Chronic constrictive pericarditis
Pericarditis is an inflammation of the pericardium, a mesothelial cell layer overlying the heart, and usually presents with chest pain, a pericardial friction rub, and electrocardiographic changes. Patients with pericarditis may go on to develop pericardial constriction or pericardial effusions.
The aetiology has been classified as follows:
1 Idiopathic: this can account for anything between 26–84% of cases. As there are no specific clinical features to differentiate idiopathic from viral pericarditis, many may have a viral aetiology. There is seasonal variation with most cases occurring in spring and autumn.
2 Viral: proven viral pericarditis accounts for up to 10% of cases. Many viruses have been implicated, but coxsackie B and influenza viruses are probably the most common. The condition may be associated with myocarditis. HIV infection rarely causes pericarditis unless the condition is advanced.
3 Bacterial: purulent pericarditis is fortunately rare, and can be caused by many bacteria including anaerobic bacteria, particularly in children. Spread may occur from infective endocarditic lesion. Patients are often very unwell at presentation, and the mortality can be high.
4 Mycobacterium: the incidence depends upon the current prevalence of mycobacterium agents in the population. In the developed world mycobacterium pericarditis is commoner in the elderly population. It often progresses to pericardial constriction.
5 Inflammatory conditions: this includes a wide range of autoimmune disorders including rheumatoid arthritis, systemic lupus erythematosus (SLE), and scleroderma. Rheumatic fever, which is now very rare in the developed world, used to be a common cause.
6 Metabolic disorders: particularly renal failure and hypothyroidism.
7 Myocardial ‘trauma’: underlying damage to the heart can produce pericarditis and cause include acute myocardial infarction, cardiac contusion, Dressler’s syndrome (post-pericardiotomy syndrome), and aortic dissection.
8 Neoplasia: this is commonly lung tumours including mesothelioma, breast cancer, and haematological malignancies.
9 Iatrogenic: includes, percutaneous cardiac interventions, adverse drug reactions, and complications of radiation therapy.
• Chest pain is almost universal and can be described as sharp, but it is not uncommon for the pain to be undistinguishable from ischaemic type cardiac pain. The pain may be exacerbated by movement and respiration, and can be relieved by sitting forward.
• Shortness of breath is common and would require exclusion of pericardial effusion and tamponade.
• Fever is less usual unless there is purulent pericarditis.
• A pericardial friction rub, if heard, is pathogonomic of pericarditis.
• The patient may have to be moved into various positions to reveal the rub, which is best heard using the diaphragm of the stethoscope, and has been likened to the sound made by rubbing hair between fingers.
• Fever, tachycardia, and tachypnoea may be present.
• Kussmaul’s sign, a rise is the JVP on inspiration, and pulsus paradoxus, an exaggeration of the normal fall in blood pressure on inspirations, are not features of uncomplicated pericarditis and suggest pericardial constriction or cardiac tamponade due to pericardial effusion respectively.
Is the most useful diagnostic test is acute pericarditis. There are J-point ST segment elevations, which are usually described as concave or ‘saddle-shaped’ and T-wave changes. In contrast to the regional changes suggestive of myocardial ischaemia, these changes are usually widespread affecting the majority of limb and chest leads. The changes will evolve over time with the ST segments becoming isoelectric, and the T waves inverting, before returning to normal usually over a few days.
• Markers of myocardial damage, such as troponin may be elevated if there is concomitant myocarditis.
• Virological, microbiological, biochemical, and autoimmune studies should be performed as indicated by the clinical history or examination.
Is usually performed to exclude concomitant pericardial effusion, but is rarely abnormal unless there is some myocardial or endocardial cause for the pericarditis.
With agents that identify inflammation such as gallium-67 or MRI scans with gadolinium are rarely used in the diagnosis.
Is usually normal unless there is a pericardial effusion or a pulmonary cause for the pericarditis.
• Oxygen and cardiac monitoring should be provided.
• It is important to rule out other life-threatening causes of chest pain, such as myocardial infarction or aortic dissection.
• Monitoring should continue if there is any evidence of haemodynamic instability.
