Download - Pericarditis - Orphanet · PDF file · 2005-03-11neoplastic pericardial effusions may also present with supraventricular arrhythmias, or features of constrictive pericarditis. Generally,

Pericarditis Authors: Doctor Sabiha Gati BSc (Hons), MBBS and Doctor Sanjay Sharma BSc (Hons), MBChB, MRCP (UK), MD1

Creation Date: March 2005

Scientific Editor: Professor William McKenna 1 University Hospital Lewisham, Lewisham High Street, London SE13 6LH. [email protected]

Abstract Keywords Background Definition and Classification Frequency ACUTE PERICARDITIS Clinical manifestation Other investigations Management Complications PERICARDIAL EFFUSION CARDIAC TAMPONADE Clinical Manifestations Investigations Management CONSTRICTIVE PERICARDITIS Clinical manifestations Investigations Management References Abstract Pericarditis is an inflammatory disorder of the serous pericardium resulting from a primary insult to the heart or is secondary to a systemic disorder. Of the many causes, the most frequently encountered include acute idiopathic pericarditis and viral infections. The condition is classically diagnosed by the presence of chest pain, presence of a pericardial friction rub and characteristic changes on ECG. Extensive investigations to elicit a cause are not necessary as they are of low diagnostic yield. Because of its frequently self-limiting nature, non-steroidal anti-inflammatory drugs are normally used as the first line treatment with the aim of dampening the inflammatory process and expediting recovery. Specific therapy should be initiated for an underlying disorder perpetuating pericarditis. Complications of pericarditis include pericardial effusions and subsequent tamponade and long term constrictive pericarditis. Further laboratory evaluation, echocardiography and pericardiocentesis should be considered for individuals likely to have these complications.

Keywords Pericarditis, pericardial effusion, pericardial constriction

Background The heart is surrounded by a protective pericardium made up of two layers, a serous and a fibrous component. The serous component is further divided into an inner, visceral and outer, parietal layer. A potential space, the pericardial cavity exists between

the two layers of the serous pericardium that normally contains 15 to 50 mls of plasma fluid (1).

Definition and Classification Pericarditis is an inflammatory disorder of the serous pericardium which may result from either a primary insult directly to the heart or be secondary to a large number of systemic disorders (Table 1). In most

Gati S., Sharma S. Pericarditis. Orphanet Encyclopedia, March 2005. http://www.orpha.net/data/patho/GB/uk-Pericarditis.pdf

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instances the disorder is self-limiting but may be complicated by pericardial effusion or constriction.

Table 1: Diseases affecting the pericardium. Adapted from (1). Trauma Myocardial Infarction

Radiotherapy Aortic dissection Blunt or penetrating injury

Infection Bacterial – Tuberculosis, Streptococcus, Staphylococcus, Pneumococcus, Haemophilus, Legionella, Salmonella, Lyme disease, Neisseria meningitides & Gonorrhoea, Chlamydia psittaci & Trachomotis Viral – HIV, Coxsackie virus A&B, echovirus, EBV, mumps, hepatitis B, influenza, varicella Fungal – Histoplasmosis, aspergillosis, nocardia, candida, coccidiomycosis Parasitic – Echinococcus, amebiasis, toxoplasmosis

Neoplasm Primary or secondary Drugs Hydralazine, Procainamide, Cytotoxics,

Phenytoin, Penicillin, Anticoagulants Endocrine Hypothyroidism Metabolic Uraemia, Amyloidosis Inflammatory Dressler’s syndrome, Postcardiotomy Connective Tissue disorders

RA, SLE, SS, PAN, Churg-strass, GCA, sarcoidosis, IBD, wegener’s granulomatosis

Acute Idiopathic

HIV= Human immunodeficiency virus, EBV= Ebstein bar virus, RA= Rheumatoid arthritis, SLE= Systemic lupus erythematosus, SS= Systemic sclerosis, PAN= Polyarteritis nodosa, GCA= giant cell arteritis, IBD= inflammatory bowel disease.

