Download PERICARDIAL DISEASES

PERICARDIAL
DISEASES
PROF.DR. FERDA ÖZKAN
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To learn the structure of pericardium
To learn the types of pericardial
effusions
To learn the types of pericarditis
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The pericardium is comprised of
the parietal pericardium (an
outer fibrous layer) and the
visceral pericardium (an inner
serous membrane made of a
single layer of mesothelial cells).
The visceral pericardium is
attached to the epicardial fat and
reflects back on itself to form the
parietal pericardium.
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Pericardial physiology includes 3
main functions.
• First, mechanical function, the
pericardium promotes cardiac efficiency
by limiting acute dilation, maintaining
ventricular compliance with
preservation of the Starling curve, and
distributing hydrostatic forces.
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It also creates a closed chamber with
subatmospheric pressure that aids atrial
filling and lowers transmural cardiac
pressures.
•Second, membranous function:
-shields the heart by reducing
external friction and acting as a
barrier against extension of
infection and malignancy.
•Third, ligamentous function:
-the pericardium fixes the heart
anatomically.
Pericardial lesions
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Pericardial lesions are almost always
associated with disease in other portions
of the heart or surrounding structures or
secondary to a systemic disorder;
isolated pericardial disease is unusual.
Despite the large number of etiologies of
pericardial disease, there are relatively
few anatomic forms of pericardial
involvement.
Pericardial Effusion
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Normally there is about 30 to 50 ml of
thin, clear, straw-colored, translucent
fluid in the pericardial space.
Under a variety of circumstances,
pericardial effusions may appear
An effusion accumulates slowly and is
rarely larger than 500 ml
Rarely a large volume or rapid
accumulation of a lesser volume may
embarrass diastolic filling of the heart.
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Serous effusion (in cardiac failure)
the fluid is completely clear, or straw colored,
and sterile; the serosal surfaces remain
smooth.
Serosanguineous (in blunt chest trauma)
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Chylous effusions (in lymphatic
obstruction)
• contain lipid droplets
• benign or malignant mediastinal neoplasms.
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The pericardium normally
contains as much as 50 mL
of an ultrafiltrate of plasma.
Drainage occurs via the
thoracic duct and the right
lymphatic duct into the right
pleural space.
Hemopericardium
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Hemopericardium is the accumulation of
pure blood in the pericardial sac (distinct from
hemorrhagic pericarditis, a condition in which there is an
inflammatory exudate containing blood mixed with pus).
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Hemopericardium is almost invariably
due to rupture of the heart wall
secondary to
• myocardial infarction,
• traumatic perforation, or rupture of the
intrapericardial aorta.
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Blood rapidly fills the sac under
greatly increased pressure
Producing cardiac tamponade
Small amounts of blood may
result from the trauma sustained
during cardiopulmonary
resuscitation.
Pericarditis
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Inflammations of the pericardium are
usually secondary to a variety of
• cardiac diseases,
• to systemic disorders, or
• to metastases from neoplasms arising in
remote sites.
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Primary pericarditis is unusual and
almost always of viral origin.
The various etiologies usually evoke an
acute pericarditis,
Tuberculosis and fungi produce chronic
reactions.
Acute Pericarditis
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Acute pericarditis is an inflammation of the
pericardium characterized by chest pain,
pericardial friction rub, and serial
electrocardiographic changes.
Classification (Pathology):
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Serous Pericarditis
Fibrinous and Serofibrinous Pericarditis
Purulent or Suppurative Pericarditis
Hemorrhagic Pericarditis
Granulomatous Pericarditis.
Etiology
Bacterial
• 1-8% of cases and causes purulent
pericarditis.
• Direct extension:
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pulmonary extension, myocardial
abscess/endocarditis, penetrating injury to
chest wall from either trauma or surgery,
subdiaphragmatic suppurative lesion.
• Hematogenous spread :
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bacteriemia, sepsis, pyemia
• Organisms:
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Gram-positive species such as
Streptococcus pneumoniae and other
Streptococcus species, Staphylococcus, and
gram-negative species, including Proteus,
Escherichia coli, Pseudomonas, Klebsiella,
Salmonella, Shigella, Neisseria meningitidis,
and Haemophilus influenzae.
Less common organisms include Legionella,
Nocardia, and Actinobacillus, Rickettsia and
Lyme borreliosis (Borrelia burgdorferi).
