Pericarditis resident survival guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mugilan Poongkunran M.B.B.S [2]

Pericarditis Resident Survival Guide Microchapters
Overview
Causes
Diagnosis
Treatment Acute Pericarditis Recurrent Pericarditis Etiology Specific
Do's
Don'ts

Synonyms and keywords: Myopericarditis, perimyocarditis

Overview

Pericarditis is the inflammation of the fibroelastic sac surrounding the heart (pericardium). Pericarditis is divided into acute (<6 weeks), subacute (6 weeks to 6 months) and chronic (>6 months) and it can be dry, fibrinous or effusive independently of the etiology. Myopericarditis, or perimyocarditis refers to acute pericarditis associated with myocardial inflammation that leads to global or regional myocardial dysfunction and elevation in the concentration of troponins, creatine kinase MB, myoglobin and tumour necrosis factor. Always suspect pericarditis in the presence of pleuritic chest pain and pericardial friction rub. NSAIDs are the mainstay of the treatment of acute pericarditis; ibuprofen is the most preferred drug due to its favorable effect on the coronary flow.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

• Aortic dissection
• Myocardial infarction
• Uremia

Common Causes

• Autoimmune: Rheumatoid arthritis, Sjögren’s syndrome, SLE, systemic sclerosis, systemic vasculitis
• Bacterial: Coxiella burnetii, pneumococcus, staphylococcus, streptococcus, tuberculosis
• Cardiovascular: Dressler's syndrome, postpericardiotomy syndrome, post-traumatic pericarditis
• Idiopathic
• Metabolic: Myxedema, uremia
• Neoplastic: Breast cancer, leukemia, lung cancer, lymphoma
• Viral: Adenovirus, CMV, coxsackie, EBV, echovirus, HBV, HIV, human herpes virus 6, influenza, mumps, parvovirus B19, rubella, varicella

Diagnosis

Shown below is an algorithm summarizing the diagnostic approach to acute pericarditis in adults.

Click on boxes to expand/collapse detailed information.               Characterize the symptoms                                                                               Obtain a detailed history                                                                               Examine the patient                                                                               Order tests ❑ Laboratory tets ❑ ECG ❑ Chest X-ray ❑ Echocardiography                                                                           Does the patient have at least two of the following criteria for the diagnosis of acute pericarditis? ❑ Characteristic chest pain ❑ Pericardial friction rub ❑ Suggestive ECG changes ❑ Suggestive echocardiography changes                                                                                             Yes                   No                                                             Does the patient have any sign of myocarditis?                   Does the patient have any signs suspicious of acute pericarditis?                                                                                                   No         Yes     No         Yes                                                           Acute pericarditis       Myopericarditis       Consider alternative diagnosis and treat accordingly       Consider cardiac MRI (CMR)                                                                                           Treat as acute pericarditis or myopericarditis if there is delayed enhancement on CMR