Frequency Epidemiological data on the incidence of pericarditis are scarce, possibly because the condition can be difficult to identify, and of its self-limiting nature. The frequency of pericarditis can vary in numerous disorders (Table 2). Mortality may vary according to the aetiology, being low with viral/idiopathic pericarditis and very high with purulent pericarditis (2).

Table 2: Frequency of the disorder under different causes of pericarditis. Adapted from (14) Cause Accountable cases Infection Bacterial 1-8% Tuberculosis 4% Viral 1-10% Autoimmune Rheumatoid arthritis 11-50% Systemic lupus erythematosus

25% (after autopsy 62%)

Systemic sclerosis 5-10%(after autopsy 70%)Chronic Renal failure 12% Hypothyroidism 4% Myocardial infarction 7-23% Dressler’s syndrome 4% Postcardiotomy 10-40% Neoplasm 5-17%

ACUTE PERICARDITIS Aetiology

The cause of pericardial disease in many cases is unknown, particularly in young adults. Studies suggest a viral aetiology (coxsackie B) in most cases. Since the disease is usually self-limiting the cause is not investigated as there is a considerable time lag before the results of the viral titre are available and the initiation of treatment. The diagnosis can be made from the clinical presentation and does not require positive virology titres. Individuals may present with chest pain and flu-like symptoms. Although the condition is self-limiting, recurrence over the following 6 to 12 months may occur with an immunological basis.

Viral pericarditis Coxsackie virus A & B, echovirus, adenovirus and HIV are the most commonly implicated viral causes of acute pericarditis. Seasonal epidemics are known to occur with influenza and coxsackie B. Viral pericarditis is typically self-limiting, lasting 1-3 weeks and is treated symptomatically. HIV can facilitate infection of the pericardium by non-virulent organisms or can directly infect the pericardium. HIV typically causes small asymptomatic pericardial effusions.

Bacterial pericarditis The most common bacterial causes are Staphylococcus aureus, Streptococcus pneumonie, Haemophilus influenzae and Mycobacterium tuberculosis. The spread is either haematogenous or directly from the lungs or pleura. Less common bacterial infections may be seen in the immunocompromised and in those with altered bacterial flora from extensive prolonged antibiotic therapy.

Tuberculosis pericarditis Tuberculous pericarditis is rare in the western world, however, it is still an important cause of pericardial disease in the third world. Both parietal and visceral layers of pericardium are infected and the myocardium is involved in most cases. Tuberculous pericarditis can present in the form of acute pericarditis, pericardial effusion or with constrictive pericarditis. Treatment is with antituberculous drugs. Steroids hasten recovery in the first 11 weeks but should be avoided in tuberculous pericarditis secondary to HIV syndrome.

Fungal infection Fungal pericarditis is rare and is usually manifest in immunocompromised patients. Histoplasma is the most common fungal organism in the immuno-sufficient individuals and is known to cause constriction and calcification. Infections with actinomycosis (Actinomyces), coccidiodomycosis (Coccidioides), Candida and Aspergillus have also been reported.

Malignancy Neoplastic disorders affecting the pericardium include rare primary tumours such as mesotheliomas or myosarcomas or, more commonly are secondary to metastases from the lung, breast, gastro-intestinal tract and haematological malignancies. Malignancy is frequently associated with moderate to large effusions (2,3), and may lead to tamponade. Patients with


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neoplastic pericardial effusions may also present with supraventricular arrhythmias, or features of constrictive pericarditis. Generally, malignant pericardial effusions require drainage. The diagnosis of neoplastic pericardial effusion is based on cytological examination of pericardial fluid or direct histological tests on the pericardium (4,5). Recurrent effusions are treated by surgical fashioning of a continuum window into the pleural cavity.

Hypothyroidism Hypothyroidism causes silent pericardial effusion that is rarely of haemodynamic significance. Low voltage complexes are seen on the ECG. Thyroxine replacement alone is sufficient to resolve the pericardial effusion.

