Pericarditis I31.9

Galen was probably the first to describe constrictive pericarditis. As early as 190 AD, Galen observed a thickening of the pericardium in animals and considered it possible in humans as well (Gall 1992).

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 s.d. (Erdmann 2009).

Pericarditis is an infectious or non-infectious acute or chronic inflammation of the pericardium, often accompanied by pericardial effusion (so-called exudative pericarditis), in contrast to pericarditis sicca, which is accompanied by no effusion (Maisch 2008).

In most cases, subepicardial myocardial layers are also involved in the inflammatory process.

The disease begins as so-called acute pericarditis. This acute form can develop into chronic, constitutional or recurrent pericarditis (Hombach 2009).

A distinction is made according to their clinical course between:

• acute pericarditis
• chronic pericarditis
• constrictive pericarditis
• recurrent pericarditis

According to Fritze (Fritze 2012), acute pericarditis is subdivided into the following forms - regardless of the aetiology:

• dry pericarditis (so-called sicca pericarditis 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 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 pericarditis are very different. Over 50% are idiopathic, about 30% - 50% infectious, the other causes are rather rare.

We distinguish between the following forms:

1. Infectious pericarditis:

Infective 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. pericarditis 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%)
• Acute myocardial infarction (in approx. 5% - 20%)
• 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 ingestion of penicillin (see also penicillin allergy); sometimes it is accompanied by eosinophilia as a sign of a hypersensitivity reaction (Herold 2018)

Patients primarily complain of retrosternal pain, often dependent on breathing (especially in the acute form). However, the pain may be completely absent in slowly developing pericarditis (e.g. neoplastic or tuberculous). With tuberculous or uremic etiology there is usually a gradual onset with unspecific symptoms such as

• Adynamics
• unknown fever
• Dyspnea
• Weight loss

The other symptoms depend on the type of pericarditis and are therefore described in more detail under the respective articles.

Chest X-ray: There is an enlargement of the cardiac shadow in the X-ray without signs of pulmonary congestion. The heart takes on 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: CT allows accurate quantification of the effusion. Initially, the diagnosis is based on the following findings:

• clinical picture
• typical changes in the ECG
• Echocardiography
• According to the ESC guideline of 2015, the diagnosis "acute pericarditis" can be made when 2 of the following criteria are met:
• Chest pain
• Friction murmur
• ST elevation
• Pericardial effusion

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

Cave: The anechoic space behind the heart and in case of large effusions also in front of the heart has to be considered (Herold 2018).

The extent of the effusion is further subdivided echocardiographically into:

• small effusion (the anechoic diastolic separation of peri- and epicardium is < 10 mm)
• moderate effusion (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 controls: In the case of constrictive pericarditis, echocardiography can be used to determine the thickness of the pericardium. This is thickened to > 3 mm in the case of constrictive pericarditis .It is even superior to echocardiography in visualizing anteriorly located local effusions. The thickness of the pericardial wall can also be visualized more accurately, 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.

Troponin I/T increased

CK-MB increased

Myoglobin increased

occasionally also TNF (tumour necrosis factor) increased

If the pericarditis is infectious, CRP and SPA may increase. In this case, virus serology and cultures for bacteria and mycobacteria are recommended (Herold 2018).

If the inflammatory processes spread to the myocardium, CRP and STL may be elevated:

• Creatine kinase
• Isoenzymes

Cardiac magnetic resonance imaging

Computer tomography and cardiac magnetic resonance imaging are the two best imaging techniques for the diagnosis and clarification of the cause of pericarditis. Since cardiac magnetic resonance imaging allows the administration of a contrast medium (gadolinium) in addition to computed tomography, pericardial inflammatory regions can also be visualized in this way by detecting a "late enhancement" (Maisch 2008).

