Pericarditis, cardiac tamponade and myocarditis


Pericarditis

Essentials

  • 1

    Myocarditis is often associated with pericarditis. This has important clinical implications.

  • 2

    Pericarditis is diagnosed based on history and findings on both electrocardiography (ECG), and echocardiography. The diagnosis is based on two of the four criteria outlined in Table 5.6.1 .

    Table 5.6.1
    European Society of Cardiology definition of and diagnostic criteria for pericarditis (2015)
    Pericarditis Definition and diagnostic criteria
    Acute Inflammatory pericardial syndrome to be diagnosed with at least two of the four following criteria:

    • 1.

      Pericarditic chest pain

    • 2.

      Pericardial rub

    • 3.

      New widespread ST-segment elevation or PR depression in the ECG

    • 4.

      Pericardial effusion (new or worsening)

    Additional supporting findings:

    • Elevation of markers of inflammation

    • Evidence of pericardial inflammation by an imaging technique (CT, MRI)

    Incessant Pericarditis lasting for >4 to 6 weeks but <3 months without remission
    Recurrent Recurrence of pericarditis after a documented first episode of acute pericarditis and a symptom-free interval of 4 to 6 weeks or longer
    Chronic Pericarditis lasting for 3 months
    CT , Computed tomography; ECG , electrocardiography; MRI , magnetic resonance imaging.

  • 3

    The majority of cases of pericarditis are idiopathic or viral in aetiology and run a benign course.

  • 4

    Multimodality imaging for pericardial disease is essential for comprehensive evaluation of the disease condition.

  • 5

    The correct identification of pericarditis as opposed to ST-segment elevation myocardial infarction (STEMI) is essential. Administration of fibrinolysis in cases of pericarditis may result in life-threatening complications and delay in reperfusion therapy; it is also deleterious to patient outcome.

  • 6

    Longer-term follow up is important, as a sub-acute or chronic course can develop, with complications such as chronic constrictive pericarditis.

Introduction

Pericarditis is an inflammation of the pericardium and may be acute, sub-acute or chronic, depending on symptom duration and their recurrence. In the majority of cases there are variable degrees of associated ‘epimyocarditis’. The condition is therefore better described as perimyocarditis. It most frequently affects young and middle-aged individuals, is often recurrent and affects men more often than women. It has an overall mortality rate of 1.1%. The causes of pericarditis are listed in Table 5.6.2 .

Table 5.6.2
Causes of acute pericarditis
Idiopathic/viral Coxsackie viruses, echoviruses, adenovirus, influenza A and B viruses; enterovirus; mumps virus; Epstein-Barr virus; HIV; herpes simplex virus; type I varicella zoster virus (VZV); measles; parainfluenza viruses type II; RSV; CMV; hepatitis A, B and C; parvovirus B 19
Bacterial Gram positive and gram negative species (streptococci, Staphylococcus, Pneumococcus), Mycobacterium tuberculosis . Less common— Legionella, Norcardia, Actinobacillus, Rickettsia, Borrelia burgdorferi (Lyme disease), Listeria, Leptospira, Chlamydophila psittaci, Treponema pallidum (syphilis), Coxiella burnettii , Meningococcus species, Haemophilus species, Mycoplasma species
Fungal Histoplasma , Blastomyces , Coccidia, Aspergillus, Candida
Parasitic Toxoplasma, Entamoeba , Echinococcus
Autoimmune/connective tissue Systemic lupus erythematosus, Sjogren syndrome, rheumatoid arthritis, scleroderma, vasculitides (eosinophilic granulomatosis) (Churg-Strauss syndrome), Takayasu disease, Behcet syndrome, sarcoidosis, familial Mediterranean fever, inflammatory bowel disease, Still disease, mixed connective tissue disorder, Reiter syndrome, Wegener granulomatosis, ankylosing spondylitis, giant cell arteritis, dermatomyositis, serum sickness
Trauma, iatrogenic Coronary interventions, permanent pacemaker/ICD implantation, radiofrequency ablation, penetrating, non-penetrating trauma, oesophageal perforation, rupture
Other Dissecting aneurysm, radiation injury
Myocardial infarction Acute: days to weeks following transmural myocardial infarction
Dressler syndrome: weeks to months following myocardial infarction
Drugs Daunorubicin, doxorubicin, cyclophosphamide, 5 fluorouracil, amiodarone, cyclosporine, mesalazine, clozapine, methysergide, anti–tumour necrosis factor, hydralazine, procainamide, methyldopa, phenytoin, isoniazid, reserpine.
Hypersensitivity syndromes (e.g. penicillin)
Metabolic causes Uraemia, myxoedema, cholesterol pericarditis
Malignancy Primary (e.g. sarcoma and mesotheliomas)
Secondary (e.g. haematological, breast, lung and melanoma)
CMV , cytomegalovirus; RSV , respiratory syncytial virus; HIV , Human immunodeficiency virus; ICD , implantable cardioverter-defibrillator.