Myocardial infarction Acute pericarditis and/or effusion, are manifested in approximately 15% of patients following an myocardial infarct (MI) (5,6,7,8). The overall incidence of MI induced pericardial disease is reduced by 50% with thrombolytic therapy (7). Infarct size and early initiation of thrombolytic therapy determines the incidence and extent of pericardial involvement (7). The acute insult to the pericardium presents in the first 24 to 72 hours, and gives rise to a friction rub, dull retrosternal chest pain varying with posture and respiration and pericarditis, widespread ST elevation is labile. Patients are also more prone to supraventricular arrhythmia. An acute effusion may develop secondary to the inflammatory process related with the infarct but is usually silent.

Dressler’s syndrome Dressler’s syndrome is seen in 3 to 4% of patients and occurs 2 to 4 weeks following a myocardial infarction. Also known as postcardiac injury syndrome, it is immunologically mediated and presents with a self-limiting febrile illness, pleural or pericardial pain and a pneumonitis. Patients with successful reperfusion after treatment with thrombolysis following MI are less likely to develop Dressler’s syndrome (9).

Metabolic cause of pericarditis Patients with untreated severe chronic renal failure are prone to uraemic pericarditis. Uraemic pericarditis is often complicated by pericardial effusion (9,10). The typical electrocardiogram (ECG) changes of pericarditis are not seen, however, patients do develop pericardial pain and friction rub. The incidence of uraemic pericarditis is very rare in areas where dialysis is readily available.

Autoimmune disorders Patients with rheumatoid arthritis, systemic lupus erythematosus and other connective tissue disorders (Table 1) can develop extensive pericardial disease. Clinically signs of pericardial involvement are often absent and the ECG rarely demonstrates typical changes of pericarditis. Treatment is recommended on symptomatic grounds.

Drug induced pericardial disease Certain drugs such as procainamide and hydralazine may precipitate a lupus like syndrome involving the pericardium. Other drugs implicated in the aetiology of pericarditis include isoniazid and phenytoin. Cytotoxic drugs such as doxarubicin and daunorubicin cause pericardial and cardiac muscle disease. The

pericardium may also be involved in allergic hypersensitivity reactions to penicillins.

Clinical manifestation Clinical presentation of acute pericarditis consists of a triad of chest pain, pericardial friction rub and generalised widespread ST segment and T wave changes on the ECG.

Chest pain The onset of the pain is sudden. The pain is classically located in the retrosternal area and characteristically sharp in nature with exacerbation during movement or deep inspiration. The intensity of the pain decreases with sitting forward. Dyspnoea is the consequence of the pain aggravated by breathing.

Pericardial friction rub Precordial examination of a patient with acute pericarditis often reveals a scratchy superficial sound audible over the precordium, in any position termed a “pericardial rub”. The rub can be fleeting and may vary with posture and is best heard with the diaphragm of the stethoscope at the left sternal border. A pericardial rub can be distinguished from a pleural rub by halting respiration for a few seconds. The pericardial rub is an effect of maximal movement of the heart within the pericardial sac.

Electrocardiogram (ECG) There are four stages to the ECG changes in acute pericarditis: Stage 1: Diffuse symmetrical concave ST segment elevation by 1mm or more (Fig 1). This is associated with reciprocal ST segment depression in aVR and V1. There is also elevation of the PR segment in aVR and reciprocal depression in the left chest leads and remaining limb leads (1). Stage 2: ST and PR segment normalisation. Stage 3: Diffuse T wave inversions occurring over 2 to 3 weeks. This stage is not always seen in all patients. Stage 4: Normalisation of the ECG. Persistence of T wave inversion is indicative of chronic pericarditis. Arrhythmias are generally uncommon in isolated acute pericarditis (11) but their presence should raise the suspicion of concomitant myocardial disease.

Other investigations Cardiac Troponin I and T

Troponin I and T elevation may be seen in viral or acute idiopathic pericarditis and are related to the extent of myocardial inflammatory involvement. Raised cardiac troponins are observed particularly in the young, males, and patients with ST segment elevation and pericardial effusion at presentation (12).