Anaerobes also have been isolated in 40%
of patients in reviews of the pediatric
population.
Tuberculosis
 This accounts for 4% of cases.
 Especially in high-risk groups such as
elderly patients in nursing homes.
 Elevated adenosine deaminase in
pericardial fluid is useful for
diagnosing tuberculosis.
 High adenosine deaminase values
may indicate a poorer prognosis.
 Approximately half the patients
develop constrictive pericarditis.
Viral
• 1-10% of cases.
• Causative viruses include coxsackievirus B,
echovirus, adenoviruses, influenza A and B
viruses, Enterovirus, mumps virus, EpsteinBarr virus, human immunodeficiency virus
(HIV), herpes simplex virus type 1, varicella
zoster virus, measles virus, parainfluenza 2
virus, and respiratory syncytial virus.
• Viruses, especially coxsackie B and influenza,
can occur as seasonal epidemics.
• It usually is a short self-limited disease lasting
1-3 weeks.
• Patients may have associated myocarditis.
• HIV:
Pericardial involvement is frequent, but
it usually is an asymptomatic pericardial
effusion of small volume.
It is found more frequently in advanced
HIV infection, with one study noting
right atrial diastolic compression in 5%
of cases. Symptomatic pericarditis is
observed in less than 1%.
Etiology can include usual causes,
opportunistic infection, Kaposi sarcoma,
and HIV virus.
Other infectious agents include the
following:
• Fungal organisms include Histoplasma,
Blastomyces, Coccidioides, Aspergillus, and
Candida.
• Parasitic organisms include Entamoeba,
Echinococcus, and Toxoplasma.
Idiopathic
• Between 26% and 86% of cases are idiopathic
in nature.
• No clinical features distinguish these from
viral pericarditis.
• Most likely, the majority of idiopathic cases
are undiagnosed viral infections.
• Seasonal peaks occur in spring and fall.
Rheumatoid arthritis
• Autopsy studies show incidence of 11-50% in patients
with rheumatoid arthritis (RA).
• Pericardial involvement usually is clinically silent.
• Diagnosis is suggested by serous or hemorrhagic -pericardial fluid with a glucose level of less than 45
mg/dL,
-white blood cell count higher than 15,000/mm2 with
-cytoplasmic inclusion bodies,
-protein level higher than 5 g/dL,
-low total serum hemolytic complement (CH50),
-high immunoglobulin G (IgG) level, and
-high rheumatoid factor.
-Cholesterol levels may be high in the fluid of patients
with RA who have nodules.
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Systemic lupus erythematosus
• 25% of patients with lupus.
• Autopsy series reveal pericardial involvement
in 62%.
• Fluid analysis reveals
-increased leukocytes of (PMN)
- high protein,
- low-to-normal glucose,
- low complement, and possibly a
- pH of less than 7.
- Also, it will have positive autoantibodies, such
as the antinuclear antibody or anti–doublestranded DNA..
• Development of tamponade and constrictive
pericarditis is rare.
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Scleroderma
• 5-10% of patients with scleroderma.
• Autopsy incidence rate is 70%.
• Pericardial effusions are observed in
40% of patients and can be due to
scleroderma, myocardial failure
(restrictive cardiomyopathy), and
renal failure.
• Restrictive cardiomyopathy and
pericardial constriction can coexist.
• Usually, pulmonary hypertension, right
heart failure, and systolic dysfunction
are observed.
• These patients have a poor prognosis.
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Rheumatic fever
• It often accompanies endocarditis
and myocarditis, with a worse
prognosis.
• In adults, pericarditis may not
occur with myocardial or valvular
involvement, and it is associated
with a better prognosis.
• Antistreptolysin O titer usually is
greater than 400.
• Rarely is a large effusion present.
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Sarcoidosis
•Pericarditis is present in
autopsies of approximately
25% of these patients.
•Patients may not have
significant myocardial
infiltration.
•It rarely causes cardiac
tamponade or constrictive
pericarditis.
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Pericarditis is described in other
inflammatory conditions, including the
following:
• Sjögren syndrome
• Mixed connective tissue disease
• Reiter syndrome
• Ankylosing spondylitis
• Inflammatory bowel disease
• Wegener granulomatosis
• Vasculitis, such as giant cell arteritis and
polyarteritis
• Polymyositis
• Behçet syndrome
• Whipple disease
• Familial Mediterranean fever
• Serum sickness
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Renal failure
• Renal failure accounts for approximately
12% of cases.