Clinical features

History

Chest pain is the most common presentation (>85% to 90% of cases). It tends to be sharp in nature and is usually localized to the precordial/retrosternal area. The pain is classically worse on inspiration and is often positional. Pain may radiate to the trapezius ridge, neck or shoulder due to involvement of the phrenic nerve. It is typically worse when the patient is supine and improves when he or she is sitting up and leaning forwards. The aetiology is often idiopathic, but a focused history should be directed towards the known causative pathologies, such as post–cardiac injury syndromes, rheumatological manifestations of heart disease (e.g. systemic lupus erythematosus) and complications of malignancy. Dyspnoea is not an expected associated feature unless there are secondary complications, such as cardiac tamponade or constrictive pericarditis.

Examination

A careful history and physical examination should be undertaken in any patient with a suspected pericarditis. Temperature above 38°C is uncommon and may indicate purulent (i.e. bacterial) pericarditis. Sinus tachycardia is common. A pericardial friction rub may be audible when the diaphragm of the stethoscope is placed over the lower left sternal edge with the patient leaning forwards in the left lateral decubitus position (<33% of cases). The rub has a superficial scratching or ‘Velcro-like’ quality. Pericardial friction rubs may be difficult to detect, as they can be transient and migratory. A raised jugular venous pressure (JVP), hypotension, and pulsus paradoxus are suggestive of cardiac tamponade. Pericardial tamponade, a potentially lethal complication of pericarditis, complicates up to 15% cases of acute idiopathic pericarditis.

High-risk features

The following high-risk clinical features should raise suspicion of serious underlying pathology, such as tuberculosis (TB) or other bacterial infection, malignancy or autoimmune disease:

  • Fever

  • True dyspnoea

  • A sub-acute course

  • Significant effusion or tamponade

  • Failure to respond to aspirin or non-steroidal anti-inflammatory drugs (NSAIDs)

  • Patients on anticoagulant therapy

  • Immunosuppression

  • Recent trauma

  • Patients with suspected significant associated myocarditis indicated by arrhythmias, significantly elevated ST segments or significantly elevated troponin levels

Clinical investigations

Blood tests

  • Full blood count: leucocytosis is common, but a markedly elevated white cell count should raise suspicion of bacterial infection.

  • Serum biochemistry: may identify underlying renal failure.

  • Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) provides confirmatory evidence of an inflammatory process and can be used to follow resolution.

  • The troponin level can be minimally elevated in patients with acute pericarditis. Usually the troponin level returns to normal in 1 to 2 weeks. Sustained elevations are suggestive of concomitant myocarditis.

  • Other blood tests will be dictated by the clinical assessment and the degree of clinical suspicion for any given causative pathology.

Chest x-ray

The chest x-ray is usually normal in acute pericarditis unless there is a large pericardial effusion. This investigation has a class 1 indication in the European Society of Cardiology (ESC) guidelines for pericardial disease.