Characterize the symptoms ❑ Chest pain ❑ Sudden onset ❑ Sharp or dull, aching and pressure like ❑ Pleuritic (exacerbated by inspiration and coughing) ❑ Retrosternal ❑ Radiation to neck, arms, trapezius muscle ridges ❑ Affected by position (improved by sitting up and leaning forward) ❑ No pain (uremia and tuberculosis pericarditis develop slowly) Symptoms associated with pericardial effusion: ❑ Without a hemodynamically significant pericardial effusion ❑ No specific symptoms ❑ With a hemodynamically significant pericardial effusion ❑ Fatigue ❑ Breathlessness ❑ Orthopnea ❑ Dizziness ❑ Hoarseness (recurrent laryngeal nerve compression) ❑ Hiccups (phrenic nerve compression) ❑ Abdominal pain (mesenteric ischemia) ❑ Nausea (diaphragm irritation) ❑ Loss of consciousness ❑ Cool extremities ❑ Peripheral cyanosis ❑ Peripheral edema Other etiology associated symptoms: ❑ Fever (suggestive of infectious etiology) ❑ Cough (suggestive of infectious etiology) ❑ Palpitations ❑ Malaise ❑ Joint pains (suggestive of autoimmune etiology) ❑ Odynophagia ❑ Weight loss (suggestive of malignant etiology) Obtain a detailed history ❑ Infections ❑ Pneumonia ❑ Tuberculosis ❑ HIV ❑ Travel history ❑ Travel to Central or South America (Chagas disease) ❑ Travel to Central Asia or South Africa or South America (Tuberculosis) ❑ Travel to North and Central America (Ohio and Mississippi River valleys) (Histoplasmosis) ❑ Travel to North America (Blastomycosis) ❑ Medications ❑ 5-Fluorouracil ❑ Amiodarone ❑ Anticoagulants ❑ Cyclosporine ❑ Cyclophosphamide ❑ Cytarabine ❑ Daunorubicin ❑ Doxorubicin ❑ Drug-induced lupus erythematosus ❑ Methysergide ❑ Penicillins ❑ Sulfa drugs ❑ Thiazides ❑ Thrombolytic agents ❑ Systemic illness ❑ Collagen vascular disease ❑ Hypothyroidism ❑ Inflammatory bowel disease ❑ Malignancy ❑ Uremia ❑ Others ❑ Cardiac surgery ❑ Radiation exposure ❑ Dressler's syndrome ❑ Postpericardiotomy syndrome ❑ Trauma history Examine the patient Vital signs ❑ Pulse ❑ Tachycardia (typical) ❑ Bradycardia (in hypothyroidism and uremia) ❑ Pulsus paradoxus (in cardiac tamponade) ❑ Blood pressure ❑ Normal (typical) ❑ Hypotension (in cardiac tamponade) ❑ Temperature ❑ Fever less than 39°C or 102.2°F ❑ Hypothermic (in elderly and renal failure) ❑ Respiratory rate ❑ Tachypnea (typical) Cardiovascular system Auscultation ❑ Heart sounds ❑ Normal (typical) ❑ New S3 heart sound ❑ Distant and muffled (in cardiac tamponade) ❑ Murmur (for concomitant heart disease) ❑ Pericardial friction rub ❑ High pitched, scratchy or squeaky sound ❑ Best heard at the left sternal border ❑ Best heard with the diaphragm of the stethoscope ❑ Varies in intensity overtime and needs repeated examinations {{#ev:youtube|watch?v=EUCp_3_vwtw|300}} Palpation ❑ Jugular venous pulse ❑ Elevated (in cardiac tamponade and constrictive pericarditis) ❑ Kussmaul sign (in constrictive pericarditis) Percussion ❑ Cardiac dullness beyond the apical point of maximal impulse (in pericardial effusion) Respiratory system ❑ Wheeze or rales ❑ Pleural effusion Abdomen ❑ Pulsatile hepatomegaly (in constrictive pericarditis) ❑ Ascites Order tests (urgent) Order laboratory tests (urgent): ❑ CBC (leucocytosis) ❑ ESR (elevated) ❑ C reactive protein (elevated) ❑ Serum cardiac troponin I and T ❑ Creatine kinase (CK-MB) ❑ Serum myoglobin ❑ Serum tumour necrosis factor ❑ Serum urea and creatinine Order electrocardiogram (urgent): ❑ Typical findings in pericarditis ❑ ST segment elevation in leads I, II, aVL, aVF, and V3-V6 ❑ PR segment depression ❑ Low QRS voltage (in large pericardial effusion and constrictive pericarditis) ST elevation in leads I, II, V2, V3, V4, V5, and V6 depicting acute pericarditis ❑ Electrical alternans (in cardiac tamponade) Order imaging (urgent): ❑ Chest X-ray ❑ Clear lung fields (typical) ❑ A flask-shaped, enlarged cardiac silhouette (in pericardial effusion and cardiac tamponade) ❑ Lateral view may reveal ❑ Thickened pericardial line (in pericarditis, pericardial effusion) ❑ Irregular contours of cardiac silhouette (in chronic pericarditis, pericardial fibrosis, post surgery, metastasis) Pericardial effusion ❑ Echocardiography (diagnostic test of choice) ❑ Typical findings in pericarditis ❑ Presence of moderate and large pericardial effusion ❑ Right atrial collapse ❑ Diastolic collapse of right ventricle and left atrium (specific for cardiac tamponade) ❑ Check for concomitant heart disease or paracardial pathology Does the patient have at least two of the following criteria for the diagnosis of acute pericarditis? ❑ Characteristic chest pain ❑ Sharp and pleuritic that is improved by sitting up and leaning forward ❑ Pericardial friction rub ❑ High pitched, scratchy sound at the left sternal border best heard with diaphragm of the stethoscope ❑ Heard during atrial systole, ventricular systole and rapid ventricular filling during early diastole ❑ Suggestive EKG changes ❑ Diffuse ST elevation with reciprocal ST depression in leads aVR and V1 ❑ Suggestive echocardiography changes ❑ New or worsening pericardial effusion Does the patient have any sign of myocarditis? ❑ Elevated cardiac enzymes, or ❑ Global or regional myocardial dysfunction on echocardiography Does the patient have any signs suspicious of acute pericarditis? ❑ Ongoing fever ❑ Poor response to treatment ❑ Hemodynamic compromise

