Acute pericarditisI30.9

A special form of pericarditis, which occurs as acute post-infarction pericarditis after myocardial death, was first described in 1956 by William Dressler (1890 - 1969) and named after him, the so-called Dressler syndrome (Erdmann 2009).

Acute pericarditis is an infectious or non-infectious inflammation of the pericardium, often accompanied by pericardial effusion (so-called exudative pericarditis), in contrast to sicca pericarditis, which is accompanied by no effusion (Maisch 2008). In most cases, subepicardial myocardial layers are also involved in the inflammatory process. In such cases one speaks of perimyocarditis (Hombach 2009).

Acute pericarditis is divided into the following forms - regardless of the etiology (Fritze 2012):

• dry pericarditis (so-called sicca or fibrinous pericarditis)
• Pericarditis with accompanying effusion (so-called exudative pericarditis)

Clinically, a reliable separation between pericarditis and myocarditis is not always possible and not even useful, since subepicardial myocardial layers are often also affected by the infection in pericarditis. The term "perimyocarditis" was introduced for these cases (Herold 2018).

The incidence of acute pericarditis is about 1,000 new cases per 1 million inhabitants per year. Since the disease can also be inapparent, a much higher number of unreported cases is suspected (Erdmann 2009).

Pericarditis can occur at any age, but it prefers young adults of both sexes (Herold 2018).

Autopsies show pericarditis in about 2% - 10% of cases (Maisch 2008).

The causes of acute pericarditis are very different. More than 50% are idiopathically caused (in the vast majority of cases, however, they are caused by viruses (Herold 2018) - with appropriate diagnostics -, approx. 30% - 50% are infectious, the other causes are rather rare.

We distinguish between the following forms:

1. infectious pericarditis:

Infectious pericarditis is caused by:

• viruses: Coxsackie A9, B1-4, mumps, Epstein-Barr virus, chickenpox, rubella, cytomegalovirus, echovirus, parvovirus B9, HIV and others
• Bacteria: Meningo-, Pneumo-, Gonococci, Treponema pallidum, Haemophilus, Borreliosis, Tuberculosis, Chlamydia and others
• Fungi: Histoplasma, Candida and others
• Parasites: Echinococcus, Entameba histolytica, Toxoplasma and others

2. pericarditis in systemic autoimmune diseases such as

• Scleroderma (systemic sclerosis) in > 50%.
• Lupus erythematodes disseminatus ( in approx. 30%)
• rheumatoid arthritis (in approx. 30%)
• Ankylosing spondylitis (in approx. 1%)
• Dermatomyositis (very rare)
• Reiter's syndrome (in approx. 2%)
• Periarthritis nodosa (very rare)
• familial Mediterranean fever ( in approx. 0,7%)
• amongst others

3. as autoimmune disease type 2 in:

• feverish rheumatoid arthritis (in approx. 20% - 50%)
• Postmyocardial infarction syndrome (in approx. 1% - 5%)
• Postcardiotomy syndrome (also known as Dressler syndrome) 20
• autoreactive chronic pericarditis (in approx. 23.1%)

4. pericarditis in diseases of the surrounding organs:

• Myocarditis ( in about 30%)
• Pericarditis epistenocardica (occurs in larger infarcts near the epicardium within one week after the acute event [Herold 2018])
• Pneumonia (rare)
• Pulmonary infarction (rare)
• Aortic aneurysm (rare)
• Myocarditis (rare)
• Esophageal diseases (rare)
• paraneoplastic pericarditis (rare)
• Hydropericardium in decompensated heart failure (rare)

5. pericarditis associated with metabolic diseases such as:

• Myxedema (in about 30%)
• Uraemia (no precise incidence data available)
• diabetic ketoacidosis (rare)
• Addison's disease (rare)
• Pregnancy (rare)
• Cholesterol pericarditis (very rare)

6. traumatic conditional pericarditis

• by direct injuries penetrating the thorax, foreign bodies, esophageal perforation (rare)
• by indirect injuries not penetrating the thorax or after mediastinal radiation (rare)

7. tumorous pericardial diseases (affects about 35% of the diseases)

• secondary metastatic tumours (frequent)
• primary tumours (rare)
• Bronchial carcinoma ( in approx. 40%)
• Breast carcinoma ( in approx. 22%)
• Leukemia and lymphoma (in approx. 15%)
• Sarcoma ( in about 4%)
• Melanoma ( in about 3%)
• tumours among others

8. drug-induced pericarditis (occurs very rarely)

• this form of pericarditis is found, for example, after taking penicillin; sometimes it is accompanied by eosinophilia as a sign of a hypersensitivity reaction (Herold 2018)

Acute pericarditis can begin either acutely or insidiously. In the case of an infectious genesis, the onset is usually sudden.