European Society of Cardiology guidelines

The ECG is a critical investigative tool and will show abnormalities in 90% of patients with acute pericarditis. ECG changes are the result of the associated epimyocarditis. The pericardium is electrically neutral and does not produce ECG changes. Therefore, in the occasional ‘pure’ case of pericarditis, the ECG will be normal.

The typical ECG pattern of pericarditis follows four stages:

  • Stage 1: hours to days

  • Widespread concave upwards ST elevation (60% of cases). This may occur in all leads apart from AVR and VI ( Fig. 5.6.1 ).

    • PR depression.

    • ‘Spodick’ sign (down-sloping TP segments) seen in 80% of patients with acute pericarditis. It is not usually observed in patients with acute coronary syndrome or early repolarization.

    Fig. 5.6.1, Typical electrocardiogram in pericarditis.

  • Stage 2: the PR and ST segments normalize, which can lead to a transiently normal ECG.

  • Stage 3: days to weeks: T-wave inversion occurs.

  • Stage 4: normalization of the ECG: over a period of up to 3 months; however, in some cases the T-wave changes may be permanent.

Atypical ECG findings may include the following:

  • A normal ECG in cases of pure pericarditis (remembering that during stage 2 the ECG may also be transiently normal during a typical evolution).

  • The PR-segment depression may occur in isolation, without any ST-segment elevation.

  • Stages 1 and 2 without progression to stage 3.

  • Localized as opposed to diffuse ECG changes.

Echocardiography

As per ESC guidelines for pericardial diseases, routine transthoracic echocardiography (TTE) is recommended in all patients with acute pericarditis (class 1, level C). It is a crucial imaging technique for detecting pericardial fluid and its haemodynamic effects on the heart if cardiac tamponade or constrictive physiology is suspected. It is also helpful to differentiate from acute myocardial ischaemia by excluding wall motion abnormality. A normal echocardiogram does not rule out a diagnosis of pericarditis. Transoesophageal echocardiography (TOE) is better at measuring thickness of the pericardium than TTE.

Computed tomography scan/magnetic resonance imaging

Computed tomography (CT) and magnetic resonance imaging (MRI) should be considered as further imaging modalities in patients with underlying aetiologies such as malignancy, TB or systemic inflammatory conditions. In most patients, they provide excellent images of the pericardium, including thickness, the presence of effusion and any pericardial lesions. In patients with myopericarditis, MRI shows late gadolinium enhancement in the pericardium. Pericardial uptake of F-fluorodeoxyglucose (FDG) tracer is found in patients with solid cancers and lymphoma and is indicative of malignant pericardial involvement. Positron emission tomography (PET)/CT is also of value in identifying the nature of inflammatory pericarditis. In particular, TB pericarditis yields a higher FDG uptake than idiopathic forms.

Pericardiocentesis and biopsy

Rarely required, pericardiocentesis and biopsy may be indicated in suspected bacterial, tuberculous or neoplastic pericarditis, when cardiac tamponade is present or in chronic or recurrent cases for diagnostic purposes.

Criteria for diagnosis

The clinical diagnosis is based on the presence of two out of four diagnostic criteria in the ESC guidelines. These include (1) typical clinical history, (2) the presence of a pericardial friction rub, (3) typical ECG changes, (4) pericardial effusion.

Convenient diagnoses— such as ‘muscular’, ‘fibrositis’, ‘costochondritis’ and ‘viral’—should be avoided until more important conditions such as pericarditis, pulmonary embolus and pneumothorax are excluded.

The most difficult clinical decision in the ED is differentiating between pericarditis, ST-elevation myocardial infarction (STEMI) and benign early repolarization (BER). These ECG features assist in distinguishing between the possible diagnoses are summarized in Table 5.6.3 .