Chest X-ray (CXR) The chest X-ray is rarely informative in acute pericarditis. The presence of cardiac enlargement does not differentiate between pericardial effusion, pericardial wall thickness or cardiac chamber enlargement. Patients with acute pericarditis typically have a normal CXR. Up to 200mls of pericardial fluid would have to accumulate to manifest as an enlarged cardiac shadow (13) (Fig 2).

Echocardiogram The echocardiogram is often normal in individuals with acute pericarditis. The presence of a pericardial effusion (Fig 3) provides support for the diagnosis of


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acute pericarditis and identifies individuals who may be at risk of developing tamponade or constrictive pericarditis. However, the absence of an effusion does not exclude the diagnosis of acute pericarditis.

Pericardiocentesis Diagnostic pericardiocentesis is not routinely warranted (2). Pericardiocentesis can be considered for therapeutic purpose if malignancy, tuberculosis or purulent pericarditis are suspected. Pericardiocentesis is performed on therapeutic grounds in patients with moderate to severe tamponade, and recurrent symptomatic effusions.

Specific aetiological tests In patients without a classical history for viral pericarditis, further investigations are recommended to elucidate the aetiology of acute pericarditis which include blood cultures, antistreptolysin-O (ASO) titre, rheumatoid factor and screens for various autoimmune conditions and tuberculin skin testing.

Management The majority of cases of acute pericarditis are either viral or idiopathic, and usually self-limiting. Therefore, non-steroidal anti-inflammatory drugs (NSAIDs) remain the first line treatment for uncomplicated cases. Pain relief with simple analgesics and bed rest if myocarditis is a possibility should also be considered. The use of NSAIDs for example aspirin and ibuprofen aims to dampen the inflammatory process and expedite recovery. Empirical treatment with steroids may be considered if patients are not responding to NSAIDs, if symptoms are prolonged, severe, or recurrent and if a specific cause cannot be identified (13). Specific therapy should be initiated for patients with an underlying disorder.

Complications Pericarditis can be complicated by pericardial effusions that may be large enough to compromise cardiac function and lead to collapse. Both acute and chronic pericarditis and small effusion can culminate in constrictive pericarditis. These complications are discussed below.

PERICARDIAL EFFUSION The pericardial space contains approximately 15 – 50mls fluid of electrolyte and protein composition similar to plasma and serves to lubricate the layers of the pericardium. The abnormal accumulation of fluid in the potential pericardial space defines pericardial effusion (Fig 3). Pericardial effusion may complicate pericarditis from any cause but is more common in inflammatory, infectious, malignant, and autoimmune processes. Effusion may be acute or chronic, and patient’s symptoms depend upon the time course of effusion development. Rapid accumulation of pericardial fluid as little as 80mls can raise the intrapericardial pressures extensively with significant adverse hemodynamic effects, whilst slowly accumulating fluid can measure more than 2 litres and the patient may not have any symptoms. Treatment is directed at removal of the underlying cause and aspiration of pericardial fluid if the patient is symptomatic. Pericardial effusion can lead to tamponade if not treated and in the long term can result in pericardial constriction.

CARDIAC TAMPONADE Cardiac tamponade is an acute medical emergency, that results from increased pressure within the pericardial cavity from fluid accumulation in the space surrounding the heart. Consequently, there is interference with ventricular filling, reduced cardiac output and fall in blood pressure.

Clinical Manifestations Symptoms include dyspnoea, fatigue, dull central chest pain, facial engorgement, abdominal and ankle swelling, or altered mental state may present. Physical signs include raised jugular venous pressure with prominent ‘x’ descent (Fig 4), hypotension, a rapid low volume pulse, muffled heart sounds (Becks triad), and pulsus paradoxus (fall in the arterial pressure by more than 10mmHg on inspiration). Paradoxical filling of the neck veins on inspiration (Kussumaul’s sign) is less specific. Oliguria or anuria due to a profound drop in cardiac output are well recognised but resolve rapidly after percardiocentesis.

