guideline reading diagnosis and management of pericardial disease

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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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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&.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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& ac e a e ca d s( u0e cu oss)


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& 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

guideline reading diagnosis and management of pericardial disease

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