Pericardial Disease: Difference between revisions

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=Acute Pericardial Disease=
The pericardium comprises two layers; the visceral layer that adheres to epicardial surface of the heart, and the parietal layer that surrounds most of the heart. Pericardial disease is common, and diagnosis is usually straightforward as the pericardium reacts to disruption by a wide variety of agents and processes in a relatively uniform manner. Typical presentation is with chest pain and fever, production of pericardial fluid with possible cardiac tamponade, or a constrictive pattern by thickening, retraction and calcification.  
The pericardium comprises two layers; the visceral layer that adheres to epicardial surface of the heart, and the parietal layer that surrounds most of the heart. Pericardial disease is common, and diagnosis is usually straightforward as the pericardium reacts to disruption by a wide variety of agents and processes in a relatively uniform manner. Typical presentation is with chest pain and fever, production of pericardial fluid with possible cardiac tamponade, or a constrictive pattern by thickening, retraction and calcification.  
==Cardiac Tamponade==
 
==Pericardial Disease - Acute==
===Cardiac Tamponade===
An increase in intrapericardial pressure, resulting in compression of the heart, and thereby a resitriction of cardiac inflow, is termed cardiac tamponade. Tamponade may result from pericardial effusion of any cause. The intrapericardial pressure importantly determines to what extent cardiac inflow is decreased, but two factors need to be taken into consideration. First, intrapericardial pressure is determined not only on the amount of fluid that accumulates, but also on the rate with which this accumulation proceeds, and the available distensibility of the pericardium. Chronic effusions may therefore lead to small increases in intrapericardial pressures in the presence of large fluid accumulations, and small accumulations may directly lead to severe cardiac tamponade for example after free wall rupture. Second, the intravascular volume and intra-atrial, and -ventricular pressures determine at what pressure inflow becomes impaired. When intrapericardial pressure exceeds right atrial pressure (approximately 8 mmHg), tamponade typically follows. However, in patients in whom intra-atrial pressure is decreased, for example due to volume depletion, tamponade may already occur at lower intrapericardial pressures; low-pressure cardiac tamponade.
An increase in intrapericardial pressure, resulting in compression of the heart, and thereby a resitriction of cardiac inflow, is termed cardiac tamponade. Tamponade may result from pericardial effusion of any cause. The intrapericardial pressure importantly determines to what extent cardiac inflow is decreased, but two factors need to be taken into consideration. First, intrapericardial pressure is determined not only on the amount of fluid that accumulates, but also on the rate with which this accumulation proceeds, and the available distensibility of the pericardium. Chronic effusions may therefore lead to small increases in intrapericardial pressures in the presence of large fluid accumulations, and small accumulations may directly lead to severe cardiac tamponade for example after free wall rupture. Second, the intravascular volume and intra-atrial, and -ventricular pressures determine at what pressure inflow becomes impaired. When intrapericardial pressure exceeds right atrial pressure (approximately 8 mmHg), tamponade typically follows. However, in patients in whom intra-atrial pressure is decreased, for example due to volume depletion, tamponade may already occur at lower intrapericardial pressures; low-pressure cardiac tamponade.


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When secondary to inflammatory pericarditis, tamponade up to moderate severity may be treated by anti-inflammatory drugs. In severe tamponade, pericardiocentesis should be performed to immediately alleviate intrapericardial pressure. Surgical drainage should be considered when pericardiocentesis is unsuccessful, or when tamponade recurs.
When secondary to inflammatory pericarditis, tamponade up to moderate severity may be treated by anti-inflammatory drugs. In severe tamponade, pericardiocentesis should be performed to immediately alleviate intrapericardial pressure. Surgical drainage should be considered when pericardiocentesis is unsuccessful, or when tamponade recurs.


==Acute pericarditis==
===Acute pericarditis===
Acute inflammation of the pericardium may result from a wide variety of etiologies (Table 1), and typically presents with chest pain, a pericardial friction rub on auscultation, and repolarization changes on the electrocardiogram.   
Acute inflammation of the pericardium may result from a wide variety of etiologies (Table 1), and typically presents with chest pain, a pericardial friction rub on auscultation, and repolarization changes on the electrocardiogram.   


