year in an italian
urban.
Data collected from a finnish national registry showed a
standardized incidence rate of hospitalization for acute
pericarditis of 3.32 per 100.000 person years.
Man ages 16-65 years were at higher risk of pericarditis
than woman in the general admitted population, with
the highest risk difference among young adult compered
with the overall population.

2.2 Aetiology

3.PericardialSyndrom e
3.1 Acute
Pericarditis
Inflamatiory
pericardial
syndrome with or
without pericardial
effusion
The clinical
diagnosis can be
made with two
following
criteria(Table 4)

3.1.1 Clinical Management and

Therapy
It is not mandatory to seacrh for aetiology in all patient,

especially in countries with a low prevalence of TB.

The major risk factor associated with poor prognosis
after multivariate analysis include high fever(>38 C),
subacute course(symptoms over several days without a
clear acute onset)evidence of large pericardial
effusion(diastolic echo space >20 mm), cardiac
tamponade, and failure to respond within 7 days to
NSAIDs)
Other risk factor include pericarditis associate wth
myocarditis, immunodepression, trauma and oral
anticoagulant therapy.
On this basis a triage for acute pericarditis is proposed.

3.1.1 Clinical Management and

Therapy(cont..)
The choice of drug should besed on the

history of the patient(CI, previous efficacy, or

side effect), the presence of concomitant
disease(favouring aspirin over other NSIDs
when aspirin is already needed as antiplatelet
treatment) and physician expertise.
Colchicine is recommended at low, weightadjusted doses to improve the response to
medical therapy and prevent reccurences.
Corticosteroid should be considered as second
option in patient with contraindication and
failure of aspirin or NSIDs.

3.1.2. Prognosis
Most patient with acute pericarditis have good long-

term prognosis.
Cardiac tamponade rarely occures in patients with
acute idiopathic pericarditis and is more common
in patient with a specific underlying aetiology
such as malignancy,TB or purulentpericarditis.
Contrictive pericarditis may occurs in <1 % of
patients with acute idiopathic pericarditis
The risk of developing constriction can be
clssified as low(<1 %) for idiopathic and
presumed viral pericarditis, Intermediate(2-5%)
for autoimmune,immunemediated and neoplastic
aetiologies and high risk(20-30%) for bacterial
aetiologies, especially TB and purulent
pericarditis

3.2 Incessant and Chronic

Pericarditis
Inccesant is pericarditis lasting for

>4-6 weeks but <3 months without

remission.
Chronic pericartitis is pericarditis
lasting for >3 months.

3.3 Reccurent Pericarditis

Is diagnosesd with documented first episode of

acute pericarditis, a symptom free interval of 4-6

weeks or longer and evidence of subsequent
reccurence of pericarditis.
Diagnosis of reccurent is established according
to the same criteria as those used for acute
pericarditis.
CRP, CT and /or CMR my provide confirmatory
findings to support the diagnosis in atypical or
doubtful cases showing pericardial inflamation
through evidence of oedema and contrast
enhancement of pericardium.

3.3 Reccurent Pericarditis

(therapy)
Reccurent pericarditis therapy should targeted at the

underlaying aetiology.
Asprin or NSAIDs remain the mainstay of therapy.
Colchicine is recommended on top standard anti-inflamatory
therapy.
In cases of incomplete response to aspirin/NSIDs and colchicine,
corticosteroid may be used, but they should be added at low to
moderate doses to aspirin/NSAIDs and clochicine as triple
therapy.
Corticosteroid at low to moderate dose should be avoided if
infection, particularly bacterial and TB cannot be excluded and
should be restriction in patient with specific indication (systemic
inflamatory diseases, post pericardiotomy syndromes,
pregnancy) or NSAIDs contraindication (true allergy, recent
peptic ulcer or gastrointestinal bleeding, oral anticoagulant
therapy when bleeding risk is considered high or unacceptible).
Or intolerance or persintent disease dispite appropriate doses.

Therapeutic algorithym for acute pericarditis and reccurent

pericarditis

Recom m endation for the m anagem ent of

reccurent pericarditis

3.3.2 Prognosis
Severe complication are uncommon

in idiopathic reccurent pericarditis.

Cardiac tamponade is rare and
generallly accurs at the beginning of
the disease.
Constrictive pericarditis has never
been reported in these patients,
dispite numerous reccurences, and
overall risk is lower than that
recorded after a first episode of

3.4 Pericarditis Associated w ith

m ycardialinvolvem ent(M yopericarditis
3.4 1. Definition and diagnosis

The diagnosis of predominant pericarditis

with myocarditis involvement or
myopericarditis can be clinically establish if
patient with definite criteria for acute
pericarditis show elevated biomarcer of
myocardial injury(Trop I or T, CKMB) without
newly developed focal or diffuse impairment of
LV function in echocardiography or CMR.
Difinite confirmation of the presence of
myocarditis will require endomyocardial biopsy.

