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7/24/2019 Guideline Reading Diagnosis and Management of Pericardial Disease
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D IAG N O SIS AN DM AN AG EM EN T O F
PERICARD IAL D ISEASE
ESC Guidline 2015
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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 "brous parietal layer
#t encloses the pericardial cavity which containspericardial $uid
%ericardial disease may be either isolated diseaseor apart of systemic disease!
The main pericardial syndromes that areencountered in clinical practice include pericarditis&pericardial e'usion& cardiac tamponade&constrictive pericarditis and pericardial masses!
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2.1 Epidem iology
(ispite the relative high frecuency of pericardialdisease& there are few epidemiological data& especiallyfrome primary care!
%ericarditis is the most common disease of thepericardium encountered in clinical practice!
The incidence of acute pericarditis has been reported as2)) cases per 100000 population per year in an italianurban!
(ata collected from a "nnish national registry showed astandardi*ed incidence rate of hospitali*ation for acute
pericarditis of +!+2 per 100!000 person years! ,an ages 1-.-5 years were at higher ris/ of pericarditisthan woman in the general admitted population& withthe highest ris/ di'erence among young adult comperedwith the overall population!
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2.2 Aetiology
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3. Pericardial Syndrom e
3.1 AcutePericarditis
#n$amatiory
pericardialsyndrome with orwithout pericardiale'usion
The clinicaldiagnosis can bemade with two
followingcriteriaTable
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+!1!1 Clinical ,anagement andTherapy
#t is not mandatory to seacrh for aetiology in all patient&especially in countries with a low prevalence of T3!
The ma4or ris/ factor associated with poor prognosis
after multivariate analysis include high fever+6 C&subacute coursesymptoms over several days without aclear acute onsetevidence of large pericardiale'usiondiastolic echo space 20 mm& cardiactamponade& and failure to respond within ) days to
7S8#(s 9ther ris/ factor include pericarditis associate wth
myocarditis& immunodepression& trauma and oralanticoagulant therapy!
9n this basis a triage for acute pericarditis is proposed!
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+!1!1 Clinical ,anagement andTherapycont!!
The choice of drug should besed on thehistory of the patientC#& previous e:cacy& orside e'ect& the presence of concomitantdiseasefavouring aspirin over other 7S#(s
when aspirin is already needed as antiplatelettreatment and physician e;pertise!
Colchicine is recommended at low& weight.ad4usted doses to improve the response to
medical therapy and prevent reccurences! Corticosteroid should be considered as second
option in patient with contraindication andfailure of aspirin or 7S#(s!
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3.1.2. Prognosis ,ost patient with acute pericarditis have good long.
term prognosis! Cardiac tamponade rarely occures in patients with
acute idiopathic pericarditis and is more commonin patient with a specifc underlying aetiologysuch as malignancy,TB or purulentpericarditis.
Contrictive pericarditis may occurs in 1 ! o"patients with acute idiopathic pericarditis
The ris# o" developing constriction can $eclssifed as low%1 !& "or idiopathic andpresumed viral pericarditis, 'ntermediate%2()!&
"or autoimmune,immunemediated and neoplasticaetiologies and high ris#%2*(3*!& "or $acterialaetiologies, especially TB and purulentpericarditis
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3.2 Incessant and Chronic
Pericarditis #nccesant is pericarditis lasting for
.- wee/s but
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3.3 Reccurent Pericarditis
#s diagnosesd with documented "rst episode ofacute pericarditis& a symptom free interval of .-wee/s or longer and evidence of subse=uent
reccurence of pericarditis! (iagnosis of reccurent is established according
to the same criteria as those used for acutepericarditis!
C>%& CT and ?or C,> my provide con"rmatory"ndings to support the diagnosis in atypical ordoubtful cases showing pericardial in$amationthrough evidence of oedema and contrastenhancement of pericardium!
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3.3 Reccurent Pericarditis(therapy) >eccurent pericarditis therapy should targeted at the
underlaying aetiology!
8sprin or 7S8#(s remain the mainstay of therapy!