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!Table 1. Causes of acute pericarditis
!Table 1. Causes of acute pericarditis
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Treatment consists of aspirin while pain and fever are present, which usually adequately alleviates symptoms. Another option is NSAIDs, which are recommended when aspirin in insufficient or contraindicated. Corticosteroids should, however, be avoided as they are associated with relapsing pericarditis. Hospital admission may be necessary in patients with high fever, large effusions or cardiac tamponade.
Treatment consists of aspirin while pain and fever are present, which usually adequately alleviates symptoms. Another option is NSAIDs, which are recommended when aspirin in insufficient or contraindicated. Corticosteroids should, however, be avoided as they are associated with relapsing pericarditis. Hospital admission may be necessary in patients with high fever, large effusions or cardiac tamponade.


==Recurrent pericarditis==
===Recurrent pericarditis===
In 8 to 80% of patients, pericarditis recurs after a first episode of acute pericarditis. A continuous type, in which symptoms recur shortly after cessation of anti-inflammatory therapy, and an intermittent type, in which symptom-free periods of more than 6 weeks separate recurrences, can be distinguished. Frequently resulting from inadequate therapy or corticosteroid-use during the initial episode, subsequent recurrences are usually less severe. A recurrence should be treated according to the same procedures as for the first event. Pericardiectomy may be considered the last resort in severely refractory recurrent pericarditis, but its results are unpredictable. Prognosis of the disease is excellent, as severe complications are rare.  
In 8 to 80% of patients, pericarditis recurs after a first episode of acute pericarditis. A continuous type, in which symptoms recur shortly after cessation of anti-inflammatory therapy, and an intermittent type, in which symptom-free periods of more than 6 weeks separate recurrences, can be distinguished. Frequently resulting from inadequate therapy or corticosteroid-use during the initial episode, subsequent recurrences are usually less severe. A recurrence should be treated according to the same procedures as for the first event. Pericardiectomy may be considered the last resort in severely refractory recurrent pericarditis, but its results are unpredictable. Prognosis of the disease is excellent, as severe complications are rare.  


==Pericardial effusion==
===Pericardial effusion===
Fluid accumulation in the pericardium, pericardial effusion, is a common finding on routine echocardiography, and is asymptomatic in the absence of inflammation or cardiac tamponade. It may result from any disease of the pericardium, or be iatrogenic. Most frequently it results from idiopathic pericarditis, malignancy, or iatrogenic defects (Table 2).  
Fluid accumulation in the pericardium, pericardial effusion, is a common finding on routine echocardiography, and is asymptomatic in the absence of inflammation or cardiac tamponade. It may result from any disease of the pericardium, or be iatrogenic. Most frequently it results from idiopathic pericarditis, malignancy, or iatrogenic defects (Table 2).  


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!Table 2. Causes of pericardial effusion
!Table 2. Causes of pericardial effusion
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Any form of pericarditis may end in constrictive pericarditis, presenting with chronic fatigue, dyspnoea, jugular distension, proto-diastolic pericardial knock, hepatomegaly, ascites, peripheral oedema, and pleural effusion. Atrial fibrillation is a common finding, and diffuse flattened or negative T-waves are usually present. These suggestive clinical findings, in addition to a physiology of restriction or constriction on echocardiography, and the presence of a thickened pericardium provide the diagnosis. However, a thickened pericardium may be absent, which does not rule out constrictive pericarditis. Pericardiectomy is the only effective treatment, which should be instituted shortly after diagnosis, as surgical mortality increases with increasing age and functional impairment.
Any form of pericarditis may end in constrictive pericarditis, presenting with chronic fatigue, dyspnoea, jugular distension, proto-diastolic pericardial knock, hepatomegaly, ascites, peripheral oedema, and pleural effusion. Atrial fibrillation is a common finding, and diffuse flattened or negative T-waves are usually present. These suggestive clinical findings, in addition to a physiology of restriction or constriction on echocardiography, and the presence of a thickened pericardium provide the diagnosis. However, a thickened pericardium may be absent, which does not rule out constrictive pericarditis. Pericardiectomy is the only effective treatment, which should be instituted shortly after diagnosis, as surgical mortality increases with increasing age and functional impairment.