3.4.2.M anagem ent M yopericarditis

Hospitalization is recommended for diagnosis and

monitoring of patient with myocardial involvement

and differential diagnosis, especially with ACS
In th setting of myopericarditis, management is
simmilar to that recommended for pericarditis
Empirical anti-inflamatory therapies(ie aspirin
1500-3000 mg/day) or NSAIDs(ibuprofen 12002400 mg/day or indomethacin 75-150 mg/day) are
ususally prescribe to control chest pain.
Costicosteroid are prescribes as a second choice in
cases of contraindication, intoleranc3e,failure of
aspirin/NSAIDs.

3.5 PericardialEff
usion

35.1 Clinicalpresentation and diagnosis

PericardialEff
usion

Clinical presentation of PE varies

according to the speed of pericardial

fluid accumulation.
If pericardial fluid is rapidly
accumulating it can lead to cardiac
tamponade, on the other hand, a
slow accumulation of pericardial fluid
allow the collection of large effusion
in day to weeks before a significant
increase in pericardial pressure

35.1 Clinicalpresentation and diagnosis

PericardialEff
usion(cont..)
Classical symptoms include dyspnoea on exertion

progresing to orthopnea,chest pain and /or

fullnes.
Additional symptoms due to local compresion my
include nusea(diaphragm),
dysphagia(oesophagus), hoarseness(reccurent
laryngeal nerve).
Non spesific symptoms include cough,
weakness,fatigue, anorexia and palpitations.
Fever is non spesific sign that may be associated
with pericarditis, either infectious or immune
mediated(ie systemic inflamatory disesase)

35.1 Clinicalpresentation and diagnosis

PericardialEff
usion(cont..)
Physical examination may be absolutly

normal in patient without hemodynamic

compromise.
When tamponade develops, classic signs
include neck vein distention with elevated
JVP , pulsus paradoxus and diminished
heart sound on cardiac auscultation in
cases of moderate to large effusion.
Pericardial friction rub are rarely heard,
they can usually be detected in patient
with concomintant pericarditis

3.5.2 Triage and M anagem ent of

Pericardialeff
usion

3.6 Cardiac Tam ponade

Cardiac tamponade is life threatening, slow or

rapid compression of the heart due to the

pericardial accumulation of fluid,pus,blood clots
or gas as a result of inflamation,trauma,rupture
of the heart or aortic disessection.
Clinical signs in the patients with cardiac
tamponade include
tachycardia,hypotension,pulsus
paradoxus,elevateted JVP,muffled heart sounds,
decrease ECG voltage either electrical alternans
and erlarge cardiac silhouette on chest x ray.

3.7 Constrictive pericarditis

CP can occur after virtually any pericardial disease

procces. The risk of progression is especially

related to aetiology. Low (<1%) in viral and
idiopathic pericarditis, intermediate(2-5%) in
immune-mediated pericarditis and neoplastic
pericardial disease and high(20-30%) in bacterial
pericarditisespecially purulent pericartidits.
CP is characterized by impaired diastolic filling of
the ventricles due to pericardial disease. The
clinical picture is characterized by sign and
symtoms of right heart failure with preserved right
and left ventricular function in the absence of
previous or concomitant myocardial disease.

3.7 Constrictive pericarditis

Patient complain about fatigue,

peripheral oedema,breathlessness, and

abdominal swelling,venous congestion,
hepatimegaly, pleural effusion and
ascites my occur.
Haemodynamic impairment of the
patient can be additionally aggravated
by a systolic dysfunction due to
myocardial fibrosis or athrophy in more
advance cases.

4.M ultim odality

Cardiovascular Im aging and
D iagnostic W ork U p

4.1 M ultim odality Im aging

4.2 G eneralD iagnostic W ork U p

5.Specifi
c Aetiology of
PericardialSyndrom e

5.1 ViralPericarditis
5.1.2. Definition and clinical spectrum

Most cases of acute pericarditis in

deve;oped countries are beased on viral
infection or autoreactive.
Acute viral pericarditis often present as a
self-limited disease with most patient
recovering without complications.
Howeever as a consequence of it cardiac
tamponade,reccurent pericarditis and more
rarely constrictive pericarditis ma also
develop.

5.1 ViralPericarditis
5.1.3. Pathogenesis
Cardiotropic virus can cause
pericardial and myocardial
inflamation via direct cytolytic or
cytotoxic effect(enterovirus0 and/or
via T and /or B cll driven immunemediated mechanism(herpesvirus)

5.1 ViralPericarditis

5.2.1 BacterialPericarditis( Tuberculosis)

5.2.1 Diagnosis
a definite diagnosis of tuberculosis pericarditis is
beased on the presence of tubercle bacilli in the
pericardial fluid or on histological section of the
pericardium, by culture or by PCR(Xpert MTB/RIF)
testing
A probable diagnosis is made when there is proof
of TB elsewhere in a patient with unexplained
pericarditis, alymphoticytic pericardial exudate
with eleveted uIFN-y, ADA, or lysozyme level and
or an appropriate response to antituberculosis
chemotherapy in endemic area.