Initially the diagnosis is based on the following findings:

• clinical picture
• typical changes in the ECG
• Echocardiography

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

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

Auscultation: Pulse-synchronous systolic-diastolic, creaking noise close to the ear, most clearly above the lingula near the sternum (so-called pericardial rubbing). Pericardial rubbing may also be present only passagally

• intensifies with the inspiration
• no change in noise during a respiratory pause (in contrast to pleural rubbing)
• missing pericardial rubbing does not rule out pericarditis!

ECG: The ECG does not have to be fundamentally changed by the pericarditis. The damage to the outer layer, which is usually present in all leads, is rather caused by inflammation of the adjacent myocardial layers (Herold 2018).

If the ECG is altered, concave ST segment elevations from the ascending S-wave can be found in the initial stage. These changes can be followed over several leads, whereby the leads cannot be assigned to any coronary artery supply area.

Sometimes a peripheral / central low voltage is also found.

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

(Maisch 2008)

For details on ECG changes see also acute pericarditis

Pericardiocentesis / pericardial biopsy: If an underlying disease is suspected that requires further therapy (e.g. tuberculosis, neoplasia, infectious pericarditis), such interventions may be useful. The diagnostic benefit of pericardiocentesis is 39% and that of pericardial biopsy 54%, if these interventions are performed to relieve a pericardial tamponade. The success of these interventions drops to 14% if they are performed exclusively on patients with pericardial effusion and a one-week minimum duration of the disease (Erdmann 2009).

Other: The other diagnostic findings depend on the type of pericarditis and are therefore described in more detail under the respective articles.

• Acute myocardial infarction (in perimyocarditis, both Q-spikes and R-loss are absent; in infarction, reciprocal ST depressions 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)

(Herald 2018)

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

(Erdmann 2009)

As the therapeutic measures depend on the cause and course of pericarditis, these are described in detail in the respective article.

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

The acute disease heals without consequences in about 70% - 90% of cases. However, despite sufficient therapy in the acute phase, relapse occurs in about 15% - 30% of patients (Hombach 2009).

The transition to chronic constrictive pericarditis is rare (approx. 1% [(Herold 2018]).

The prognosis of the chronic form of the disease and also of constrictive pericarditis is rather unfavourable.

The pericardium normally consists of a double-layer pouch divided into a visceral and a parietal membrane. Between the two membranes there is a so-called ultrafiltrate with a liquid quantity of between 15 - 50 ml.

The pericardium prevents sudden dilatation of the heart cavities under stress and also in hypovolemia, preferably in the area of the right atrium and right ventricle.

It is also believed that the pericardium can protect the heart from infections from the lung and/or pleural cavity.

On the other hand, however, it is also possible that the pericardium is congenitally absent or is missing due to surgery, without a clear clinical disease.

However, partial defects in the area of the left ventricle may result in a protrusion of the left atrium and the main trunk of the pulmonary arteries. In very rare cases it is possible that the left atrium is strangled by a hernia formation and then causes sudden cardiac death (Kasper 2015).

1. Erdmann E (2009) Clinical Cardiology: Diseases of the heart, the circulation and the vessels near the heart. Springer publishing house. 342
2. Fritze J et al. (2012) The medical review: legal issues, functional tests, assessments. Springer publishing house 371-376
3. Gall F P et al (1992) Progress in surgery in the last decade. Langenbeck's Archive for Surgery. 109th Congress of the German Society for Surgery. Springer Verlag. 488
4. Herold G et al (2018) Internal Medicine Herold Verlag 235-237
5. Hombach V et al (2009) Cardiovascular Magnetic Resonance Imaging: Atlas and DVD. Schattauer Publishing House 161-163
6. Kasper D L et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 1571-1577
7. Kasper D L et al (2015) Harrison's Internal Medicine. Georg Thieme Publisher 1912-1918
8. Krombach G A et al. (2015) Radiological Diagnostics Abdomen and Thorax: Image interpretation considering anatomical landmarks and clinical symptoms. Georg Thieme publishing house. 4.2.2.2
9. Maisch B et al. (2008) New possibilities for the diagnosis and therapy of pericarditis. The internist (49) Springer Verlag 17-26