• Treatment for specific causes of pericarditis is directed according to the underlying cause. For patients with idiopathic or viral pericarditis, therapy is directed at symptom relief. Non-steroidal anti-inflammatory drugs (NSAIDs) are the mainstay of therapy, with relief of chest pain in about 85–90% of patients within a few days of treatment.
• Ibuprofen has the advantage of few adverse effects and increased coronary flow.
• Indomethacin has a poor adverse effect profile and reduces coronary flow, and should be avoided in patients with post-myocardial infarction pericarditis.
• Aspirin is recommended for treatment of pericarditis after STEMI.
• Colchicine, in combination with NSAIDs can be considered in the initial treatment to prevent recurrent pericarditis. Colchicine, alone or in combination with NSAIDs, can be considered for patients with recurrent or continued symptoms beyond 14 days.
• Corticosteroids should not be used for initial treatment of pericarditis unless it is indicated for the underlying disease, the patient has no response to NSAIDs or colchicine, or both drugs are contraindicated.
• Once the diagnosis is confirmed and the patient is haemodynamically stable, it is safe to discharge the patient and review on an outpatient basis.
• The absence of continued fever, immunosuppression, trauma, oral anticoagulation therapy, myopericarditis, severe pericardial effusion, and cardiac tamponade are good prognostic signs.
Outpatient follow-up should ideally be within one month, and should assess the patient for complications which include:
• A significant risk of recurrent pericarditis, which can cause significant morbidity secondary to pain. The patient should be warned that this may occur.
• Pericardial effusions leading to a risk of cardiac tamponade.
• Pericarditis, especially if recurrent, leads to a significant risk of constrictive pericarditis, or a combination of effusive and constrictive pericarditis.
• The prognosis depends on the aetiology.
• Idiopathic and viral aetiologies usually have a self-limited course.
• Purulent, tuberculous, and neoplastic pericardial involvement have a more complicated course with worse outcomes.
• NSAIDs and corticosteroids should be used with caution in patients with pericarditis secondary to acute myocardial infarction, as they may interfere with ventricular healing and remodelling.
• Pericarditis can be difficult to distinguish from acute myocardial infarction and repolarization in patients who present with chest pain and ST-segment elevation on an ECG. A careful and thorough history and physical examination, together with the fact that the repolarization does not progress through stages, and is uncommonly associated with PR depression, will help to differentiate the conditions (Figure 9.1.1).
• Serial monitoring of ECGs in young patients with chest pain helps differentiate early repolarization from acute pericarditis.
• The ST segment in acute myocardial infarction is usually convex, bowing upward with reciprocal changes, as opposed to concave ST segments without reciprocal changes observed in acute pericarditis and repolarization.
• Children may present with abdominal pain.
• The classic feature of chest pain and dyspnoea with pericarditis may be subtle and can be confused with other diagnoses, particularly in elderly people.
Fig. 9.1.1 Typical electrocardiographic changes in acute pericarditis including diffuse concave upward ST segment elevation, except in aVR where there is ST segment depression. The T waves are upright in the leads with ST elevation, and the PR segment deviates opposite to P-wave polarity.
Chronic constrictive pericarditis (CCP) is a clinical constellation that includes a thickened and rigid or semi-rigid pericardium with fusion of the visceral and parietal layers associated with impaired cardiac filling and elevated ventricular filling pressures and which presents with signs and symptoms of heart failure, most notably an elevated jugular venous pressure, dyspnoea, peripheral oedema, hepatomegaly, and ascites.
Worldwide the leading causes are as sequelae to acute pericarditis especially tuberculous. In the developed world constriction as a complication of cardiac surgery or mediastinal radiation is more common. Pericardial fibrosis and calcification may be idiopathic. A major clinical challenge is to determine the relative contribution of pericardial restraint and underlying myocardial dysfunction in determining the signs and symptoms.
Characteristic pathophysiological features of chronic constrictive pericarditis include:
• Fibrosis, fusion and usually thickening of visceral parietal pericardium
• Pericardial calcification can result in the formation of calcified plaques (eggshell appearance)
• Myocardium classically not involved but can be in radiation induced CCP, or if an underlying pericarditis involves the myocardium.
Causes of chronic constrictive pericarditis include:
• Tuberculosis
• Post-viral pericarditis
• Post-mediastinal radiotherapy
• Neoplastic pericardial infiltration
• Other infective pericarditities
• Post-Dressler’s syndrome
• Connective tissue diseases with chronic pericarditis
• Chronic renal failure.
