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

Overview

Pericarditis refers to the inflammation of the fibroelastic sac surrounding the heart (pericardium), and it can be dry, fibrinous or effusive independently from the etiology. Myopericarditis, or perimyocarditis refers to acute pericarditis cases that also demonstrate myocardial inflammation resulting in global or regional myocardial dysfunction, elevations of troponins, creatine kinase MB, myoglobin and tumour necrosis factor. Depending on the time of presentation and duration, pericarditis is divided into acute (<6 weeks), subacute (6 weeks to 6 months) and chronic (>6 months) forms. Acute pericarditis is more common than chronic pericarditis (a form of which is constrictive pericarditis), and can occur as a complication of infections, with viral etiology being the most common. Always suspect acute pericarditis based on a history of characteristic pleuritic chest pain and on a pericardial friction rub finding. NSAIDs are the mainstay in the treatment of acute pericarditis and ibuprofen is the most preferred drug for its favourable 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: Adeno virus, 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.

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 ❑ Hoarsenes (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 (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: Vitals ❑ 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: ❑ Heart sounds ❑ Normal (typical) ❑ New S3 heart sound ❑ Distant and muffled (in cardiac tamponade) ❑ 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 reapeated examinations {{#ev:youtube|watch?v=EUCp_3_vwtw|300}} ❑ Jugular venous pulse ❑ Elevated (in cardiac tamponade and constrictive pericarditis) ❑ Kussmaul sign (in constrictive pericarditis) ❑ Any murmur (for concomitant heart disease) ❑ Percuss cardiac dullness ❑ Dullness beyond the apical point of maximal impulse is seen in pericardial effusion Respiratory system: ❑ Wheeze or rales ❑ Pleural effusion Abdominal examination: ❑ Pulsatile hepatomegaly (in constrictive pericarditis) ❑ Ascites

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-voltage QRS complexes (in large pericardial effusion and constrictive pericarditis) Above EKG shows 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 ❑ 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

Diagnosis of acute pericarditis: Atleast two of the following criteria: ❑ 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

Yes

No or equivocal

❑ Elevated cardiac enzymes ❑ Global or regional myocardial dysfunction on echo

Consider cardiac MRI (CMR)

Consider alternative diagnosis and treat accordingly

No

Yes

Treat as acute pericarditis or myopericarditis if there is delayed enhancement on CMR

Acute pericarditis

Myopericarditis

Treatment

Treatment of Acute Pericarditis

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

Acute pericarditis or myopericarditis

High risk features ❑ 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

Yes

No

Inpatient treatment

Outpatient treatment

Stable

Unstable ❑ Cardiac tamponade ❑ Hypotension ❑ Pulsus paradoxus ❑ Jugular vein distention

No pre-existing coronary artery disease

Pre-existing coronary artery disease

❑ NSAID's or aspirin or colchicine (usual regimen) ❑ Clinical testing for underlying etiology ❑ Order tests for specific etiologies according to the clinical presentation ❑ Management of recurrent pericarditis ❑ Pericardiocentesis: ❑ High suspicion of purulent or neoplastic pericarditis ❑ Asymptomatic pateints with effusions > 20 mm in echocardiography in diastole ❑ Large or symptomatic effusions despite one week of medical therapy

❑ Immediately transfer the patient to ICU ❑ Telemetry monitoring and frequent vital checks ❑ Call cardiology team immediately ❑ Management of cardiac tamponade management ❑ Pericardiocentesis is life saving in cardiac tamponade ❑ Make sure patient is oxygenating well ❑ Clinical testing for underlying etiology ❑ Order tests for specific etiologies according to the clinical presentation ❑ NSAID's or aspirin or colchicine (usual regimen)