Investigations ECG may show low voltage QRS complex tachycardia and depending on the quantity of the effusion, electrical alternans (alternating QRS complex morphology). CXR may demonstrate a large globular heart depending on the quantity and time duration of fluid accumulation. Emergency echocardiography will confirm the presence and quantity of fluid, and identify diastolic right ventricular collapse (an echocardiographic hallmark of tamponade) from the rising pericardial pressure.

Management Short-term benefit is provided from pericardial needle aspiration with a maximum of 500mls of fluid removal. Larger volumes should be drained more slowly by insertion of a pericardial drain to prevent cardiovascular collapse. Recurrent effusions (usually due to malignancy) are treated by fashioning a pericardial window into the pleural cavity.

CONSTRICTIVE PERICARDITIS Constrictive pericarditis is caused by pathological activation of fibrocystic deposition of collagen building a thickened fibrotic pericardium that eventually restricts cardiac filling. Clinical features of constrictive pericarditis occur as a result of restricted ventricular filling. There is elevation and equalisation of right and left atrial pressure with subsequent venous engorgement and reduced cardiac output. Constrictive pericarditis may be difficult to distinguish from restrictive cardiomyopathy since the physiological, clinical and radiographic imaging studies may be similar. The differentiation between the two entities is set out below (Table 3). In the vast majority of cases of constrictive pericarditis, the aetiology is not ascertained. In the developed world idiopathic or viral aetiology is commonly implicated. Infectious diseases such as tuberculosis are profoundly a dominant cause in the developing world.


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Table 3 Difference between constrictive pericarditis and restrictive cardiomyopathy Constrictive pericarditis Restrictive

cardiomyopathy Right heart failure Right heart failure Pericardial calcification on CXR

Normal size atria Enlarged atria Myocardial texture normal Myocardial texture

infiltrated e.g. amyloid No mitral or tricuspid regurgitation

Mitral and tricuspid regurgitation common

Systolic function normal Systolic function impaired LVEDP =RVEDP LVEDP >RVEDP

Clinical manifestations Patients with constrictive pericarditis may present with gradual onset of symptoms and signs of predominantly right-sided heart failure. The most common presenting complaints include dyspnoea, abdominal swelling and ankle oedema although patients may also present with fatigue and orthopnoea. Characteristically, the jugular vein pressure (JVP) exhibits prominent X and Y decents (Fig 4) (that are persistent even in patients with atrial fibrillation) and may rise during inspiration (Kussumaul’s sign). Other clinical features including pleural effusion, hepatosplenomegaly, ascites and ankle oedema. The apex beat is often impalpable and the heart sounds are soft. The sudden cessation of ventricular filling in early diastole corresponds with a pericardial knock which is often mistaken for the third heart sound, however, it is of a higher frequency and occurs slightly earlier than third heart sound. In advanced case, cardiac cachexia with marked muscle wasting is noted.

Investigations Chest radiograph is rarely helpful in the diagnosis of constrictive pericarditis, however the presence of pericardial calcification in a patient with right heart failure may be indicative of previous tuberculous pericarditis (Fig 5). Generally, the cardiac silhouette appears normal. Two-dimensional echocardiography is not regarded to be a very sensitive tool in the diagnosis of constrictive pericarditis since the pericardium is not echodense in most cases. However, doppler echocardiography across mitral and tricuspid valves show filling patterns that vary with respiration (Fig 6). The early (E) and late (A) filling ratio (E/A) across the mitral valve is reduced during inspiration and increased with expiration. The pattern is reversed at the tricuspid valve with increased velocity with inspiration and reduction during expiration. These doppler findings are consistent with the diagnosis of constrictive pericarditis but their absence does not exclude the condition. High resolution CT scan may demonstrate pericardial thickening up to 4 mm. A normal pericardial thickness

does not exclude the diagnosis of constrictive pericarditis. Cardiac catheterisation helps differentiate from restrictive cardiomyopathy. It may demonstrate high atrial pressure with prominent X and Y descents and equalisation of left ventricular end diastolic pressure (LVEDP) and right ventricular end diastolic pressure (RVEDP) in all phases of respiration (Fig 7). Rapid early filling of both ventricles followed by diastasis is also evident.