• Depends upon the underlying aetiology
• Pericardial resection can be curative
• Clinical approach.
Typical presentation is with features of right heart failure:
• Dyspnoea
• Dizziness or syncope
• Oedema
• Ascites.
• Raised jugular venous pressure, especially with Kussmaul sign (ordinarily the JVP falls with inspiration. Kussmaul’s sign suggests impaired filling of the right ventricle due to either fluid in the pericardial space or a poorly compliant myocardium or pericardium) but this does not differentiate from tamponade.
• Signs of right heart failure such as peripheral oedema, ascites, hepatomegaly.
• Increased cardiothoracic ratio is common, but not universal.
• Pericardial calcification can occur but is neither diagnostic nor essential.
• BNP may be low despite signs and symptoms of heart failure.
• Thickened pericardium (may be regionalized). Transoesophageal echo is more reliable in estimating pericardial thickness than transthoracic two-dimensional echo. M-mode is intermediate. Note that the presence of an echo-free space between the visceral and parietal pericardium suggests pericardial effusion.
• Mitral inflow Doppler shows increased early diastolic filling velocity followed by rapid deceleration, with a shortened overall filling period.
• Early mitral inflow deceleration time usually <160 ms.
• Can be differentiated from restrictive cardiomyopathy by detection of dynamic changes with respiration: reduced early diastolic mitral inflow and prolonged isovolumic relaxation time with inspiration, shortened isovolumic relaxation time and increased early diastolic mitral inflow (typically >25%) velocity on expiration.
• Sensitivity and specificity of Doppler assessment of respiratory changes in confirming CCP diagnosis reported to be as high as 85% to 90% (Hancock 2001).
• Inferior vena cava (IVC) dilatation.
• Increased tricuspid regurgitant jet velocity from onset to peak inspiration can.
• Displacement of the interatrial septum toward the left atrium during inspiration.
• Computed tomography and magnetic resonance imaging can help in detecting an abnormal pericardium.
• The classical haemodynamic changes of CCP are attributed to a relatively fixed end-diastolic ventricular volume and isolation of the cardiac chambers from intrathoracic respiratory pressure changes.
• Normally respiratory changes in intrathoracic pressure affect pulmonary and systemic veins entering the heart and the intracardiac chambers relatively equally. In CCP the intracardiac chambers are protected from these respiratory pressure swings leading to characteristic changes.
• The pressure gradient between the pulmonary veins and the left atrium decreases with inspiration in CCP, producing a reduction in diastolic pulmonary vein flow and left atrial and filling.
• The rigid, noncompliant constricted pericardial sac couples both ventricles and hence an increase in filling on one side impedes filling on the other (ventricular interdependence). The inspiratory reduction in left atrial and left ventricular filling is therefore associated with increased diastolic right ventricular filling and a shift in the septum towards the left ventricle, with the opposite pattern being seen in the expiratory phase.
• This haemodynamic pattern causes characteristic changes in the jugular venous and right atrial pressure waveforms. The elevated mean filling pressures produce a prominent ‘a’ wave, the sharp ‘y’ descent reflects rapid early diastolic RV filling due to the shift in the septum reducing RV pressures, and a preserved ‘x’ descent due to accelerated atrial relaxation. The RV haemodynamic waveform with a dip-and-plateau (commonly called the square root pattern) reflects rapid ventricular relaxation, with a subsequent sharp increase in filling pressure as the expanding ventricle is impeded by the rigid pericardium.
• At end-diastole right atrial, right ventricular, pulmonary wedge and LV pressures are equal and elevated.
• These haemodynamic consequences of the pathophysiological effects of pericardial constriction account for the clinical, and echocardiographic findings in CCP.
• The management of CCP depends upon the aetiology and clinical state of the patient.
• Treat tuberculous cases with standard antituberculosis drugs for 6 months: neoplastic or connective tissue disease requires specific therapy for these conditions.
• Surgical pericardiectomy is the definitive treatment, depending on the underlying aetiology.
Dal-Bianco JP, Sengupta PP, Mookadam F, et al. Role of echocardiography in the diagnosis of constrictive pericarditis. J Am Soc Echo 2009;22:24–33.