NSAID's ❑ Ibuprofen: ❑ Preferred ❑ Orally 300-800 mg TDS or QID x 1-2 weeks ❑ Gradual tapering every 2-3 days ❑ Avoid in coronary artery disease patients ❑ Indomethacin: ❑ Orally 50 mg TDS x 1-2 weeks ❑ Gradual tapering every 2-3 days for Rx period of 3-4 weeks ❑ Avoid in coronary artery disease patients Or ❑ Add gastroprotective agents ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) 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) Steroids ❑ Avoid steroids to treat an initial episode of pericarditis

Aspirin ❑ High-dose aspirin: ❑ Orally 800 mg QID or TDS x 7-10 days ❑ Gradual tapering by 800 mg/week for 3 additional weeks ❑ Add gastroprotective agents ❑ Misoprostol (600 to 800 mg/day) ❑ Omeprazole (20 mg/day) ❑ Stop anticoagulants if patient develops pericardial effusion Steroids ❑ Avoid steroids to treat an initial episode of pericarditis

Response to Rx

Yes

No

Follow up ❑ Observe for recurrences or constriction ❑ Assess at 7 to 10 days for treatment response ❑ At 1 month check blood tests and CRP ❑ Thereafter only if symptoms recur

Hospital admission ❑ Indication that the underlying cause may not be viral or idiopathic in nature. ❑ Inpatient therapy ❑ Etiology specific management

Treatment of Recurrent Pericarditis

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

Recurrent pericarditis

The intermittent type ❑ Symptom free interval without therapy ❑ Widely varying symptom free interval

The incessant type ❑ Always relapses on discontinuation of anti-inflammatory

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 ❑ Early corticosteroid treatment causing augmented viral DNA/RNA replication

Medications ❑ NSAIDs or aspirin ❑ Same regimen as in acute pericarditis management ❑ Colchicine ❑ Same regimen as in acute pericarditis management ❑ Effective in cases where NSAIDs failed to prevent relapses ❑ Exercise restriction

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 ❑Clinical testing for specific etiologies

Etiology Specific Management

Clinical subgroups	Specific investigations	Treatment
Viral pericarditis	❑ Immunocompromised and HIV infected patients ❑ 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 ❑ 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 efussion and in patients with dense adhesions, loculated and thick purulent effusion ❑ Antimicrobial therapy  :❑ 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
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 ❑ 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  :❑ Increased number of lymphocytes and mononuclear cells > 5000/mm 3  :❑ 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 based on a history of characteristic pleuritic chest pain and on a pericardial friction rub finding. Pericarditis should also be suspected in a patient with persistent fever and pericardial effusion or new unexplained cardiomegaly.
• Initial efforts should focus upon excluding a significant effusion or tamponade. Suspect acute cardiac tamponade in any patient presenting with Beck's triad: hypotension, tachycardia and distended neck veins (or elevated jugular venous pressure).
• NSAIDs are the mainstay in the treatment of uncomplicated acute pericarditis and ibuprofen is the most preferred for its favourable effect on the coronary flow, fewer side effects, and the large dose range.
• Systemic corticosteroid therapy should be restricted to autoimmune or uremic pericarditis and ibuprofen or colchicine should be introduced early during tapering of steroids.
• Heparin is recommended under strict observation for patients who need anticoagulant therapy.
• Systemic corticosteroid therapy should be restricted to connective tissue diseases, autoreactive or uremic pericarditis. Intrapericardial application avoids systemic side effects and is highly effective. Always suggest the use of moderate initial dosing followed by a slow taper.
• Analyses of pericardial effusion for different etiologies should be ordered 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.

Dont's

• Never delay treatment whenever cardiac tamponade is suspected.
• Avoid pericardiocentesis in cases where the diagnosis can be made based on other systemic features or when the effusions are very small or resolving under antiinflammatory treatment
• Don't perform pericardiocentesis in aortic dissection and ruptured ventricular aneurysm and avoid in cases of uncorrected coagulopathy, anticoagulant therapy, thrombocytopenia < 50,000/mm 3 , small, posterior, and loculated effusions.

References

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