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!colspan="2"|Table 3.  Differential diagnosis between chronic constrictive pericarditis and restrictive cardiomyopathy
!colspan="2"|Table 3.  Differential diagnosis between chronic constrictive pericarditis and restrictive cardiomyopathy
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!width="50%"|Constrictive pericarditis
!Constrictive pericarditis  
!Restrictive cardiomyopathy
!Restrictive cardiomyopathy
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====Subacute elastic constriction====
===Subacute elastic constriction===
Elastic cardiac constriction, in contrast to the rigid chronic constriction, results from an elastic thickened pericardium, which still allows distension during the respiratory cycle. It may be seen in the first period after acute inflammatory or infectious pericarditis, and may progress to chronic pericardial constriction, or prove to be a transient process.
Elastic cardiac constriction, in contrast to the rigid chronic constriction, results from an elastic thickened pericardium, which still allows distension during the respiratory cycle. It may be seen in the first period after acute inflammatory or infectious pericarditis, and may progress to chronic pericardial constriction, or prove to be a transient process.


====Effusive-constrictive pericarditis====
===Effusive-constrictive pericarditis===
Presenting with cardiac tamponade on admission, effusive-constrictive pericarditis is characterized by a persistent increase in right atrial and end-diastolic ventricular pressures after intrapericardial pressure has been alleviated by pericardiocentesis. Apart from idiopathic cases, it may accompany chest radiation, cardiac surgery, neoplasia, and tuberculosis. Most frequently, the disease will progress to persistent constriction for which epicardiectomy is indicated, but it may rarely be a transient phenomenon.
Presenting with cardiac tamponade on admission, effusive-constrictive pericarditis is characterized by a persistent increase in right atrial and end-diastolic ventricular pressures after intrapericardial pressure has been alleviated by pericardiocentesis. Apart from idiopathic cases, it may accompany chest radiation, cardiac surgery, neoplasia, and tuberculosis. Most frequently, the disease will progress to persistent constriction for which epicardiectomy is indicated, but it may rarely be a transient phenomenon.


====Transient cardiac constriction====
===Transient cardiac constriction===
Clinical and hemodynamic features of constrictive pericarditis may dissipate spontaneously, which is seen commonly (20%) in idiopathic acute pericarditis with effusion, but may also be seen in tuberculous and purulent pericarditis. Hence, a primarily conservative approach may alleviate the need for epicardiectomy.
Clinical and hemodynamic features of constrictive pericarditis may dissipate spontaneously, which is seen commonly (20%) in idiopathic acute pericarditis with effusion, but may also be seen in tuberculous and purulent pericarditis. Hence, a primarily conservative approach may alleviate the need for epicardiectomy.


===Specific types of pericardial disease===
==Pericardial Disease - Acute==
====Infectious pericarditis====
===Infectious pericarditis===
=====Idiopathic/Viral pericarditis=====
 
===Idiopathic/Viral pericarditis===
This is the most frequent form of pericarditis, accounting for more than 80% of cases, of which most probably are of viral etiology, but virus identification is cumbersome and no treatment consequences exist. The disease is frequently accompanied by pericardial effusion, cardiac tamponade, and left pleural effusion, but prognosis is notably good.
This is the most frequent form of pericarditis, accounting for more than 80% of cases, of which most probably are of viral etiology, but virus identification is cumbersome and no treatment consequences exist. The disease is frequently accompanied by pericardial effusion, cardiac tamponade, and left pleural effusion, but prognosis is notably good.


=====Tuberculous pericarditis=====
===Tuberculous pericarditis===
Predominantly found in developing countries, and in patients with human immunodeficiency virus  (HIV) infection, tuberculous pericarditis is rare in the Western world and presents typically with symptoms of acute pericarditis. Identification of Mycobacterium tuberculosis yields the diagnosis, which may be found in pericardial or other bodily fluids, or may be assumed when caseating granulomas are found. Routine tuberculosis treatment, comprising three antituberculous agents, yields a good prognosis although a subacute constrictive pericarditis is common, requiring pericardiectomy but with excellent outcome.
Predominantly found in developing countries, and in patients with human immunodeficiency virus  (HIV) infection, tuberculous pericarditis is rare in the Western world and presents typically with symptoms of acute pericarditis. Identification of Mycobacterium tuberculosis yields the diagnosis, which may be found in pericardial or other bodily fluids, or may be assumed when caseating granulomas are found. Routine tuberculosis treatment, comprising three antituberculous agents, yields a good prognosis although a subacute constrictive pericarditis is common, requiring pericardiectomy but with excellent outcome.