Hancock EW. Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis. Heart 2001;86:343–9.
Hurrell DG, Nishimura RA, Higano ST, et al. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation 1996;93;2007–13.
Pericardial effusion is defined as the presence of an abnormal amount of pericardial fluid. There is normally between 10 and 50 mL of an ultra-filtrate of plasma (‘pericardial lubrication’) within the pericardial space. There is significant overlap of the causes of pericardial effusion with those of pericarditis (see Chapter 9.1). Pericardial effusions, however, are more likely to be caused by malignant disease, myocardial trauma (including iatrogenic causes and acute myocardial infarction), bacterial or mycobacterium infections, or renal failure. Pericardial effusions can be acute or chronic, and the rate of fluid accumulation has a major impact on development of the patient’s symptoms. The treatment of pericardial effusion varies, from watchful waiting to emergency pericardiocentesis, and is directed at both removal of the pericardial fluid and alleviation of the underlying cause, which usually is determined by a combination of fluid analysis and correlation with co-morbid illnesses.
In health the pericardium:
• Acts as a restraint to right heart dilatation maintaining diastolic pressure, and is responsible for most of the pressure in the right atrium and ventricle.
• Evenly distributes forces across the heart to ensure uniform contraction of the myocardium.
• Stretches to accommodate small amounts of fluid without significant change in intrapericardial pressure (pericardial compliance).
When excessive pericardial fluid is produced:
• The pressure–volume curve becomes steep.
• Slow increases in volume cause increased pericardial compliance and lessen the increase in intrapericardial pressure.
• Clinical manifestations are highly dependent upon the rate of accumulation of fluid in the pericardial sac.
• During inspiration increased intrapericardial pressure is transmitted to the right heart. This leads to the shifting of the atrial and ventricular septa to the left, reducing left heart diastolic volumes and causes a fall in cardiac output.
• As intrapericardial pressures rise, this effect becomes pronounced, leading to the development of pulsus paradoxus and the signs of cardiac tamponade (Figure 9.3.1).
• Chest pain is common and similar to pericarditis pain
• Palpitation
• Cough
• Hiccoughs.
As the patient develops tamponade the symptoms are those of reduced cardiac output:
• Dyspnoea
• Presyncope or syncope
• Confusion.
• The classical Beck’s triad of hypotension, muffled heart sound and a raised jugular venous pressure is specific, but not sensitive for a diagnosis of pericardial effusion, as they are signs of incipient tamponade.
• Pulsus paradoxus is an exaggeration of the normal fall in blood pressure (normally less than 10 mmHg) on inspiration. The reason that it is termed paradoxical is that in extreme cases the peripheral pulses will disappear but on auscultation the heart continues to beat.
• A pericardial friction rub that is pathogonomic of pericarditis may disappear with development of an effusion.
• Tachyopnoea and cyanosis usually indicates the development of tamponade.
• Ewart’s sign: dullness to percussion in the left subscapular region due to compression of the right lung.
• Peripheral oedema and hepatosplenomegaly are unusual with acute pericardial effusion and usually occur with constrictive pericarditis or rarely with chronic effusions.
This is the mainstay of the diagnosis of pericardial effusions.
• The presence of an echo-free space between the visceral and parietal pericardium is pathogonomic for pericardial effusion.
• Pericardial effusions are usually best imaged via the subxiphisternal approach with the patient supine or with the back slightly elevated.
• The effusion may be localized and may require other views to exclude loculated fluid.
Signs suggestive of cardiac tamponade (which is a predominantly clinic diagnosis) include:
• Early diastolic collapse of the right ventricular free wall.
• Late diastolic collapse/compression of the right atrium.
• Swinging of the heart in the pericardial sac.
• A greater than 40% relative inspiratory augmentation of right side (tricuspid) flow.
• A greater than 25% relative inspiratory decrease of left sided (mitral) flow.
It is important to exclude pleural effusions, pericardial thickening and mediastinal lesions which may be confused with pericardial fluid.
• Often non-specific in the absence of acute pericarditis. Sinus tachycardia is common particularly with incipient tamponade. There may be low voltage QRS complexes and electrical alternans, which are due to swinging of the heart within the pericardial sac.