• In the predialysis era, pericarditis developed
in 35-50% of patients with uremia who had
chronic renal failure and less commonly in
those with acute renal failure.
• Death often followed in several weeks.
• With dialysis, the incidence rate is less than
10%.
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Pericarditis occurs after the onset of dialysis in 812%.
Some authors suggest that uremic pericarditis is a
different entity from dialysis-associated pericarditis.
Asymptomatic pericardial effusions can occur in 3662% of patients with uremia who require dialysis.
The presence of a large pericardial effusion that
persists for longer than 10 days after intensive
dialysis has a high likelihood of causing tamponade.
Treatment is intensive dialysis.
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Hypothyroidism
• 4% of cases.
• Pericardial involvement usually is
observed with severe hypothyroidism.
• Patients may develop large pericardial
effusions, but rarely do they develop
tamponade.
• Pericardial fluid often is clear with high
protein and cholesterol levels and few
cells.
• Myocardial involvement is common.
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Cholesterol pericarditis
•This also is called gold-paint
pericarditis.
•Granulomatous pericarditis has
been implicated in some cases.
•Development of constriction is
rare.
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Myocardial infarction
• After a transmural infarction, a fibrinous pericardial
exudate appears within 24 hours. It begins to
organize at 4-8 days, and it completes organization
at 4 weeks.
• Prior to thrombolytic therapy, infarct-associated
pericarditis ranges from 7-23%.
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At autopsy, almost all patients were noted to have localized
fibrinous pericarditis overlying the area of infarction.
• With thrombolytic therapy and direct infarct
angioplasty, the incidence of infarct-associated
pericarditis has decreased to 5-8%.
• Overall, pericardial involvement indicates larger
infarction, greater incidence of left ventricular
dysfunction, and greater mortality.
• Pericarditis usually heals without consequence.
• Effusions may occur, but they rarely lead to
tamponade.
Dressler syndrome
• Pericarditis associated with Dressler
syndrome usually is observed 2-3 weeks
after a myocardial infarction.
• It may be a unique autoimmune-mediated
phenomenon to myocardial antigens, or it
may merely be an unrecognized
postmyocardial infarction pericarditis.
• Patients may develop pulmonary infiltrates
and large pericardial effusions.
• Because of the risk of hemorrhagic
pericarditis, anticoagulant therapy should be
stopped.
Postpericardiotomy syndrome
• This is similar to Dressler syndrome, except it
occurs after cardiac surgery.
• Several series note an incidence rate of 1040%.
• Approximately 1% develop tamponade.
• Pericardial effusions can occur in the absence
of typical features of postpericardiotomy
syndrome.
• The effusions were more common after heavy
postoperative bleeding.
• Pericarditis is observed with other cardiac
instrumentation, including percutaneous
transluminal coronary angioplasty (PTCA) and
pacemaker implantation.
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Usually, it is associated with epicardial/pericardial
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Neoplasm
• 5-17 % of cases.
• Neoplastic disease, particularly advanced, is
the most frequent cause of tamponade in
the hospital.
• Occasionally, the tumor encases the heart
and causes constrictive pericarditis rather
than tamponade.
• Primary neoplasm of the heart and
pericardium are rare.
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Pericardial mesothelioma and
angiosarcoma are lethal malignancies
with aggressive local spread that
respond poorly to treatment. Infants
and children can present with a
teratoma in the pericardial space.
These often can be removed
successfully.
• Most cases occur as a result of
metastatic disease.
Autopsy studies have noted that
approximately 10% of patient with
cancer, develop cardiac
involvement, and often, it is
clinically silent.
The neoplastic cells reach the
pericardium through the
bloodstream via lymphatic
system, or local growth.
• Metastatic tumors:
 Lung cancer, including
adenocarcinoma, squamous cell,
and small cell, accounts for
approximately 33% of cases.
 Breast cancer accounts for 25%.
 Leukemia and lymphoma, including
Hodgkin and non-Hodgkin, account
for 15% of cases.
 Malignant melanoma represents
another 5%.
 Kaposi sarcoma with the AIDS.
• The pericardial carcinoembryonic
antigen (CEA) level often is elevated.
•Cytology is positive in 80-90%
of breast and lung cancer
cases, but the percentage is
lower in other malignancies.