Treatment

Treatment of Acute Pericarditis

Shown below is an algorithm summarizing the management of acute pericarditis in adults.

Click on boxes to expand/collapse detailed information.                       Does the patient have high risk features?                                                                                                                                 Yes                         No                                                               Inpatient treatment                         Outpatient treatment                                                                                                                     Stable patient           Unstable patient           Absence of pre-existing coronary artery disease         Presence of pre-existing coronary artery disease                                                                                                 ❑ Initiate medical therapy (first line: NSAIDs) ❑ Oder tests to identify the specific etiology ❑ Order pericardiocentesis if indicated           ❑ Immediately treat cardiac tamponade ❑ Initiate medical therapy (first line: NSAIDs) ❑ Oder tests to identify the specific etiology           ❑ Initiate medical therapy (first line: NSAIDs) ❑ Educate about life style modification         ❑ Initiate medical therapy (first line: aspirin) ❑ Educate about life style modification                                                                         Assess response to treatment                                                                                                           Response       No response                                                                                   Follow up as outpatient       Admit to the hospital

Does the patient have high risk features? High risk features include: ❑ Fever >38°C ❑ Leucocytosis ❑ Subacute presentation ❑ Cardiac tamponade ❑ Large pericardial effusion ❑ Elevated troponins (myopericarditis) ❑ Concurrent oral anticoagulation ❑ Lack of response to aspirin or NSAIDs after at least 1 wk of therapy ❑ Immunosuppressed state ❑ Acute trauma ❑ Relapsing pericarditis Unstable patient ❑ Cardiac tamponade ❑ Hypotension ❑ Pulsus paradoxus ❑ Jugular vein distention Management Initiate medical therapy Administer NSAIDs (First line) Avoid NSAIDs in coronary artery disease patients ❑ Ibuprofen ❑ Preferred ❑ Orally 300-800 mg TDS or QID x 1-2 weeks ❑ Gradual tapering every 2-3 days, OR ❑ Indomethacin ❑ Orally 50 mg TDS x 1-2 weeks ❑ Gradual tapering every 2-3 days for Rx period of 3-4 weeks OR Administer Aspirin ❑ Drug of choice in patients with pre-existing coronary heart disease ❑ High-dose aspirin: ❑ Orally 800 mg QID or TDS x 7-10 days ❑ Gradual tapering by 800 mg/week for 3 additional weeks ❑ Stop anticoagulants if patient develops pericardial effusion AND/OR Administer Colchicine ❑ Combination with NSAIDs (better response rate) ❑ Can be used alone ❑ Orally 0.5 mg BID x 3 months (>70 kg) ❑ Orally 0.5 mg OD x 3 months (≤70 kg) Administer gastroprotective agents ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) Do not administer steroids ❑ Avoid steroids in an initial episode of pericarditis Order tests to identify the specific etiology ❑ Order specifc tests based on the clinical suspicion ❑ Treat according to the etiology Order pericardiocentesis in case of ❑ High suspicion of purulent or neoplastic pericarditis ❑ Effusions > 20 mm in echocardiography in diastole ❑ Large or symptomatic effusions despite one