Colchicine is recommended on top standard anti.in$amatorytherapy!
#n cases of incomplete response to aspirin?7S#(s and colchicine&corticosteroid may be used& but they should be added at low tomoderate doses to aspirin?7S8#(s and clochicine as tripletherapy!
Corticosteroid at low to moderate dose should be avoided ifinfection& particularly bacterial and T3 cannot be e;cluded and
should be restriction in patient with speci"c indication systemicin$amatory diseases& post pericardiotomy syndromes&pregnancy or 7S8#(s contraindication true allergy& recentpeptic ulcer or gastrointestinal bleeding& oral anticoagulanttherapy when bleeding ris/ is considered high or unacceptible!9r intolerance or persintent disease dispite appropriate doses!
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herapeutic algorithym !or acute pericarditis and reccurentpericarditis
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Recom m endation !or the m anagem ent o!reccurent pericarditis
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3.3.2 Prognosis
Severe complication are uncommonin idiopathic reccurent pericarditis!
Cardiac tamponade is rare andgenerallly accurs at the beginning ofthe disease!
Constrictive pericarditis has never
been reported in these patients&dispite numerous reccurences& andoverall ris/ is lower than that
recorded after a "rst episode of
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3." Pericarditis Associated # ithm ycardial in$ol$em ent(% yopericarditis
+! 1! (e"nition and diagnosis
The diagnosis of predominant pericarditiswith myocarditis involvement or
myopericarditis can be clinically establish ifpatient with de"nite criteria for acutepericarditis show elevated biomarcer ofmyocardial in4uryTrop # or T& CA,3 withoutnewly developed focal or di'use impairment ofB function in echocardiography or C,>!
(i"nite con"rmation of the presence ofmyocarditis will re=uire endomyocardial biopsy!
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3.".2. % anagem ent % yopericarditis
Dospitali*ation is recommended for diagnosis andmonitoring of patient with myocardial involvementand di'erential diagnosis& especially with 8CS
#n th setting of myopericarditis& management is
simmilar to that recommended for pericarditis Empirical anti.in$amatory therapiesie aspirin
1500.+000 mg?day or 7S8#(sibuprofen 1200.200 mg?day or indomethacin )5.150 mg?day areususally prescribe to control chest pain!
Costicosteroid are prescribes as a second choice incases of contraindication& intoleranc+e&failure ofaspirin?7S8#(s!
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3.& Pericardial E!!usion
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3&.1 Clinical presentation and diagnosisPericardial E!!usion
Clinical presentation of %E variesaccording to the speed of pericardial$uid accumulation!
#f pericardial $uid is rapidlyaccumulating it can lead to cardiactamponade& on the other hand& a
slow accumulation of pericardial $uidallow the collection of large e'usionin day to wee/s before a signi"cant
increase in pericardial pressure
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3&.1 Clinical presentation and diagnosisPericardial E!!usion(cont..)
Classical symptoms include dyspnoea on e;ertionprogresing to orthopnea&chest pain and ?orfullnes!
8dditional symptoms due to local compresion myinclude nuseadiaphragm&dysphagiaoesophagus& hoarsenessreccurentlaryngeal nerve!
7on spesi"c symptoms include cough&
wea/ness&fatigue& anore;ia and palpitations! ever is non spesi"c sign that may be associated
with pericarditis& either infectious or immunemediatedie systemic in$amatory disesase
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3&.1 Clinical presentation and diagnosisPericardial E!!usion(cont..)
%hysical e;amination may be absolutlynormal in patient without hemodynamiccompromise!
Fhen tamponade develops& classic signsinclude nec/ vein distention with elevated
% & pulsus parado;us and diminishedheart sound on cardiac auscultation in
cases of moderate to large e'usion!%ericardial friction rub are rarely heard&they can usually be detected in patientwith concomintant pericarditis
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3.&.2 riage and % anagem ent o!Pericardial e!!usion
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3.' Cardiac am ponade
Cardiac tamponade is life threatening& slow orrapid compression of the heart due to thepericardial accumulation of $uid&pus&blood clotsor gas as a result of in$amation&trauma&ruptureof the heart or aortic disessection!