Management Treatment of constrictive pericarditis is primarily surgical. Pericardiectomy is the definitive treatment with rapid improvement of symptoms and haemodynamic status.

References 1. Shabetai R. Evaluation and management of acute pericarditis. www.uptodate.com 2. Sagrista-Sauleda, J, Merce, J, Permanyer-Miralda, G, Soler-Soler, J. Clinical clues to the causes of large pericardial effusions. Am J Med 2000; 109:95 3. Wilkes, JD, Fidias, P, Vaickus, L, Perez, RP. Malignancy-related pericardial effusion. 127 cases from the Rosewell park cancer institute. Cancer 1995; 76:1377 4. Porte. HL. Janecki-Delebeca. TJ. Finzi. L. et al. Pericardoscopy for primary management of pericardial effusion in cancer patients. Eur J Cardiothracic Surg 1999; 16:287 5. Galve, E, Garcia-del-Castillo, H, Evangelista, A, et al. Pericardial effusion in the course of myocardial infarction: Incidence, natural history, and clinical relevance. Circulation 1986; 73:294 6. Correale, E, Maggioni, AP, Romano, S, et al. Comparison of frequency, diagnostic and prognostic significance of pericardial involvement in acute myocardial infarction treated with and without thrombolytics. Am J Cardiol 1993; 71:1377 7. Belkin, RN, Mark, DB, Aronson, L. Pericardial effusion after intravenous recombinant tissue-type plasminogen activator for acute myocardial infarction. Am J Cardiol 1991; 67:496 8. Shahar, A, Hod, H, Barabash, GM, et al. Disappearance of a syndrome: Dressler’s syndrome in the era of thrombolysis. Cardiology 1994; 85:255 9. Rostand, SG, Rutsky, EA. Pericarditis in end-stage renal disease. Cardiol Clin 1990; 8:701 10. Gunukula SR, Spodick, DH. Pericardial disease in renal patients. Semin Nephrol 2001; 21:52 11. Spodick, DH. Arrhymias during acute pericarditis. A prospective study of 100 consecutive cases. J Am Med Assoc 1976; 235:39 12. Imazio M, Demichelis B, Cecchi E, et al. Cardiac Troponin I in acute pericarditis. J Am Coll Cardiol 2003; 42:2144 13. Spodick, DH. Acute cardiac tamponade. N Engl J Med 2003; 349:684 14. John S. Constrictive pericarditis. www.emedicine.com 2003 .


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Figure 1. ECG in pericarditis showing diffuse symmetrical concave ST segment elevation in most/all leads

Figure 2. Chest radiograph in pericardial effusion showing enlarged cardiac shadow


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Figure 3. Echo showing presence of pericardial effusion

Pericardial effusion

Figure 4. JVP waveform in cardiac tamponade and constrictive pericarditis

:

Cardiac TamponadeDeep x decent

ecentDeep x & y dConstrictive Pericarditis

Normala

x v

y

yv

x

a

y v x

a


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Figure 5. Chest radiograph in constrictive pericarditis showing pericardial calcification of tuberculous aetiology


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Figure 6. Early (E) and late (A) filling across mitral and tricuspid valves during respiration. E/A ratio across mitral valve decreases during inspiration and increases with expiration. E/A ratio across tricuspid valve increases during inspiration and decreases with expiration.

Mitral

Tricuspid

25% increase in E wave

25% decrease of E wave

Inspiration Expiration

Figure 7. Dip and Plateau waveforms differentiating restrictive cardiomyopathy from constrictive pericarditis


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Download - Pericarditis - Orphanet · PDF file · 2005-03-11neoplastic pericardial effusions may also present with supraventricular arrhythmias, or features of constrictive pericarditis. Generally,

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