Guidelines on the Diagnosis and Management of Pericardial Diseases Executive Summary

Guidelines and Expert Consensus documents aim to present all the relevant evidence on a particular issue in order to help physicians to weigh the benefits and risks of a particular diagnostic or therapeutic procedure. They should be helpful in everyday clinical decision-making. A great number of Guidelines and Expert Consensus Documents have been issued in recent years by different organisations, the European Society of Cardiology (ESC) and by other related societies. By means of links to web sites of National Societies several hundred guidelines are available. This profusion can put at stake the authority and validity of guidelines, which can only be guaranteed if they have been developed by an unquestionable decision-making process. This is one of the reasons why the ESC and others have issued recommendations for formulating and issuing Guidelines and Expert Consensus Documents. In spite of the fact that standards for issuing good quality Guidelines and Expert Consensus Documents are well defined, recent surveys of Guidelines and Expert Consensus Documents published in peer-reviewed journals between 1985 and 1998 have shown that methodological standards were not complied within the vast majority of cases. It is therefore of great importance that guidelines and recommendations are presented in formats that are easily interpreted. Subsequently, their implementation programmes must also be well conducted. Attempts have been made to determine whether guidelines improve the quality of clinical practice and the utilisation of health resources. The ESC Committee for Practice Guidelines (CPG) supervises and coordinates the preparation of new Guidelines and Expert Consensus Documents produced by Task Forces, expert groups or consensus panels. The Committee is also responsible for the endorsement of these Guidelines and Expert Consensus Documents or statements. The strength of evidence related to a particular diagnostic or treatment option depends on the available data: (1) level of evidence A: multiple randomised clinical trials or meta-analyses; (2) level of evidence B: a single randomised trial or non-randomised studies; and (3) level of evidence C: consensus opinion of the experts. Indications for various tests and procedures were ranked in three classes: Conditions for which there is evidence and/or general agreement that a given procedure or treatment is useful and effective. Conditions for which there is conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of a procedure or treatment. Weight of evidence/opinion is in favour of usefulness/efficacy. Usefulness/efficacy is less well established by evidence/opinion. 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. The spectrum of pericardial diseases consists of congenital defects, pericarditis (dry, effusive, effusive-constrictive, and constrictive), neoplasm, and cysts. The aetiological classification comprises: infectious pericarditis, pericarditis in systemic autoimmune diseases, type 2 (auto) immune process, postmyocardial infarction syndrome, and auto-reactive (chronic) pericarditis (Table 1).1–3 Review of aetiology, incidence and pathogenesis of pericarditis1–3 CHF, congestive heart failure; DDg, differential diagnosis; MI, myocardial infarction; P., pericarditis; PE, pericardial effusion. Percentage related to the population of 260 subsequent patients undergoing pericardiocentesis, pericardioscopy and epicardial biopsy (Marburg pericarditis registry 1988–2001).1 Percentage related to the incidence of pericarditis in the specific population of patients (e.g., with systemic lupus erythematosus). Percentage related to the population of patients with neoplastic pericarditis. Review of aetiology, incidence and pathogenesis of pericarditis1–3 CHF, congestive heart failure; DDg, differential diagnosis; MI, myocardial infarction; P., pericarditis; PE, pericardial effusion. Percentage related to the population of 260 subsequent patients undergoing pericardiocentesis, pericardioscopy and epicardial biopsy (Marburg pericarditis registry 1988–2001).1 Percentage related to the incidence of pericarditis in the specific population of patients (e.g., with systemic lupus erythematosus). Percentage related to the population of patients with neoplastic pericarditis. Congenital defects of the pericardium (1/10.000 autopsies) comprise partial left (70%), right (17%) or total bilateral (rare) pericardial absence. Additional congenital abnormalities occur in ∼30% of patients.4 Most patients with a total pericardial absence are asymptomatic. Homolateral cardiac displacement and augmented heart mobility impose an increased risk for traumatic aortic dissection.5 Partial left side defects can be complicated by herniation and strangulation of the heart through the defect (chest pain, shortness of breath, syncope or sudden death). Surgical pericardioplasty (Dacron, Gore-tex, or bovine pericardium) is indicated for imminent strangulation.6 Acute pericarditis is dry, fibrinous or effusive, independent from its aetiology. The diagnostic algorithm can be derived from Table 2.8–18 A prodrome of fever, malaise, and myalgia is common, but elderly patients may not be febrile. Major symptoms are retrosternal or left precordialchest pain (radiates to the trapezius ridge, can be pleuritic or simulate ischemia, and varies with posture) and shortness of breath. The pericardial friction rub can be transient, mono-, bi- or triphasic. Pleural effusion may be present. Heart rate is usually rapid and regular. Microvoltage and electrical alternans are reversible after effusion drainage.19 Echocardiography is essential to detect effusion, concomitant heart or paracardial disease.11,12 Diagnostic pathway and sequence of