week of medical therapy Management Treat cardiac tamponade ❑ Immediately transfer the patient to ICU ❑ Telemetry monitoring and frequent vital checks ❑ Call cardiology team immediately ❑ Pericardiocentesis is a life saving procedure in cardiac tamponade ❑ Make sure that the patient is oxygenating well Initiate medical therapy Administer NSAIDs ❑ Avoid in coronary artery disease patients ❑ Ibuprofen ❑ Preferred ❑ Orally 300-800 mg TDS or QID x 1-2 weeks ❑ Gradual tapering every 2-3 days, OR ❑ Indomethacin ❑ Orally 50 mg TDS x 1-2 weeks ❑ Gradual tapering every 2-3 days for Rx period of 3-4 weeks OR Administer aspirin ❑ Drug of choice in patients with pre-existing coronary heart disease ❑ High-dose aspirin: ❑ Orally 800 mg QID or TDS x 7-10 days ❑ Gradual tapering by 800 mg/week for 3 additional weeks ❑ Stop anticoagulants if patient develops pericardial effusion AND/OR Administer colchicine ❑ Combination with NSAIDs (better response rate) ❑ Can be used alone ❑ Orally 0.5 mg BID x 3 months (>70 kg) ❑ Orally 0.5 mg OD x 3 months (≤70 kg) Administer gastroprotective agents ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) Administer steroids ❑ Avoid steroids to treat an initial episode of pericarditis Order tests to identify the specific etiology ❑ Order specifc tests based on the clinical suspicion ❑ Treat according to the etiology Management Initiate medical therapy Administer NSAID's ❑ Ibuprofen ❑ Preferred ❑ Orally 300-800 mg TDS or QID x 1-2 weeks ❑ Gradual tapering every 2-3 days, OR ❑ Indomethacin ❑ Orally 50 mg TDS x 1-2 weeks ❑ Gradual tapering every 2-3 days for Rx period of 3-4 weeks AND/OR Colchicine ❑ Combination with NSAIDs (better response rate) ❑ Can be used alone ❑ Orally 0.5 mg BID x 3 months (>70 kg) ❑ Orally 0.5 mg OD x 3 months (≤70 kg) Administer gastroprotective agents ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) Avoid steroids ❑ Avoid steroids to treat an initial episode of pericarditis Educate about life style modification ❑ In case of pericarditis, avoid sternous physical activity until symptom resolution ❑ In case of myopericarditis, avoid competitive sports for six months and until normalization of lab findings Management Intitate medical therapy ❑ Administer High-dose aspirin ❑ Orally 800 mg QID or TDS x 7-10 days, OR ❑ Gradual tapering by 800 mg/week for 3 additional weeks ❑ Administer gastroprotective agents ❑ Misoprostol (600 to 800 mg/day), OR ❑ Omeprazole (20 mg/day) ❑ Stop anticoagulants if patient develops pericardial effusion ❑ Avoid steroids to treat an initial episode of pericarditis Life style modification ❑ In case of pericarditis, avoid sternous physical activity until symptom resolution ❑ In case of myopericarditis, avoid competitive sports for six months and until normalization of lab findings Follow up as outpatient ❑ Observe for recurrences or constriction ❑ Assess again at 7 to 10 days for treatment response ❑ At 1 month check blood tests and CRP ❑ Thereafter assess only if symptoms recur Admit to the hospital ❑ Indication that the underlying cause may not be viral or idiopathic in nature. ❑ Inpatient therapy ❑ Order sepcific tests to identify the etiology and treat accordingly

Treatment of Recurrent Pericarditis

Shown below is an algorithm summarizing the management of recurrent pericarditis in adults which encompass the incessant type (relapses on discontinuation of anti-inflammatory) and the intermittent type (widely varying symptom free interval without medical therapy).

