The umbrella term ‘inflammatory myopericardial syndrome’ (IMPS) sometimes used as there is an overlap between pericarditis and myocarditis, which have similar aetiologies. See also myocarditis
Clinical features
- History
-
- Duration of symptoms usually <1/12 in acute pericarditis
- Chest pain (in 90%)
-
- Often sharp, retrosternal, pleuritic (and worse on coughing) and positional
- Worse lying flat, better sitting forward – although this is also true for the pain of cardiac ischaemia, so doesn’t discriminate
- Left trapezius ridge pain is characteristic
-
- SOB in the elderly
- Sometimes recent fevers (70%), flu-like illness, gastroenteritis
-
- Signs
-
- May be signs of pericardial effusion / tamponade – pulsus paradoxus (reduced pulse in inspiration), elevated JVP, quiet heart sounds
- Pericardial friction rub (<33% of cases)
- Signs of right sided HF in constrictive pericarditis
- Arrythmias rare (and usually AF)
- Check for signs of an associated systemic disease – joints, eyes, mouth, skin, hands
-
Definition
- Pericarditis is defined as a typical clinical presentation with one of (possible pericarditis) or > 1 of (definite pericarditis):
-
- Pericardial rub
- Typical ECG changes
- Elevated CRP
- New/worsening pericardial effusion
- Pericardial oedema/LGE on CMR (If one present = possible pericarditis)
-
Causes
- Infections – viral (eg coxsackie B), rheumatic fever, TB common worldwide, Lyme (ask about tick bites and rashes)
- Post cardiac injury syndrome
-
- Post MI pericarditis (Dressler’s) – usually in larger MIs, 1-2 weeks later (if effusion >10mm, investigate for possible subacute heart rupture). Treat with high dose aspirin for 7 days plus colchicine
- Post-trauma, pericardiotomy, device implantation or ablation
-
- Uraemia
- Autoimmune
-
- Hypothyroidism, RA, SLE, PAN, ANCA-associated vasculitis, scleroderma, Sjogren’s, inflammatory myositis, Kawasaki’s, Familial Mediterranean Fever and TRAPS
-
- Malignancy
-
- Often related to metastatic lymphatic involvement in lung cancer, or homogenous spread in breast cancer.
- Can be from haematological cancers, melanoma and oesophageal cancer. Pericardial mesothelioma is rare
- Even if cancer elsewhere, 2/3 of pericardial effusions in these patients are caused by something else (eg infection, radiation, drugs)
-
- Radiotherapy
- Drugs – eg. Isoniazid, ciclosporin
- Aortic dissection
ECG changes
- Widespread concave ST elevation (40%) and PR depression throughout most of the limb leads and precordial leads
- PR depression suggests viral cause and can last for only hours
- Reciprocal ST depression and PR elevation in lead aVR (but no reciprocal changes elsewhere unlike MI)
- Sinus tachycardia
- Check for changes of a pericardial effusion (low voltage, electrical alterans)
- In 1-3 weeks normalisation of ST changes and T wave flattening
- TWI beyond 3 weeks before ECG normalises (< 50% progress through all stages)
- Some argue that since the pericardium is electrically silent, ECG changes imply concomitant inflammation of the myocardium, warranting investigation for concurrent myocarditis
- Sometimes AF if predisposed
- Benign early repolarisation can mimic the ECG in pericarditis
- MI is the key differential
-
- Convex/horizontal ST elevation suggests MI (but concave ST elevation could still be either)
- ST elevation greater in III than II suggests MI
- PR depression occurs in both but in pericarditis it’s more likely to occur in multiple leads
- Downsloping TP segment (Spodick’s sign) much more likely in pericarditis than STEMI
- Pan ST elevation (not following an arterial territory) makes myopericarditis more likely
- History useful can’t reliably differentiate
- Pericarditis more likely in younger patients with no CV risk factors
-
Other investigations
- CxR – can be a globular heart. Check for pleural effusions
- Do echo
-
- Normal in around 40%
- Pericardial effusion present in around 60% and generally mild (ie <10mm)
- A large effusion (>20mm) is more likely in non-idiopathic causes
- Rule out tamponade
- New reduced EF suggests myocarditis
-
- Bloods
-
- Check trop in case of myocarditis (and if trop rise, do cardiac MRI).