Clinical signs in the patients with cardiactamponade includetachycardia&hypotension&pulsus
parado;us&elevateted %&muHed heart sounds&decrease ECG voltage either electrical alternansand erlarge cardiac silhouette on chest ; ray!
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3. Constricti$e pericarditis
C% can occur after virtually any pericardial diseaseprocces! The ris/ of progression is especiallyrelated to aetiology! Bow
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3. Constricti$e pericarditis
%atient complain about fatigue&peripheral oedema&breathlessness& andabdominal swelling&venous congestion&
hepatimegaly& pleural e'usion andascites my occur!
Daemodynamic impairment of the
patient can be additionally aggravatedby a systolic dysfunction due tomyocardial "brosis or athrophy in moreadvance cases!
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". % ultim odality
Cardio$ascular Im aging andiagnostic * or+ ,p
" 1 % lti d lit I i
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".1 % ultim odality Im aging
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".2 -eneral iagnostic * or+ ,p
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&.Speci!ic Aetiology o!
Pericardial Syndrom e
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&.1 iral Pericarditis
5!1!2! (e"nition and clinical spectrum
,ost cases of acute pericarditis indeveIoped countries are beased on viral
infection or autoreactive! 8cute viral pericarditis often present as a
self.limited disease with most patientrecovering without complications!
Doweever as a conse=uence of it cardiactamponade&reccurent pericarditis and morerarely constrictive pericarditis ma alsodevelop!
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&.1 iral Pericarditis
5!1!+! %athogenesis
Cardiotropic virus can causepericardial and myocardialin$amation via direct cytolytic orcytoto;ic e'ectenterovirus0 and?orvia T and ?or 3 cll driven immune.
mediated mechanismherpesvirus
& 1 iralPericarditis
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&.1 iral Pericarditis
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&.2.1 /acterial Pericarditis( u0erculosis)
5!2!1 (iagnosis
a de"nite diagnosis of tuberculosis pericarditis isbeased on the presence of tubercle bacilli in thepericardial $uid or on histological section of thepericardium& by culture or by %C>Jpert ,T3?>#testing
8 probable diagnosis is made when there is proofof T3 elsewhere in a patient with une;plained
pericarditis& alymphoticytic pericardial e;udatewith eleveted u#7.y& 8(8& or lyso*yme level andor an appropriate response to antituberculosischemotherapy in endemic area!
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&.2.1 /acterial Pericarditis(u0erculosis)
5!2!2 ,anagement a regimen consisting of rifampicin&
isonia*id& pyra*inamide and ethambutol forat least 2 months followed by isonia*id and
rifampicintotal of - months of therapy ise'ective in treating e;trapulmonary T3!
Treatment K L months gives no betterresults and has disadvantages of increased
cost and increased ris/ of poor compliance!
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&.2.1 /acterial Pericarditis(u0erculosis)
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& ac e a e ca d s( u0e cu oss)
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&.2.1 /acterial Pericarditis(u0erculosis)
& 2 2 / t i lP i diti(P l t)
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&.2.2 /acterial Pericarditis(Purulent)
& 2 2 /acterialPericarditis(Purulent)
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&.2.2 /acterial Pericarditis(Purulent)
& 3 Pericarditisin Renalailure
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&.3 Pericarditis in Renal ailure
& " Pericardialin$ol$em entin System ic
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&." Pericardial in$ol$em ent in System icAutoim une and Autoin!lam atory isease.
%ericardial involvement in systemicautoimune disease may besymtomatic or asymtomatic andgenerally re$ect the degree of activity
of the underlying disease %ericardial involvement is common in
SBE&s4ogrenMs syndrome&rheumatoid
arthritis and scleroderma& but alsopresent in systemic vasculities&behcetsyndrome&sarcoidosis and in$amatorybowel syndrome!
& & Postcardiac Inury Syndrom es
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&.& Postcardiac Inury Syndrom es.