Recurrent pericarditis

Characterize the clinical, EKG and imaging findings ❑ Characteristic acute pericarditis symptoms ❑ Characteristic acute pericarditis EKG changes ❑ Characteristic acute pericarditis echocardiography changes ❑ Massive pericardial effusion, cardiac tamponade, and pericardial constriction are rare ❑ Other clinical suspicion: ❑ Insufficient dose or/and insufficient treatment duration in an autoimmune pericardial disease ❑ Corticosteroid treatment during the first episode ❑ Early corticosteroid treatment causing augmented viral replication

Initiate medical therapy NSAIDs ❑ Avoid in coronary artery disease patients ❑ Ibuprofen: ❑ Preferred ❑ Orally 300-800 mg TDS or QID x 1-2 weeks ❑ Gradual tapering every 2-3 days ❑ Indomethacin: ❑ Orally 50 mg TDS x 1-2 weeks ❑ Gradual tapering every 2-3 days for Rx period of 3-4 weeks Aspirin ❑ Drug of choice in patients with pre-existing coronary heart disease ❑ High-dose aspirin: ❑ Orally 800 mg QID or TDS x 7-10 days ❑ Gradual tapering by 800 mg/week for 3 additional weeks ❑ Stop anticoagulants if patient develops pericardial effusion Colchicine: ❑ Effective in cases where NSAIDs failed to prevent relapses ❑ Combination with NSAIDs (better response rate) ❑ Can be used alone ❑ Orally 0.5 mg BID x 3 months (>70 kg) ❑ Orally 0.5 mg OD x 3 months (≤70 kg) Add gastroprotective agents: ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) Life style modification ❑ Excercise restriction until symptom resolution

Multiple relapses

Add corticosteroids ❑ Prednisone: 1-1.5 mg/kg x 1 month

Positive response

No response

Taper steroids ❑ Taper dose over a three-month period ❑ If symptoms recur ❑ Start the last dose that suppressed the symptoms ❑ Maintain the dose for 2-3 weeks and then taper ❑ Add colchicine or NSAIDs at the end of tapering of steroids

❑ Add azathioprine (75–100 mg/day) or cyclophosphamide

Treatment failure

Pericardiectomy ❑ Maintain the patient on steroid free regimen for several weeks before the procedure ❑ Order tests to identify the specific etiology and treat accordingly

