Chronic pericarditis is long-lasting, gradual inflammation of the pericardium, causing accumulation of fluid in the pericardial space or thickening of the pericardium. The chronic varieties of pericarditis are rare. Chronic pericarditis will usually be preceded by acute pericarditis (although acute pericarditis is more common and usually self-limiting).

There are two main types encompassed by this term:

• Chronic effusive pericarditis
• Chronic constrictive pericarditis

Effusive-constrictive pericarditis (persistence of symptoms and signs of constrictive pericarditis after removal of pericardial fluid) suggests a clinical continuum initiated by acute pericarditis and progressing through pericardial effusion, chronic effusive pericarditis, effusive-constrictive pericarditis to chronic constrictive pericarditis.¹

Anatomy

The normal pericardium has two layers (outer fibrous pericardium and inner serous pericardium). There are approximately 50 ml of fluid in the intrapericardial space or pericardial cavity, i.e. the space between the serous pericardium next to the heart and the serous pericardium next to the fibrous pericardium.

The pericardium:

• Helps cardiac efficiency by limiting dilatation, aids atrial filling, etc.
• Protects the heart by reducing external friction and providing a barrier to extension of infection and malignancy.
• Fixes the heart anatomically through ligamentous connections.

Aetiological factors

The range of aetiologies should be considered alongside prevalence as many on the list are possible but unlikely diagnoses:

• Idiopathic:
  • It is common for no antecedent diagnosis to explain the inflammatory process.
  • Unrecognised viral pericarditis (Coxsackie virus, echovirus and adenovirus) may explain some of these, which constitutes the most common aetiological factor for all froms of pericarditis (both acute and chronic), particularly in developed countries.
• Infection:
  • Infection can be viral, bacterial, fungal or parasitic.
  • Tuberculosis is the main cause of constrictive pericarditis in developing nations but is less common in developed countries.
• Inflammation:
  • Many different inflammatory conditions can affect the pericardium.
  • Between 11% and 50% of patients with rheumatoid arthritis have pericarditis at autopsy.
  • Systemic lupus erythematosus (SLE), scleroderma, sarcoidosis, Wegener's granulomatosis, and others can all cause chronic pericarditis.
• Metabolic:
  • Renal failure (35-50% of patients with uraemia, pre-dialysis have pericarditis).
  • Hypothyroidism.
  • Cholesterol pericarditis (gold paint pericarditis).
• Cardiovascular disease:
  • Myocardial infarction causes acute pericarditis (transmural infarcts, persistent ST elevation).
  • Dressler's syndrome (2-3 weeks after myocardial infarction).
  • Aortic dissection.
• Neoplastic:
  • 5-17% of pericarditis.
  • This is caused mostly by metastatic disease (lung 33%, breast 25%, haematological 15%).
• Miscellaneous:
  • Drugs: doxorubicin; cyclophosphamide; drug-induced SLE (methyldopa, isoniazid, hydralazine); methysergide; smallpox vaccination; dantrolene, phenytoin; minoxidil.
  • Irradiation
  • Post-pericardiotomy syndrome (i.e. after cardiac surgery 10-40%).
  • Trauma, especially with oesophageal rupture or pancreatitis.

Chronic effusive pericarditis

Aetiology

This is most commonly idiopathic but can follow any of the causes of acute pericarditis, particularly malignancy (most commonly breast and lung), tuberculosis (TB) - more common in developing countries - or hypothyroidism.

Presentation

• Variable but dyspnoea on exertion, particularly in severe cases.
• Cardiovascular symptoms include: chest pain, pressure or discomfort; syncope, light headedness; palpitations.
• Many patients are asymptomatic until the disease is advanced (up to 2 L of fluid can build up).
• Respiratory symptoms, including cough and hoarseness, may occur.
• Other symptoms include fatigue, hiccoughs, anxiety and confusion.
• Attention should be given to any history of specific antecedent conditions (e.g. cardiac surgery, renal failure, radiation treatment, malignancy, TB).

Signs may include hypotension, elevated jugular venous pressure (JVP), and diminished heart sounds (Beck's triad or acute compression triad described in 1935 with cardiac tamponade). Signs may develop towards those of cardiac tamponade (Beck's triad, pulsus paradoxus, Kussmaul's sign, etc). Cardiac dullness is an unreliable sign. Pericardial friction rub is best heard in the supine position at the end of exhalation. Hepatojugular reflux may be observed.

Fluid (and hence signs) usually develop slowly with cardiac tamponade developing late as a complication.

Chronic constrictive pericarditis

Aetiology

These parallel those of antecedent acute pericarditis and hence are many and varied.
The more common causes of constrictive pericarditis are:

• Idiopathic.
• Viral.
• TB (the highest total incidence, as common in developing countries).
• Mediastinal irradiation (5-10 years later).
• Post-surgical (including cardiac catheterisation).

Less common causes are:

• Other infections.
• Neoplasms.
• Uraemia.
• Connective tissue disorders.
• Drugs.
• Trauma.
• Cardiovascular disease.

Other aetiologies are rare - hereditary, chemical trauma, etc.