5.2.1 BacterialPericarditis(Tuberculosis)
5.2.2 Management
a regimen consisting of rifampicin,
isoniazid, pyrazinamide and ethambutol for
at least 2 months followed by isoniazid and
rifampicin(total of 6 months of therapy) is
effective in treating extrapulmonary TB.
Treatment >= 9 months gives no better
results and has disadvantages of increased
cost and increased risk of poor compliance.

5.2.1 BacterialPericarditis(Tuberculosis)

5.2.1 BacterialPericarditis(Tuberculosis)

5.2.2 BacterialPericarditis(Purulent)

5.2.2 BacterialPericarditis(Purulent)

5.3 Pericarditis in RenalFailure

5.4 Pericardialinvolvem ent in System ic

Autoim une and Autoinfl
am atory D isease.
Pericardial involvement in systemic

autoimune disease may be

symtomatic or asymtomatic and
generally reflect the degree of activity
of the underlying disease
Pericardial involvement is common in
SLE,sjogrens syndrome,rheumatoid
arthritis and scleroderma, but also
present in systemic vasculities,behcet
syndrome,sarcoidosis and inflamatory
bowel syndrome.

5.5 Post-cardiac Injury Syndrom es.

The terms post cardiac injury syndromes(PCIS)

is an umbrella term indicating a group of

inflamatory pericarardial syndromes including
post-myocardial infarction pericarditis, postpericardiotomy syndromes (PPS) and posttraumatic pericarditis (iatrogenic or not).
The diagnosis of PCIS may be reached after a
cardiac injury following clinical criteria : 1.
fever without alternative causes,2. pericarditic
or pleuritic chest pain, 3. pericardial or pleural
rubs, 4. evidence of pericardial effusion and
/or 5. pleural effusion with elevated CRP.
At least two of five criteria should be fulfilled.

5.5 Post-cardiac Injury Syndrom es.

5.6 Traum atic pericardialeff

usion and
hem opericardium

5.7 PericardialInvolvem ent in neoplastic

disease
Primary tumours of the pericardium,either

benign(lipomas and fibromas) or

malignant(misetheliomas,angiosarcomas,fibrosarco
mas are very rare.
Misethelioma, the most common malignant tumour
is almoust always incureble.
The most common secondary malignant tumour are
lung cancer, breast cancer, malignant melanoma,
lymphomas and leukemias.
Malignant pericardial effusions may be small,
medium or large with imminent
tamponade(frequent reccurences) or constriction,
they may even be the initial sign of malignant
disease.

5.7 PericardialInvolvem ent in neoplastic

disease

5.8.1 O ther form pericardial

disease(radiation pericarditis)

5.8.2 O ther form pericardial

disease(Chylopericardium )

5.8.3 O ther form pericardialdisease(D rug

related pericarditis and PE)
Pericardial damage has also been

associated with polymer fume inhalation,

foreign antisera, venoms(scorpion
fishting), foreign substance reaction by
direct pericardial application(talc,
magnesium silicate), silicones,
tetracyclines,sclerosants,asbestos and iron
in -thalassemia.
Management is based on discontinuation
of the causative agent and symtomps
treatment.

5.8.4 O ther form pericardialdisease(PE in

m etabolic and endocrine disorders)
The main cause of pericardial

disease in this setting is represented

by hypothyroidism.
PE may occure in 5-30 % of patient
with hypotyroidsm.
It is diagnosed by a high TSH level,
and clinically is characterized by
relative bradycardia and low voltage
in the ECG.

5.8.5 O ther form pericardial

disease(Pericardialinvolvem ent in PAH )
PE in this setting of PAH is common(25-

30%) and typically small in size, but rarely

causes haemodynamic compromise.
PE development in PAH appears to relate
right ventricular failure and a subsequent
increase in right sided filling prasure along
with right atrial hypertension and increase
in the thebesian vein and coronary sinus.
These processes result in increased
filtration and lymphatic obstruction,
resulting pericardial effusion.

5.8.6 O ther form pericardial

disease(Pericardialcysts)
Pericardial cysts are rare mediastinal masses with an

incidence of 1 in 100.000 patients that have been discribed

as diverticulae or cystic formations on chest x ray.
They often found in either one of the cardiophrenic angles.
Cysts do not communicate with the pericardial space,
whereas diverticulae do.
They may be uni or multiloculated.
They are mostly asymtomatic and detected incidentially,
but can also present with chest discomfort,dyspnea, or
palpitations due to cardiac compression.
The first treatment for this condition is percutaneous
aspiration possibly associated with ethanol sclerosis.
If the diagnosis is not completely established by imaging ar
cyst recurs after drainage, surgical resection my be
necessary.