Table 5.6.3
Pericarditis vs acute myocardial infarction vs benign early repolarization
ECG feature Acute pericarditis STEMI BER
ST-segment morphology Concave upwards ST elevation Convex upwards ST elevation Concave upwards ST elevation
ST segment elevation Usually <5 mm If >5 mm, more suspicious Usually <5 mm
ST-segment changes distribution Diffuse Anatomic Precordial only
Reciprocal changes No, mild depressions only in AVR, V1 Deep reciprocal changes opposite ST-elevated segments No
Q waves No (unless associated with infarction) Yes No
PR segments PR-segment depressions (may be elevated in AVR and V1) No No
T-wave inversion T-wave inversion after ST segments normalize T waves may invert concurrently with elevation of ST segments No
ST/T ratio >0.25 N/A <0.25
Usual pattern of evolution of changes Days to weeks Minutes to days Stable over many years
AMI , Acute myocardial infarction; AVR , augmented vector right; BER , benign early repolarization; ECG , electrocardiography; STEMI , ST-elevation myocardial infarction.

Classification of pericarditis

The ESC Task Force has classified pericarditis into three broad categories based on clinical behaviour. The term ‘acute’ should be adopted for new-onset pericarditis. The term ‘incessant’ refers to cases with persistent symptoms without a clear-cut remission after the acute episode. The term ‘chronic’ refers to disease processes lasting more than 3 months. Incessant refers to pericarditis with symptoms persisting for more than 4 to 6 weeks (that is generally the approximate length of conventional anti-inflammatory therapy and its tapering). Finally, ‘chronic’ refers to pericarditis lasting longer than 3 months.

Clinical management and treatment

It is no longer considered mandatory to search for an aetiology in all patients, particularly in areas with a low prevalence of TB. This is because of the relatively benign course and low diagnostic yield. There are some high-risk clinical features that are associated with increased risk of complications. These include a high fever (>38°C), a sub-acute clinical course, evidence of a large pericardial effusion (i.e. diastolic echo-free space of >20 mm), cardiac tamponade and failure to respond within 7 days to NSAIDs. Other risk factors include pericarditis in association with myocarditis, immunodepression, trauma and anticoagulation.

Any clinical presentation that suggests an underlying aetiology such as a systemic inflammatory disease or with any of the high-risk criteria should be admitted to hospital for further assessment and monitoring; patients with none of these conditions can be managed in an ambulatory setting with close follow-up to assess response to treatment and monitor for complications.

Non-steroidal anti-inflammatory drugs

The goals of therapy for acute pericarditis are relief of symptoms, decrease in inflammation, and prevention of recurrences. Aspirin or NSAIDs are the mainstay of therapy. Treatment duration should be based on the resolution of symptoms and normalization of CRP. ESC 2015 guidelines recommend aspirin or ibuprofen as first-line therapy. Results of multiple cohorts and one randomized cohort study suggest that NSAIDs are effective in approximately 70% to 80% of viral/idiopathic pericarditis. Patients who fail to respond or have worsening of symptoms should be evaluated for other aetiologies. The recommended dose of aspirin is 750 to 1000 mg every 8 hours for 1 to 2 weeks, decreasing the dose by 250 to 500 mg every 1 to 2 weeks. The dose for ibuprofen is 600 mg every 8 hours for 1 to 2 weeks and then decreases by 200 to 400 mg every 1 to 2 weeks. In patients with history of acute myocardial infarction and pericarditis, aspirin is the drug of choice. NSAIDs should be avoided as they may hamper myocardial healing and scar formation.

Colchicine

Colchicine is an important component of therapy and has been shown to be effective not only in reducing symptoms but also leading to a 50% reduction in recurrence. It should be given with aspirin and NSAIDs. The ESC 2015 guidelines suggest 0.5 mg once daily for patients weighing <70 kg or 0.5 mg twice a day for those weighing more than 70 kg for 3 months. The initial dose should be maintained until resolution of symptoms and normalization of biomarkers when tapering should be considered.

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