=====Purulent pericarditis=====
===Purulent pericarditis===
Purulent pericarditis has a high mortality, owing to the intangible diagnosis and the related severity of the underlying disease. Cardiac tamponade is frequent, and acute constrictive pericarditis may occur. The disease should be considered in all patients presenting with high fever, dyspnoea, and tachycardia with intrathoracic or subphrenic infections, or sepsis with symptoms that suggest pericardial involvement. Pericardiocentesis is indicated even in the absence of tamponade when the disease is confirmed, and appropriate antibiotic treatment should be instituted. Long-term prognosis is however excellent in patients that survive until discharge.
Purulent pericarditis has a high mortality, owing to the intangible diagnosis and the related severity of the underlying disease. Cardiac tamponade is frequent, and acute constrictive pericarditis may occur. The disease should be considered in all patients presenting with high fever, dyspnoea, and tachycardia with intrathoracic or subphrenic infections, or sepsis with symptoms that suggest pericardial involvement. Pericardiocentesis is indicated even in the absence of tamponade when the disease is confirmed, and appropriate antibiotic treatment should be instituted. Long-term prognosis is however excellent in patients that survive until discharge.


====Post myocardial infarction pericarditis====
===Post myocardial infarction pericarditis===
Pericardial effusion frequently occurs in the early stage after myocardial infarction, which remains asymptomatic and can be left untreated. Within the first week after myocardial infarction, acute pericarditis may occur, which is related to the extent of the infarction. The presence of a pericardial rub may distinguish chest pain and ECG changes resulting from acute pericarditis from recurrent ischemia.
Pericardial effusion frequently occurs in the early stage after myocardial infarction, which remains asymptomatic and can be left untreated. Within the first week after myocardial infarction, acute pericarditis may occur, which is related to the extent of the infarction. The presence of a pericardial rub may distinguish chest pain and ECG changes resulting from acute pericarditis from recurrent ischemia.


Weeks to months after myocardial infarction, pleuropericarditis of autoimmune nature may prevail, termed Dressler’s syndrome. However, this syndrome is rare, and treatment with corticosteroids yields a good prognosis.
Weeks to months after myocardial infarction, pleuropericarditis of autoimmune nature may prevail, termed Dressler’s syndrome. However, this syndrome is rare, and treatment with corticosteroids yields a good prognosis.


====Neoplastic pericarditis====
==Neoplastic pericarditis==
Lung cancer is the most frequent cause of neoplastic pericarditis. Cardiac tamponade in patients with a history of malignancy, in the absence of inflammatory signs indicates a possible malignant etiology, as is lack of response to NSAIDs in this patient group. When the effusion is indeed of malignant origin (approximately 40% of cases), treatment aims at alleviation of symptoms and the prevention of recurrences. A balance should be sought between pericardiocentisis in which recurrence is frequent, and pericardiectomy, which may be overly aggressive in this critically ill subset of patients.
Lung cancer is the most frequent cause of neoplastic pericarditis. Cardiac tamponade in patients with a history of malignancy, in the absence of inflammatory signs indicates a possible malignant etiology, as is lack of response to NSAIDs in this patient group. When the effusion is indeed of malignant origin (approximately 40% of cases), treatment aims at alleviation of symptoms and the prevention of recurrences. A balance should be sought between pericardiocentisis in which recurrence is frequent, and pericardiectomy, which may be overly aggressive in this critically ill subset of patients.


====Hypothyroidism====
===Hypothyroidism===
With increasing severity of primary hypothyroidism, the prevalence of pericardial effusion increases. Thyroid hormone replacement therapy results in remission of the effusion.
With increasing severity of primary hypothyroidism, the prevalence of pericardial effusion increases. Thyroid hormone replacement therapy results in remission of the effusion.


====Post-pericardiotomy pericarditis====
===Post-pericardiotomy pericarditis===
Pericarditis is common after cardiac surgery (18%), of which the etiology is unclear although an autoimmune origin has been proposed. In contrast to other forms of pericarditis, post-pericardiotomy pericarditis may be effectively treated with corticosteroids and NSAIDs.
Pericarditis is common after cardiac surgery (18%), of which the etiology is unclear although an autoimmune origin has been proposed. In contrast to other forms of pericarditis, post-pericardiotomy pericarditis may be effectively treated with corticosteroids and NSAIDs.
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