performance in acute pericarditis (level of evidence B for all procedures) 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). Cardiac troponin in and \(>\) in of patients with acute pericarditis in with ST in by In troponin in patients with a of to \(>\) in patients with the of to the of of the total Diagnostic pathway and sequence of performance in acute pericarditis (level of evidence B for all procedures) 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). Cardiac troponin in and \(>\) in of patients with acute pericarditis in with ST in by In troponin in patients with a of to \(>\) in patients with the of to the of of the total is by or myocardial elevations of and and of a new heart segment in the of and in are but only biopsy is is to determine the and for as well as the of treatment. are the (level of evidence should be in elderly patients to its in the is for its on the and the large on and may be and can be for or the effusion must be to an or as also to be for the and the of (level of evidence It is well with side should be to diseases, or pericarditis. systemic side and is (level of evidence of or should be Indications for are in patients should be for or \(>\) pericarditis or in heart and are usually (chest pain, related to the of cardiac and pericardial The diagnostic algorithm is similar as in acute pericarditis (Table The of the (e.g., and systemic specific treatment and for are as in acute pericarditis. and or should be (level of evidence The pericarditis (1) the type and (2) the type of a pericardial effusion, or are for an (1) the for (2) the of (3) the to treatment and the and of pericarditis with other autoimmune infarction syndrome, and of were also with and pericarditis with on and the in acute pericarditis. and to of only new the of of the patients The is 2 for one or by (level of evidence should be only in patients with general or in (level of evidence A is to a to be or to the The for at one If patients not or can be should be a If symptoms to the that the that for and the end of the treatment with or treatment should for at three is indicated only in and to treatment (level of evidence the should be on a for several were also to of the effusion may as or are with pericarditis, and that can be can present with are (e.g., pericardial are of all large is the of cardiac by effusion and the increased In is in the of to a is to before cardiac Heart are and occasionally with of can be may present with the of its and In of the the of pericardial effusion may be a or pain, pericardial friction fever, diffuse ST segment is usually with a effusion may QRS and and electrical alternans seen in the absence of In large are as with or pericardial is by within the This is useful for the of The of pericardial can be in the pericardial The of can be (1) in (2) (3) large or very large and of the In the pericardial at the the left to the In large pericardial the heart may within the pericardial and of the of the and aortic large with a or pericardium are after of the are within or to the and may diagnostic of with paracardial left left effusion, left epicardial (best differentiated in and left the pericardium and the may that cardiac may be is as well as in and pericardial and can also be to the and of and pericardial by to be in to of patients with large pericardial effusion cardiac for and acute pericarditis. Diagnostic for cardiac are in Table and classification of pericardial A: B: of and pericardium and of and pericardium effusion \(>\) and of and pericardium with pericardial of and pericardium with large \(>\) Heart of cardiac left left right right is less in patients or in or of of the in the with or after pericardial Heart rate is usually but may be in and in is in and in patients with aortic patients are if they have may be as can be in right and right or in right If after of pericardial effusion does not the should be of cardiac left left right right is less in patients or in or of of the in the with or after pericardial Heart rate is usually but may be in and in is in and in patients with aortic patients are if they have may be as can be in right and right or in right If after of pericardial effusion does not the should be is not the can be made or the are or treatment. and cardiac is an for with and may improve with treatment should be at the aetiology. in pericardial with a in the of neoplastic or Surgical is only in patients with very large effusion in and/or were not pericarditis is a but of the of the to an of the and increased pericardial been an essential diagnostic of pericarditis. in the large from the present in of the patients with pericardial and cardiac procedures are of the which can present in several pericarditis may only in the epicardial in patients with pericarditis is but these patients are not indicated for about and which may be by a there is a between the pericardial and the of In patients and may of the can be by a to myocardial or and can be derived from Table to acute of the right effusion, and The to pericarditis from is the of with or of by and/or but and and biopsy may be helpful as of pericarditis of pericardial with bilateral of the pericardium the with of and of of pericardial with pericardium the left and right of the with of left and of and partial is of pericardial with pericardium the right and left of the with of right and of and partial is and of pericardial with bilateral of the pericardium from the right myocardial by a of of and of of the and of the left is myocardial is and of pericardial with bilateral of the pericardium the right pericardium cannot be from the of right is of pericardial with bilateral of the pericardium from the right myocardial by a of of and of and is with pericardium that and with of Diagnostic in pericarditis left left right right – In and The is at the end of pericarditis is after of In with in does not with in pericarditis. of the pericardium is not always to in of or should not be on the of pericardial is in in is the is with increased or and A with or with of may the In the early stage or in the these may not be present and the rapid of of may be to the may be or complicated by and Diagnostic in pericarditis left left right right – In and The is at the end of pericarditis is after of In with in does not with in pericarditis. of the pericardium is not always to in of or should not be on the of pericardial is in in is the is with increased or and A with or with of may the In the early stage or in the these may not be present and the rapid of of may be to the may be or complicated by and is the only treatment for permanent The are clinical and heart are at the pericardium as as (1) The and (2) to the and right for A of is not systemic If between and or a general of the are present a risk of or myocardial in cases may be a an of or may be left as to for pericarditis a rate of The of cardiac is in only of the The may and of are in can to a Major acute cardiac and Cardiac and at is by the of myocardial or myocardial of patients with myocardial and/or the rate for to cardiac should be by and of and in cases. If for established after to that of the general if clinical symptoms were present for a before a may not a total Congenital pericardial are they may be or with the from comprise as well as and pericardial by pericarditis, and cardiac usually from in the and Most patients are and are on as an usually at the right the patients can also present with or to the of the Echocardiography is but by or is The treatment for congenital and is and If this is not or may be The of is not and of or after with is and pericarditis is the of the abnormalities are to the immune or or Early in pericardial and and immune the and/or cardiac in pericardial may not they as a of to immune of and occasionally can be in the pericardium and for pericarditis of pericarditis the of and pericarditis an increased incidence in and may also present with pericarditis. The of pericarditis is not the of pericardial effusion and/or by or (level of evidence A in is but not diagnostic for pericarditis (level of evidence of pericarditis is to symptoms acute and the In patients with or pericardial effusion and the specific treatment is (1) on and 2 on and (2) B or (3) and at and for of can be to and neoplastic diseases and/or from the and/or from the other and and In the incidence of pericardial effusion is to Cardiac is the treatment with can (best by with paracardial to heart is in large and cardiac is The of is in patients with pericarditis, as an to treatment (level of evidence pericarditis in is (Table but always if rate in patients is to cardiac and It is usually a of an in the by or are pericardial effusion, diseases cardiac and The as an infectious with must be pericardial should and by of the pericardial and (level of evidence of the pericardial with systemic is by to pericardial and of (e.g., is useful but not of the pericardial with or large may the but through is is in patients with and effusion, of and to Surgical is to of the specific of of the specific of In the pericarditis in the developed been seen in patients The rate in acute pericarditis in The clinical is acute pericarditis with or cardiac large pericardial effusion with a symptoms with fever, acute pericarditis, effusive-constrictive, or pericarditis, and pericardial The is made by the of in the pericardial or and/or the of in the can of from only of pericardial and in pericardial effusion are also with a and pericardioscopy and pericardial biopsy have also the diagnostic for biopsy rapid with Pericarditis in a with is of should be The may be in of and in of specific for is also with of and The diagnostic of in pericarditis from to the for the of pericardial specific and of different have been only patients with or very likely pericarditis should be of in pericardial effusion of by treatment not The of A of patients with and that treatment with be with less for or (level of evidence If should be in its This is for and is to in in spite of is indicated (level of evidence is a of pericardial large pericardial in to of have been (1) pericarditis – in of patients with or before been or It from of the and pericardium and with the of \(>\) (2) pericarditis – in to of patients on and occasionally with to and/or of the pericardium between the pericardial and The clinical may and pleuritic pain but patients are asymptomatic. may in large or may be to in heart rate may and to to may the clinical The ECG does not the diffuse wave elevations with other of acute pericarditis to the of the myocardial If the ECG is of acute pericarditis, must be Most patients with pericarditis to or with of pain and pericardial effusion. should be and should be by the usually to of the pericarditis within which does not may be therapeutic in pericarditis to or if cannot be and systemic have is Cardiac and large to must be with (level of evidence should be with of after or for is indicated only in patients to its and pericarditis also been in of within or may be the The of pericarditis is established the (1) increased number of and \(>\) or the of heart in the pericardial (2) in by (3) of in pericardial effusion and in pericardial effusion, and for and other by and/or neoplastic in pericardial effusion and biopsy of and treatment with is with side Pericarditis in systemic autoimmune systemic lupus systemic systemic and syndrome, and treatment of the and are indicated level within to after pericardial or It the infarction syndrome, to be of a process. Unlike infarction syndrome, acutely a and related to of effusion also after heart It is in patients