The terms post cardiac in4ury syndromes%C#Sis an umbrella term indicating a group ofin$amatory pericarardial syndromes includingpost.myocardial infarction pericarditis& post.pericardiotomy syndromes %%S and post.traumatic pericarditis iatrogenic or not!
The diagnosis of %C#S may be reached after acardiac in4ury following clinical criteria 1!fever without alternative causes&2! pericarditicor pleuritic chest pain& +! pericardial or pleuralrubs& ! evidence of pericardial e'usion and?or 5! pleural e'usion with elevated C>%!
8t least two of "ve criteria should be ful"lled!
& & Postcardiac Inury Syndrom es
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&.& Postcardiac Inury Syndrom es.
&.' raum atic pericardiale!!usion and
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&.' raum atic pericardial e!!usion andhem opericardium
&. PericardialIn$ol$em entin neoplastic
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&. Pericardial In$ol$em ent in neoplasticdisease
%rimary tumours of the pericardium&eitherbenignlipomas and "bromas ormalignantmisetheliomas&angiosarcomas&"brosarcomas are very rare!
,isethelioma& the most common malignant tumour
is almoust always incureble! The most common secondary malignant tumour are
lung cancer& breast cancer& malignant melanoma&lymphomas and leu/emias!
,alignant pericardial e'usions may be small&medium or large with imminenttamponadefre=uent reccurences or constriction&they may even be the initial sign of malignantdisease!
&. PericardialIn$ol$em entin neoplastic
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&. Pericardial In$ol$em ent in neoplasticdisease
&.4.1 5ther!orm pericardial
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&.4.1 5ther !orm pericardialdisease(radiation pericarditis)
&.4.2 5ther!orm pericardial
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&.4.2 5ther !orm pericardialdisease(Chylopericardium )
&.4.3 5ther!orm pericardialdisease(rug
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&.4.3 5ther !orm pericardial disease(rugrelated pericarditis and PE)
%ericardial damage has also beenassociated with polymer fume inhalation&foreign antisera& venomsscorpion
"shting& foreign substance reaction bydirect pericardial applicationtalc&magnesium silicate& silicones&tetracyclines&sclerosants&asbestos and iron
in N.thalassemia! ,anagement is based on discontinuation
of the causative agent and symtompstreatment!
&.4." 5ther!orm pericardialdisease(PE in
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&.4." 5ther !orm pericardial disease(PE inm eta0olic and endocrine disorders)
The main cause of pericardialdisease in this setting is representedby hypothyroidism!
%E may occure in 5.+0 @ of patientwith hypotyroidsm!
#t is diagnosed by a high TSD level&
and clinically is characteri*ed byrelative bradycardia and low voltagein the ECG!
&.4.& 5ther !orm pericardial
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& 4 & 5 e o pe ca d adisease(Pericardial in$ol$em ent in PA6)
%E in this setting of %8D is common25.+0@ and typically small in si*e& but rarelycauses haemodynamic compromise!
%E development in %8D appears to relateright ventricular failure and a subse=uentincrease in right sided "lling prasure alongwith right atrial hypertension and increase
in the thebesian vein and coronary sinus! These processes result in increased
"ltration and lymphatic obstruction&resulting pericardial e'usion!
&.4.' 5ther !orm pericardial
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pdisease(Pericardial cysts)
%ericardial cysts are rare mediastinal masses with anincidence of 1 in 100!000 patients that have been discribedas diverticulae or cystic formations on chest ; 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& orpalpitations due to cardiac compression!
The "rst treatment for this condition is percutaneousaspiration possibly associated with ethanol sclerosis!
#f the diagnosis is not completely established by imaging arcyst recurs after drainage& surgical resection my benecessary!
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'.Age and -ender issues in
Pericardial isease
'.1 Pediatric Setting
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g
'.2 Pregnancy7 lactation and
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g yreproducti$e issues
The most common form of pericardialinvolvelment in pregnancy is hydropericardium&usually as a benign mild e'usion by the thirdtrimester!
The e'usion is usually silent& clinical e;amination
and ECG are generaly normal& in few case&slighly elevated blood pressure and?or a speci"cST.T changes have been documented!