Etiology Specific Management

Clinical subgroups	Specific investigations	Treatment
Viral pericarditis	❑ Perform testing for viral etiologies in immunocompromised and HIV infected patients not responding to intial management ❑ Diagnostic pericardiocentesis     ❑ Analysis of pericardial fluid (transudate or exudate)     ❑ PCR or in-situ hybridisation	❑ CMV pericarditis: Hyperimmunoglobulin OD 4 ml/kg on day 0, 4,and 8; 2 ml/kg on day 12 and 16 ❑ Coxsackie B pericarditis: Interferon alpha or beta 2,5 Mio. IU/m2 surface area s.c. 3 x per week ❑ Adenovirus and parvovirus B19 perimyocarditis: Immunoglobulin 10 g IV at day 1 and 3 for 6-8 hours
Purulent pericarditis	❑ Diagnostic pericardiocentesis in cases of high clinical suspicion     ❑ Gram stain, acid fast stain, fungal stain, and cultures of the pericardial fluid     ❑ Protein, glucose and cell count of the pericardial fluid ❑ Gram stain, acid fast stain, fungal stain, and cultures of other body fluids	❑ Therapeutic pericardiocentesis or pericardial window ❑ Pericardiectomy may be used in treatment of recurrent pericardial effusion and in patients with dense adhesions, loculated and thick purulent effusion ❑ Antimicrobial therapy in case of bacterial etiology     ❑ Intiate antistaphylococcal antibiotic plus aminoglycoside, followed by tailored antibiotic therapy according to pericardial fluid and blood cultures     ❑ Empiric regimen can be started for the following         ❑ Immunosuppression         ❑ Concurrent infection at another body site         ❑ Presence of intravascular lines or prosthetic devices         ❑ Recent antimicrobial therapy ❑ Antifungal therapy in case of fungal etiology
Tuberculous pericarditis	❑ Diagnostic pericardiocentesis in all suspected tuberculous pericarditis patients     ❑ PCR of pericardial fluid     ❑ High adenosine deaminase activity and interferon gamma concentration in pericardial effusion ❑ Pericardial biopsy (rapid diagnosis) ❑ Tuberculin skin test (not helpful) ❑ CT scan and/or MRI of the chest ❑ Culture of sputum, gastric aspirate, and/or urine ❑ Enzyme-linked immunospot (ELISPOT) ❑ Serum titres of antimyolemmal and antimyosin antibodies	❑Anti-tuberculosis chemotherapy     ❑ Emperic therapy in TB endemic areas and in cases with high clinical suspicion ❑ Pericardiectomy is warranted in the setting of persistent constrictive pericarditis or when no general improvement after 4-8 weeks following antituberculosis chemotherapy ❑ Prednisone can be used.
Neoplastic pericarditis	❑ CT scan and/or MRI of the chest ❑ Diagnostic pericardiocentesis when other tests couldnt identify malignancy ❑ Cytology and tumour markers ❑ Pericardial biopsy	❑ Systemic antineoplastic treatment ❑ Assess the life expectancy of the patients before proceeding with the treatment     ❑ Better prognosis patients should be treated more aggressively     ❑ Advanced malignancy should be treated palliatively with pericardiocentesis ❑ Recurrence of pericardial effusion is prevented using any of the following techniques     ❑ Prolonged pericardiocentesis     ❑ Pericardial sclerosis     ❑ Pericardiotomy     ❑ Intrapericardial chemotherapy
Pericarditis in renal failure	❑ Renal function test ❑ Diagnostic pericardiocentesis ❑ Pericardial biopsy	❑ Uremic pericarditis     ❑ Hemodialysis or peritoneal dialysis     ❑ Heparin-free haemodialysis should be used ❑ Dialysis-associated pericarditis     ❑ Pericardiocentesis for large effusion     ❑ Pericardiotomy in non resolving effusion
Pericarditis in systemic autoimmune disease	❑ Diagnostic pericardiocentesis     ❑ Elevated lymphocytes and mononuclear cells > 5000/mm3     ❑ Antisarcolemmal antibodies ❑ Exclusion of viral and bacterial etiologies	❑ NSAIDs or aspirin or colchicine ❑ Systemic corticosteroid can be used     ❑ Intrapericardial steroids has less side effects and is highly effective

Do's

• Always suspect acute pericarditis in the presence of characteristic pleuritic chest pain and pericardial friction rub. Also suspect pericarditis in a patient with persistent fever and pericardial effusion or new unexplained cardiomegaly.
• Exclude significant effusion and cardiac tamponade. Suspect acute cardiac tamponade in any patient presenting with Beck's triad: hypotension, muffled heart sounds and distended neck veins (or elevated jugular venous pressure).
• Heparin is recommended under strict observation for patients who need anticoagulant therapy.
• Restrict systemic corticosteroid therapy to connective tissue diseases, autoreactive or uremic pericarditis. Use intrapericardial application to avoid systemic side effects. Use moderate initial dosing of steroids followed by a slow taper. Introduce ibuprofen or colchicine early during tapering of steroids.
• Order an analysis of pericardial effusion for different etiologies according to the clinical presentation.
• Assess for the presence of coagulopathy or the intake of antithrombotic medications before choosing the modality of drainage of the pericardial fluid.
• Choose pericardiocentesis rather than surgical drainage as a therapeutic option unless the patient has an indication for surgical drainage. Consider surgical drainage in aortic dissection and myocardial rupture.
• When surgical drainage is indicated but the patient has severe hypotension prohibiting the induction of anesthesia, perform pericardiocentesis in the operating room before surgery.
• Monitor closely patients who underwent pericardiocentesis for postdrainage decompensation.

Don'ts

• When cardiac tamponade is suspected, do not delay its treatment.
• Avoid pericardiocentesis in cases where the diagnosis can be made based on systemic features or when the effusions are very small or resolving with anti-inflammatory treatment.
• Don't perform pericardiocentesis in aortic dissection and ruptured ventricular aneurysm and avoid it in cases of uncorrected coagulopathy, anticoagulant therapy, thrombocytopenia < 50,000/mm 3 , small, posterior, and loculated effusions.

References

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