In some cases, the following prodromas occur about 2-3 weeks before:

• infection of the gastrointestinal tract
• common cold
• Infections of the respiratory tract

Otherwise, the following symptoms exist:

• circumscribed left thoracic pain
• Occasionally, pain radiates into the left shoulder, left arm and neck
• sometimes there is also isolated pain along the upper trapezius margin (pathognomonic for irritation of the pericardium)
• the pain is reduced when the posture is bent forward
• the pain increases when lying down, during deep inspiration, occasionally also when coughing or swallowing
• (Cool 2004)
• Mostly there is tachycardia
• subfebrile or febrile temperature rise are possible
• Lassitude
• general morbidity

The pain, however, can be completely absent in slowly developing pericarditis (e.g. neoplastic or tuberculous).

According to the ESC Guideline of 2015, acute pericarditis can be diagnosed if 2 of the following criteria are met:

• chest pain
• Friction noise
• ST-segment elevation
• Pericardial effusion

In cases of tuberculous or uremic etiology there is usually a gradual onset of unspecific symptoms such as:

• Adynamics
• unknown fever
• Dyspnea
• Weight loss

Acute pericarditis can occur at the beginning or end of the course as so-called dry or fibrinous pericarditis. This is more common in pericarditis caused by uremia or myocardial infarction.

Wet or exudative pericarditis is most common in pericarditis caused by tuberculosis, viral infections, rheumatic fever and pericarditis caused by uremia. When the dry form changes to an exudative form, heart sounds often become quieter and pericardial chafing and pain may disappear (Herold 2018).

If the pericarditis is infectious, CRP, BSG and leukocytes may increase. In this case, virus serology and cultures for bacteria and mycobacteria are recommended (Herold 2018). Otherwise the following laboratory changes may occur (Fritze 2012):

• Troponin I/T increased
• CK-MB increased
• Myoglobin increased
• occasionally also TNF (tumour necrosis factor) increased

If the inflammatory processes spread to the myocardium, there may be elevated levels (Erdmann 2009):

• Creatine kinase
• Isoenzymes

Clinic (see ESC guidelines)

Auscultation:

• Pulse-synchronous systolic-diastolic, creaking, near-auricular sound, most evident over the lingula near the sternum (so-called pericardial rubbing).
• Pericardial rubbing may sometimes be present only passively
• intensifies during inspiration
• no change in sound during a pause in breathing (in contrast to pleural rubbing)
• Absence of pericardial rubbing does not exclude pericarditis!

The ECG does not have to be fundamentally altered by pericarditis. Rather, the outer layer damage that usually exists in all leads results from inflammation of the adjacent myocardial layers (Herold 2018).

However, ECG changes do occur in a large proportion of patients (90%). However, these occur hours or days after the onset of the acute phase (Erdmann 2009).

In these changes, concave ST-segment elevations from the ascending S-wave are found on the ECG in the initial stage. These changes can be followed over several leads, and the leads cannot be assigned to any coronary supply area.

Occasionally, peripheral/central low-voltage is also found (Maisch 2008).

Erdmann (2009) divides the changes in the ECG into 4 phases. In 50% of cases, all 4 phases occur in a patient and can then be evaluated as a clear indication - with otherwise negative examination results - of acute pericarditis. Changes in the PQ stretch are found in 80% of cases.

ECG changes in I, II, aVL, aVF, V3-V6

Phase 1: J-point increased T-waves upright PQ stretch decrease possible

Phase II: o.b. T waves flat to negative PQ segment depression possible

Phase III: o.b. T-waves negative o.b.

Phase IV: o.B. T-waves upright o.B.

ECG changes in aVR, V1 and occasionally V2:

Phase 1: ST-segment decreased T-wave negative PQ-segment decrease possible

Phase II: o.b. T waves flat to positive PQ stretch depression possible

Phase III: o.B. T-waves positive o.B.

Phase IV: o.B. T-waves negative o.B.

(Erdmann 2009)

In case of large pericardial effusion (so-called swinging heart), electrical alternans may occur due to the rhythmic amplitude change (Maisch 2008).

Echocardiography

Echocardiography is the simplest and also the most sensitive method to detect or also to exclude pericarditis. Likewise, the hemodynamic relevance can be well assessed in case of an effusion (from 50ml echocardiographically detectable).

Cave: The anechoic space behind the heart and, in the case of large effusions, also in front of the heart must be taken into account (Herold 2018).

The accompanying pericardial effusion may often be absent in viral genesis. In bacterial and tuberculous genesis, however, a regular effusion of varying extent is found [Kühl 2004]).