The most common disease to re=uire medical
therapy is acute pericarditis! Classic 7S8#(s ibuprofen and indometacin may
be considered during the "rst and secondtrimesters& most e;perts prefer high.dose aspirin!
'.2 Pregnancy7 lactation and
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g yreproducti$e issues(cont..)
8fter gestational 20 wee/& all7S#(se;cept aspirin
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'.3 he elderly
7o paper has speci"cally addressedpericardial disease in elderly& so only epertopinios e;ist!
Therapy adherence and compliance my be
problematic in the elderly because ofcognitive impairment& poor vision orhearing and cost!
#ndometacin is not indicated& the
colchicine dose should be halved andparticular care should be ta/en to evaluate
renal impairment and drug interaction!
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.Inter$entional techni8ues and
surgery.
1 P i di i d i di l
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.1 Pericardiocentesis and pericardialdrainage
or pericardial drainage and biopsy&the surgical approach remain thegold standart!
%ericardiocintesis must be guidedeither by $uoroscopy orechocardiography under local
anestesia! 3lind procedures must not be used to
avoid the ris/ of laceration of theheart or other organs& e;cept in very
1 P i di
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.1 Pericardioscopy
%ericardioscopy permits visuali*ationof the pericardial sac with itsepicardial and pericardial layers!
,icroscopic views show a clustering
of protusions& haemorrhagic areasand neovasculari*ation in malignantpericardial e'usion which are often
haemorrhagic& in contrast toradiogenic or viral and autoimunee'usions!
3 P i di l!lid l i i di l
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.3 Pericardial !luid analysis7 pericardialand epicardial 0iopsy
Serosanguinous or haemorrhagic $uid canbe found in malignant as well as postpericardiotomy& rheumatic and traumatic
e'usions or can be caused by iatrogeniclesions during pericardiocentesis! 3ut alsoin idiophatic and viral form!
#n case of sepsis&T3 or D# postive patient&
bacterial culture can be diagnostic! liud citology can seperete malignant
from non.malignant e'usions!
" I t i di l t t
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." Intrapericardial reatm ent
#n neoplastic pericardial e'usionbronchus carcinoma
or breast cancer intrapericardial cisplatin or thiotepatherapy have been proposed in combination withsystemic antineoplastic treatment!
#n autoreactive and lymphocytic pericardial e'usiondisease speci"c intrapericardial crystalloid
triamcinolone+00 mg?m2 body surface may beconsidered!
#n cases of uremic %E& intrapericardial therapy withtriamcinolone may be considered& apart fromintensi"ed haemo. or peritoneal dyalisis and $uidevacuation!
#n rare case of reccurent %E & balloon pericardiotomy isan option that allows a pericardio abdominal windowfor drainage
& S ! i di ldi
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.& Surgery !or pericardial disease
)!5!1 %ericardial window
a cardiac surgical procedure to create acommunication& or window from the pericardial spce tothe pleural cavity
This is to allow a perricardial e'usion malignant todrain frome the spece surrounding the heart into the
chest cavity in order to prevent a large pericardiale'usion and cardiac tamponade!
The main indication is reccurent large e'usions orcardiac tamponade when a more comple; operationsuch pericardiotomy is high ris/ or the life e;pectancy
of the patient is reduceneoplastic pericardial diseaseand the intervention is palliative!
#n reccurent constrictive pericarditis& a repeatedoperation has to be done as soon as possible& ideallyduring the "rst post operative year!
& S ! i di ldi
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.& Surgery !or pericardial disease
)!5!2 %ericardiectomy
in constrictive pericarditis the treatment ispericardiectomy!
The decortications should remove as much
of the pericardium as posible with allconstricting parietal and epicardial layers&but ta/ing care of perserving the phrenicnerves bilaterally!
#t is also necessary to liberate all of theright atrium& the superior vena cava andespecially the inferior vena cava and theinferior part of the right ventricle ad4acentto the diaphragm as far as possible!
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TERIM AKSIH