6.Age and G ender issues in

PericardialD isease

6.1 Pediatric Setting

6.2 Pregnancy,lactation and

reproductive issues
The most common form of pericardial

involvelment in pregnancy is hydropericardium,

usually as a benign mild effusion by the third
trimester.
The effusion is usually silent, clinical examination
and ECG are generaly normal, in few case,
slighly elevated blood pressure and/or a specific
ST-T changes have been documented.
The most common disease to require medical
therapy is acute pericarditis.
Classic NSAIDs (ibuprofen and indometacin) may
be considered during the first and second
trimesters, most experts prefer high-dose aspirin.

6.2 Pregnancy,lactation and

reproductive issues(cont..)
After gestational 20 week, all

NSIDs(except aspirin <=100 mg/day)

can caouse constriction of ductus
arterioosus and impair foetal renal
function and they should be withdrawn
in any gestational week 32.
ibuprofen,indometacin,naproxen and
prednisone may be considered in women
who are breastfeeding.
Colchicine is considered contraindicated
during pregnancy and breastfeeding.

6.3 The elderly

No paper has specifically addressed

pericardial disease in elderly, so only epert

opinios exist.
Therapy adherence and compliance my be
problematic in the elderly because of
cognitive impairment, poor vision or
hearing and cost.
Indometacin is not indicated, the
colchicine dose should be halved and
particular care should be taken to evaluate
renal impairment and drug interaction.

7.Interventionaltechniques and
surgery.

7.1 Pericardiocentesis and pericardial

drainage
For pericardial drainage and biopsy,

the surgical approach remain the

gold standart.
Pericardiocintesis must be guided
either by fluoroscopy or
echocardiography under local
anestesia.
Blind procedures must not be used to
avoid the risk of laceration of the
heart or other organs, except in very

7.1 Pericardioscopy
Pericardioscopy permits visualization

of the pericardial sac with its

epicardial and pericardial layers.
Microscopic views show a clustering
of protusions, haemorrhagic areas
and neovascularization in malignant
pericardial effusion which are often
haemorrhagic, in contrast to
radiogenic or viral and autoimune
effusions.

7.3 Pericardialfl
uid analysis,pericardial
and epicardialbiopsy
Serosanguinous or haemorrhagic fluid can

be found in malignant as well as post

pericardiotomy, rheumatic and traumatic
effusions or can be caused by iatrogenic
lesions during pericardiocentesis. But also
in idiophatic and viral form.
In case of sepsis,TB or HIV postive patient,
bacterial culture can be diagnostic.
Fliud citology can seperete malignant
from non-malignant effusions.

7.4 IntrapericardialTreatm ent

In neoplastic pericardial effusion(bronchus carcinoma

or breast cancer) intrapericardial cisplatin or thiotepa

therapy have been proposed in combination with
systemic antineoplastic treatment.
In autoreactive and lymphocytic pericardial effusion
disease specific intrapericardial crystalloid
triamcinolone(300 mg/m2 body surface) may be
considered.
In cases of uremic PE, intrapericardial therapy with
triamcinolone may be considered, apart from
intensified haemo- or peritoneal dyalisis and fluid
evacuation.
In rare case of reccurent PE , balloon pericardiotomy is
an option that allows a pericardio abdominal window
for drainage

7.5 Surgery for pericardialdisease

7.5.1 Pericardial window
a cardiac surgical procedure to create a
communication, or window from the pericardial spce to
the pleural cavity
This is to allow a perricardial effusion( malignant) to
drain frome the spece surrounding the heart into the
chest cavity in order to prevent a large pericardial
effusion and cardiac tamponade.
The main indication is reccurent large effusions or
cardiac tamponade when a more complex operation
such pericardiotomy is high risk or the life expectancy
of the patient is reduce(neoplastic pericardial disease)
and the intervention is palliative.
In reccurent constrictive pericarditis, a repeated
operation has to be done as soon as possible, ideally
during the first post operative year.

7.5 Surgery for pericardialdisease

7.5.2 Pericardiectomy
in constrictive pericarditis the treatment is
pericardiectomy.
The decortications should remove as much
of the pericardium as posible with all
constricting parietal and epicardial layers,
but taking care of perserving the phrenic
nerves bilaterally.
It is also necessary to liberate all of the
right atrium, the superior vena cava and
especially the inferior vena cava and the
inferior part of the right ventricle adjacent
to the diaphragm as far as possible.

TER IM A K S IH