- CRP elevated in 85% (normal doesn’t exclude diagnosis, can help guide duration of anti-inflammatory treatment)
- TFTs, BNP, LFTs, FBC (check eosinophils), CK, HIV, Hep C
- Consider sending autoantibodies, cold agglutinins, Lyme serology
- Don’t bother with other viral serology – low yield
-
- CMR if diagnostic uncertainty
-
- Best done in first 2 weeks
- Can detect thickening, oedema, and LGE of the pericardium (which can imply ongoing inflammation and neovascularization rather than simple scarring)
-
- CT useful if large effusion to help make aetiological diagnosis (and if considering pericardiectomy)
- Genetic tests – 10% of recurrent pericarditis have genetic variants (sometimes related to monogenic autoinflammatory disease like familial mediterranean fever or TRAPS)
Assessing risk
- Red flags
-
- Signs of heart failure
- Cardiomegaly on CxR
- Deep TWI
- Raised troponin
-
- High risk features (especially the first 4). Manage as inpatient if any one of:
-
- Fever >38°C
- Subacute onset (1 month to 3 months)
- Large effusion (>20 mm) or tamponade or signs of constrictive pericarditis (SOB, fatigue, oedema, normal EF)
- Lack of response to 1 week of NSAIDs
- Any history of trauma or oral anticoagulant use
- Immunosuppression/deficiency
- Raised troponin suggesting myopericarditis
-
Treatment
- Ibuprofen 600mg TDS plus PPI for 2 weeks. Once CRP down can reduce dose and taper.
- Or Aspirin if CV risk factors. 900mg TDS, tapering after 1 week.
- Then colchicine for 3-6 months (500mcg BD if > 70kg, OD if < 70kg)
- 25% will recur without colchicine, this is halved with treatment
- Alternative regimen – 500mcg colchicine BD for 3 months and only PRN NSAIDs
- Low-dose steroids – helpful second-line agents for managing recurrences as adjuncts to NSAIDs and colchicine (prednisolone 40mg OD for 4 weeks then taper) but should not be used 1st line (can actually increase risk of recurrence) unless autoimmune disease present
- Consider stopping anticoagulants
- Azathioprine occasionally used if recurrences
- Anakinra occasionally used if recurrent
- Betablocker used if symptomatic and HR >75
- Pericardiocentesis/drainage (image-guided)
-
- Indications:
-
- Tamponade
- Suspected bacterial (including TB) or malignant cause
- Moderate/large pleural effusion not responding to medical therapy
-
- Aim to drain <500ml to prevent pericardial decompression syndrome
- Occasionally surgical drain required if purulent, or pericardial window
- Send specific tests on pericardial fluid if suspected cancer or TB (see below)
- Indications:
-
Specific scenarios
- TB pericarditis
-
- If suspected pericardiocentesis/drain often advised
- Pericardial fluid can be bloody lymphocytic effusion with elevated adenosine deaminase (which has high sensitivity).
- Diagnosis challenging. Do culture and PCR on fluid.
- Culture has 65% sensitivity and takes >3 weeks.
- Can check interferon gamma in the fluid (high sensitivity and specificity) but IGRA not useful
- Commonly leads to pericardial constriction (50%) if TB not treated (use standard 6-month regimen).
- Consider intrapericardial urokinase and high dose adjunctive steroids for 6 weeks (but maybe avoid if HIV positive)
-
- If neoplastic pericarditis suspected
-
- Check fluid cytology
- Can check tumour markers in the pericardial fluid too (e.g. CEA, CYFRA 21-1, NSE, CA-19-9, CA-72-4, SCC, GATA3, and VEGF)
- However no tumour markers have been proven to be accurate enough for distinguishing malignant from benign effusions
-
- Purulent pericarditis
-
- Drain required irrespective of haemodynamics.
- Low pericardial to serum glucose ratio (mean 0.3, compared to 0.7 for TB and 0.8 for cancer), high neutrophil count (often around 90%, compared to 50% in TB and cancer)
- Effusions often heavily loculated and can rapidly reaccumulate
-
Complications
- Tamponade. Rare.
- Constrictive pericarditis (<1%) – a chronic or transient condition causing SOB, oedema, fatigue. Often isolated right HF with TR and normal-ish BNP. May need pericardiectomy if medical therapy fails. Especially common if bacterial cause
- Recurrent pericarditis – more common if not adherent to treatment
- Incessant pericarditis – high risk of constrictive pericarditis
On discharge
- Repeat bloods in one week with GP or via SDEC
- Refrain from exercise whilst symptomatic and then for 3 months (but can individualise this period), and until CRP/ECG normal (little evidence though)
- Small risk of recurrence – check temperature and CRP if symptomatic again. Pericardial pain is expected but there should be no inflammation.
- Safety net – return in one week if pain not responding to NSAIDs