Presentation

Typically, there is a very gradual onset (usually months, occasionally days). The pericardium becomes thickened and fibrotic (and later 'eggshell' calcification is visible on CXR in ~27%):²

• In the early stages signs are subtle and easily missed.
• In advanced disease the patient may be ill with jaundice, cachexia and muscle wasting.
• Similar to right heart failure, commonly dyspnoea (which may be relatively slight), peripheral oedema, JVP elevated (classically with a prominent y descent (Friedreich's sign), and doesn't fall with inspiration (Kussmaul's sign).
• Additionally, there may be pulsatile hepatomegaly (in as many as 70% of patients), reduced apical impulse, a pericardial 'knock' (early diastolic sound) and pulsus paradoxus.³
• A patient with intense venous congestion, but no heart enlargement or valvular disease, should lead one to suspect constrictive pericarditis
• Gastrointestinal symptoms are secondary to this venous congestion.

The heart is not enlarged. ECG may be low voltage with nonspecific T-wave changes.

Differential diagnosis

Chronic constrictive pericarditis

The main differential diagnosis is restrictive cardiomyopathy.⁴

Others include:

• Dilated cardiomyopathy
• Pericardial effusion
• Cardiac tumours

Chronic effusive pericarditis

• Cardiac tamponade
• Dilated cardiomyopathy
• Constrictive pericarditis
• Constrictive-effusive pericarditis
• Pulmonary oedema

Investigations

• Echocardiogram (tissue Doppler imaging and colour M-mode echocardiography) is usually diagnostic and helps distinguish from restrictive cardiomyopathy (in the former, myocardial relaxation is normal and diastolic dysfunction is due to impaired compliance and limited cardiac diastolic volume).
• Magnetic resonance imaging (MRI) can estimate thickness of the pericardium. Late enhancement techniques may be particularly effective.⁵
• Cardiac catheterisation is sometimes used to measure pressures.
• Occasionally, pericardial biopsy, especially if infective, malignant or granulomatous causes are suspected.

In the future, devices may be developed to access the pericardial space, even in the absence of effusion. These may improve future diagnostic and therapeutic options.⁴

Management

Treat the cause where possible.

Chronic effusive pericarditis

Usually, a conservative approach, if neither haemodynamically compromised nor too symptomatic. Otherwise, surgical drainage may be required (particularly if infection is suspected).⁶

Constrictive pericarditis

• Medical: bed rest, diuretics and salt restriction can relieve symptoms.
• Surgical: pericardiectomy is curative in most cases (85%), but mortality from the procedure is significant (5-15%).⁷

Prognosis

Prognosis is strongly linked to the underlying cause but long-term survival is more likely with surgery and the best results are achieved if surgery is offered early.⁸The surgical results are poor in patients with:⁶

• Organ failure (e.g. renal and hepatic particularly).
• Ascites.
• Untreated coronary artery disease.
• Old age.
• New York Heart Association (NYHA) class IV heart failure symptoms.
• Post-radiation pericarditis.
• Myocardial fibrosis.

Historical notes

• Richard Lower (notable for the first human blood transfusion in England in 1667) described a patient with dyspnoea and intermittent pulse, in 1669.
• Lancisi (notable for suggesting the connection between malaria and mosquitoes) reported on the constrictive syndrome in 1828.
• Corrigan is credited with the pericardial knock in 1842.
• Kussmaul published his paper on the paradoxical pulse in 1873.³
• Franz Volhard collaborated with Viktor Schmieden in 1923, leading to the first pericardiectomy for constrictive pericarditis.

Document references

1. Sagrista-Sauleda J, Angel J, Sanchez A, et al; Effusive-constrictive pericarditis. N Engl J Med. 2004 Jan 29;350(5):469-75. [abstract]
2. Troughton RW, Asher CR, Klein AL; Pericarditis. Lancet. 2004 Feb 28;363(9410):717-27. [abstract]
3. Bilchick KC, Wise RA; Paradoxical physical findings described by Kussmaul: pulsus paradoxus and Kussmaul's sign. Lancet. 2002 Jun 1;359(9321):1940-2.
4. Yazdani K, Maraj S, Amanullah AM; Differentiating constrictive pericarditis from restrictive cardiomyopathy. Rev Cardiovasc Med. 2005 Spring;6(2):61-71. [abstract]
5. Taylor AM, Dymarkowski S, Verbeken EK, et al; Detection of pericardial inflammation with late-enhancement cardiac magnetic resonance imaging: initial results. Eur Radiol. 2006 Mar;16(3):569-74. Epub 2005 Oct 25. [abstract]
6. Tuna IC, Danielson GK; Surgical management of pericardial diseases. Cardiol Clin. 1990 Nov;8(4):683-96. [abstract]
7. Khandaker MH, Espinosa RE, Nishimura RA, et al; Pericardial disease: diagnosis and management. Mayo Clin Proc. 2010 Jun;85(6):572-93. [abstract]
8. Sidney DS et al; Pericarditis, constrictive, eMedicine, Jul 2008

Internet and further reading

• Lorusso R, Gelsomino S; El corazon espinado. Lancet. 2009 Nov 7;374(9701):1626.
• Purvis JA, Roberts M, Harbinson MT; Constriction infection. Int J Cardiovasc Imaging. 2010 Jan;26(1):1-3. Epub 2009 Sep 23. [abstract]

Acknowledgements