The extent of the effusion is further subdivided echocardiographically according to Maisch (Maisch 2008) into:

• small effusion (the anechoic diastolic separation of peri- and epicardium is < 10 mm)
• moderate effusion (the diastolic separation is between 10mm - 20 mm)
• large effusion (diastolic separation is more than 20 mm)
• very large effusion (diastolic separation is more than 20 mm and there are additional signs of compression).

In order to assess the development of pericardial effusion, we recommend:

• close monitoring of blood pressure (blood pressure drops)
• regular CVD measurements (CVD increases)
• Echocardiography checks

(Herold 2018)

Chest X-ray

There is enlargement of the cardiac shadow on x-ray without signs of pulmonary congestion. The heart assumes a so-called box-bag shape with a strongly protruding midsection.

Echocardiography is recommended to rule out myogenic cardiac dilatation as a differential diagnosis (Herold 2018).

Computed tomography

In uncomplicated acute pericarditis, computed tomography is not required for diagnosis (Erdmann 2009).

CT allows accurate quantification of the effusion.

It is even superior to echocardiography in visualizing anteriorly located local effusions. The thickness of the pericardial wall can also be more accurately visualized here, as can calcifications of the pericardium.

Due to the different density values, it is possible to differentiate between hemorrhagic and serous pericardial effusions with this type of examination.

(Maisch 2008)

Cardiac magnetic resonance imaging

Cardiac magnetic resonance imaging is also not essential for diagnosis in uncomplicated acute pericarditis (Erdmann 2009).

However, if there is any uncertainty regarding the diagnosis or cause of pericarditis, cardiac magnetic resonance imaging has the advantage over computed tomography in that a contrast agent (gadolinium) can also be administered. In this way, it is possible to visualize pericardial inflammatory regions by demonstrating a "late enhancement."

(Maisch 2008)

Pericardiocentesis / Pericardial Biopsy

In uncomplicated acute pericarditis, a pericardial biopsy is not necessary. However, if an underlying disease is suspected that requires further therapy (e.g., tuberculosis, neoplasia, infectious pericarditis), such procedures may be useful. The diagnostic benefit of pericardiocentesis is 39% and that of pericardial biopsy is 54%, provided that these procedures are performed to relieve pericardial tamponade. The success of these interventions drops to 14% when performed exclusively in patients with pericardial effusion and a one-week minimum duration of disease (Erdmann 2009).

• acute myocardial infarction (in perimyocarditis, both Q-spikes and R-loss are absent; in infarction, reciprocal ST-slopes are found, which are absent in perimyocarditis; however, CK may also be slightly elevated in perimyocarditis)
• myogenic cardiac dilatation (in myogenic cardiac dilatation there are no sonographic signs of effusion and no low voltage ECG; instead, signs of pulmonary congestion are often found)

• Pericardial tamponade
• recurrent episodes (occur in 20%-30%)
• Fibrosis or calcification of the pericardium with development of constrictive pericarditis

In pericarditis caused by a myocardial infarction, no anticoagulants should be administered, as there is then a risk of hemopericardium (Herold 2018)!

1. primary treatment of the underlying disease e.g.

• in case of bacterial genesis: antibiotics according to the determination of resistance. However, this alone is usually not sufficient. The infection itself can only be controlled by early surgical opening of the pericardium with the application of a suction-irrigation drainage. Drainage alone, even with instillation of antibiotics, does not lead to the desired success. This is of particular importance considering the high mortality rate of up to 50 % (Paumgartner 2015).
• In this case, an attempt should be made to detect the pathogens in the pericardial point. However, since a negative result does not rule out tuberculosis-related pericarditis, anti-tuberculosis therapy should be started immediately if clinical suspicion exists (see also pericarditis, chronic).
• in case of rheumatic fever, penicillin plus ASA or possibly corticosteroids should be given
• in the case of uremic pericarditis, dialysis or other appropriate therapeutic measures are recommended (see also pericarditis, chronic )
• for allergic pericarditis, post-myocardial infarction or post-cardiotomy syndrome, non-steroidal anti-inflammatory drugs and possibly corticosteroids are indicated.

2. symptomatic treatment

• First-line therapy: high dosage of ASS and NSAIDs (e.g. ibuprofen 600mg every 8 h) plus (as stomach protection) proton pump inhibitor plus colchicine 0.5 mg/d (to reduce the recurrence rate)
• Indication for corticosteroid therapy exists in:

- failure of first-line therapy and

- the exclusion of an infectious genesis

- in autoimmune diseases

In 15% - 30%, recurrent attacks of acute pericarditis may occur at intervals of weeks or months. This can occur with or without pericardial effusion. The cause remains unclear in most cases, but in most cases this form of pericarditis is benign (Kühl 2004).

In only about 1% of cases is there a transition to constrictive pericarditis (Herold 2018).

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