• May show an enlarged cardiac silhouette, but may be normal when the effusion is small or of acute onset.
• The type of pericardial fluid produced depends upon the underlying aetiology.
• Transudative fluids result from obstruction of fluid drainage, which occurs through lymphatic channels.
• Exudative fluids occurs secondary to inflammatory, infectious, malignant, or autoimmune processes within the pericardium.
Fig. 9.3.1 A subxiphisternal view of a large pericardial effusion. A large echo-free space is seen (asterisk). The right ventricle (RV) and left ventricle (LV) are compressed.
Pericardial fluid can be analysed:
• To assess if it a transudate or exudates using protein and lactate dehydrogenase (LDH) levels.
• Haemoglobin and white cell count to assess haemorrhagic or purulent effusions.
• Cytological examination for malignancy.
• Viral, bacterial and mycobacterium cultures.
• To look for myocardial damage, metabolic, endocrine, and autoimmune disorders.
• The management of pericardial effusion depends upon the aetiology and clinical state of the patient.
• If the effusion is related to a major cardiovascular catastrophe such as aortic dissection or myocardial rupture then urgent referral for a cardiac surgical opinion is required.
• If the effusion is related to an interventional cardiac procedure and the patient is in the cardiac catheterization laboratory, urgent pericardiocentesis can be performed.
• If the patient is stable with no clinical symptoms or signs of tamponade, and there is no evidence of an infective cause which requires pericardial fluid for culture, then pericardiocentesis can be deferred.
• If the patient presents with or develops signs of cardiac tamponade then a therapeutic pericardiocentesis should be performed.
• Chronic pericardial effusion (defined as being present for greater than 6 months) has a good prognosis, is usually well tolerated, and has a higher risk to benefit ratio for pericardiocentesis.
• Preprocedure transthoracic echocardiography is strongly recommended unless the procedure is a life-saving emergency. This will allow appropriate patient positioning and needle length selection.
• The patient should have continuous ECG monitoring.
• Ideally the procedure should be performed in a cardiac catheterization laboratory or in a pacing room where there is access to X-ray screening if required.
• The usual approach is subxiphisternal with the patient supine, but with the back raised at about 30°.
• A sterile field is prepared and local anaesthetic is infiltrated pointing toward the left shoulder. The needle is advanced on a syringe with mild traction on the plunger and once fluid is encountered, a guidewire is passed into the pericardial space. A pigtail catheter or sheath is inserted and fluid is removed for analysis and then therapeutically is left to drain.
• Some operators use an echocardiographic guided technique from the left intercostal space, using echocardiography to identify the location and depth of the maximal fluid accumulation.
• Once the fluid is no longer draining, and the effusion is absent on echocardiography, the catheter can be removed.
• Complications of pericardiocentesis include arrhythmias, cardiac or coronary artery perforation and pneumothorax.
• If pericardial effusion recur or are of a known malignant aetiology, then percutaneous balloon pericardiotomy or formal surgical pericardial window either using a subxiphisternal incision, or video-assisted thoracoscopic (VAT) approach should be considered.
• All these techniques make a connection between the pericardial space and the abdomen where the effusion is absorbed via the lymphatic drainage. Percutaneous balloon pericardiotomy, which is performed under local anaesthesia with sedation, is associated with the best success rates and shortest hospital stays, but if pericardial tissue is require for histology a surgical approach is necessary.
• Depends upon the underlying aetiology.
• Pericardial effusion associated with significant cardiovascular disease, such as aortic dissection or myocardial rupture following myocardial infarction, have very poor prognoses and require emergency referral for a cardiac surgical opinion.
• Patients with malignant pericardial effusion have a poor prognosis consistent with a diagnosis of disseminated malignancy. Since these patients are more likely to have recurrent effusions, treatment with percutaneous balloon pericardiotomy may be considered as an initial procedure, to reduce the time spent in hospital.
• Failure to consider pericardial effusion either as a primary or secondary diagnosis may lead to rapid deterioration and death secondary to cardiac tamponade.
• Patients with dilated or congestive cardiomyopathy may present acutely with symptoms and signs similar to cardiac tamponade. Echocardiography is mandatory in these patients to distinguish the two conditions.