the Cardiac after heart is and may be related to the of pericarditis may also occur after cardiac in patients with early pericardial effusion the in not and of the treatment is as in acute pericarditis or for several or after of or and of are therapeutic in and are very of treatment or is of pericarditis can be an and a pericarditis, by in of myocardial but is from one to several after clinical of myocardial infarction with symptoms and similar to the It does not and can also as an of pericarditis. incidence is and is in patients with but in cases of pericardial after ECG are by myocardial infarction ECG are and infarction to evolve or of T waves myocardial infarction pericardial effusion \(>\) is frequently with and of these patients may treatment is if the is not available or an be an in to for differential and of treatment is which is the of to for 2 to also been risk the infarction can be for symptoms only but myocardial infarction (level of evidence pericardial can be by or and to and may and should be frequently in or after if is available and a left is present. the of the is the of the If are rapid if only the is the of symptoms and the may be for to is in with a (Table pericardial pericardial of the and acute or cardiac may occur A in the treatment of is of the by a is not and very a relevant pericardial right to the of the the may the the not been before the The rate of is to be in the of to and in less of the The incidence of pericardial in left biopsy is cardiac to be accompanied by sudden and to procedure related were in only in a of and in of the patients from the registry of an the right or epicardial may pericarditis with or A right of a usually left is a is the risk of The can lead to myocardial with cardiac pericardial or in the or should be and partial of the heart the and may also occur after In of the effusion can be in of the patients and in of the cases (Table In a clinical of aortic pericardial by or in of patients with type and in type and in type is to the risk of and of the should be level of the pericardium are less the the of the is always The are and may be or large with an imminent or It may be the of patients are asymptomatic. The of pain, is the of and of the are of cardiac The is on the of the within the in of the patients with pericardial effusion is by diseases, pericarditis, or The and may and The of pericardial pericardial or epicardial biopsy are essential for the of pericardial (level of evidence of cardiac is a for The are in neoplastic pericardial effusion (1) systemic treatment as which can in to of (level of evidence (2) to symptoms and (level of evidence (3) of (level of evidence is in all patients with large of the rate of evidence of may be by of or treatment to the type of the that of is in and of in pericardial of pericarditis level of evidence as also the pericardial effusion in of but side and are pain and (level of evidence after of and is an pericarditis to a in of very good is not of the with their (level of evidence is very in pericardial effusion (level of evidence in patients with as and of the heart can and pericarditis by is indicated cannot be (level of evidence The procedure can be in but myocardial and of pericardial the (level of evidence It is with a rate and or is for pericardial or of a which the (level of evidence In large pericardial and to be and pericarditis in patients or in the of The clinical the spectrum of pericardial diseases including pericarditis is to or – and is by and pericardial or in are also helpful in the of treatment with B or B is indicated (level of evidence and can the treatment with (level of evidence with pericarditis in the of not but to given are the of for a of three including should be given for (level of evidence or treatment is indicated for is indicated in pericarditis level The to pericarditis is by the its of and the of the pericarditis may occur the or and years – with of to The effusion may be or on with fibrinous or The symptoms may be by the or the should with by cardiac or if Pericarditis may be or by for diagnostic or if may in to of The is and the years rate is very to myocardial to a between the pericardial and the as a of congenital or as a of and or of the or may the The pericardial is and with a and the of The of the is by its specific between and for the of and or with can not only the of the but also its to the depends on the and the of after or cardiac is by and If of effusion treatment is (level of evidence treatment and is a the of the its and just the is the In the should be to are several and can pericarditis, or (Table lupus and is on the of the and treatment. and pericardial and pericardial effusion in of patients with and In some cases pericarditis may be The of is on of and of the QRS and T wave or in the in the and pericardial effusion in as well as a of effusion and may be with pericardial effusion (level of evidence is evidence that to pericardial a to by the Cardiac is ECG of acute pericarditis in should be from the and seen in in to the increased Most pericardial are as in is may the and is in and can be if and not impose a risk for subsequent pericardial can be by after and is 2 or less in should of and or or other and is in cardiac (level of evidence and indicated in \(>\) in but also in for diagnostic and and of evidence is a and Surgical is in traumatic and by is in the cardiac with ECG ECG from the is not an can be of It is to the in to the acute The been with a with a or the left at a to the This is and the and The to and of If is a of may be that the is A is and after for a It is essential to the of the in at before of the and of is less and can be at the Echocardiography should the the pericardium can be in the or in the pericardial is the of effusion by pericardial to The is in patients