Obstruction of the lymphatic
drainage can cause the
pericardial effusion to be more
significant than the tumor
mass.
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Irradiation
•Pericardial disease is the most
common cardiac toxicity.
•High incidence is observed
with high doses, especially
greater than 4000 rad.
•It can present as acute
pericarditis, with or without
effusion; chronic constrictive
pericarditis; or effusiveconstrictive pericarditis.
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Drugs
• Some medications, including penicillin and
cromolyn sodium, induce pericarditis through
a hypersensitivity reaction.
• The anthracycline antineoplastic agents, such
as doxorubicin and cyclophosphamide, have
direct cardiac toxicity and can cause acute
pericarditis and myocarditis.
• Pericarditis can develop from a drug-induced
SLE syndrome from medications, including
procainamide, hydralazine, methyldopa,
isoniazid, mesalazine, and reserpine.
• Methysergide causes constrictive pericarditis
through mediastinal fibrosis.
• Dantrolene, phenytoin, and minoxidil produce
pericarditis through an unknown mechanism.
Serous Pericarditis
 Serous inflammatory exudates are
characteristically produced by
noninfectious inflammations, such as RF
systemic lupus erythematosus,
scleroderma, tumors, and uremia.,
 In some instances, a well-defined viral
infection elsewhere –upper respiratory
tract, pneumonia, parotitis– antedates
the pericarditis and serves as the
primary focus of infection.
Serous Pericarditis
This diagram depicts the
appearance of a serous
pericarditis. The amount of
inflammation is minimal,
so no exudation of fibrin
occurs. The dark stippled
dots in the yellow fluid and
on the epicardial surface
represent scattered
inflammatory cells. Serous
pericarditis is marked by
fluid collection. Rarely, the
fluid collection may be
large enough to cause
tamponade.
Fibrinous and Serofibrinous Pericarditis
 Most frequent type of pericarditis
 a more or less serous fluid mixed with a
fibrinous exudate.
 Common causes include
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acute myocardial infarction,
the postinfarction (Dressler’s) syndrome
uremia,
chest radiation,
RF,
systemic lupus erythematosus,
trauma
cardiac surgery
pneumonia.
Fibrinous Pericarditis
This diagram depicts
the appearance of a
fibrinous pericarditis.
The red-pink squiggly
lines extending from
the epicardial surface
into the yellow fluid
represent the strands
of fibrin. This type of
pericarditis is typical
of uremia with renal
failure, underlying
myocardial infarction,
and acute rheumatic
carditis
Fibrinous Pericarditis
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A window of adherent
pericardium has been
opened to reveal the
surface of the heart.
There are thin strands
of fibrinous exudate
that extend from the
epicardial surface to
the pericarial sac. This
is typical for a
fibrinous pericarditis
Fibrinous Pericarditis
This is an example
of a fibrinous
pericarditis. The
surface appears
roughened from
the normal
glistening
appearance by the
strands of pink-tan
fibrin.
Fibrinous Pericarditis
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The epicardial surface of
the heart shows a shaggy
fibrinous exudate. This is
another example of
fibrinous pericarditis. This
appearance has often been
called a "bread and butter"
pericarditis, but you would
have to drop your buttered
bread on the carpet to
really get this effect. The
fibrin often results in the
the finding on physical
examination of a "friction
rub" as the strands of
fibrin on epicardium and
pericardium rub against
each other.
The pericardial surface here shows strands of pink fibrin
extending outward. There is underlying inflammation. Eventually,
the fibrin can be organized and cleared, though sometimes
adhesions may remain.
Purulent or Suppurative Pericarditis
 The invasion of the pericardial space by
infective organisms.
 The routes of organisms:
• (1) direct extension from neighboring
inflammations, such as an empyema of the pleural
cavity, lobar pneumonia, mediastinal infections, or
extension of a ring abscess through the
myocardium or aortic root in infective endocarditis
• (2) seeding from the blood;
• (3) lymphatic extension;
• (4) direct introduction during cardiotomy.
Immunosuppressive therapy potentiates all of these
pathways.
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The exudate ranges from a thin to a
creamy pus of up to 400 to 500 ml in
volume.
The serosal surfaces are reddened,
granular, and coated with the
exudate.
Microscopically there is an acute
inflammatory reaction.
Sometimes the inflammatory process
extends into surrounding structures
to induce a so-called
mediastinopericarditis.
Acute purulent pericarditis
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An exudate is an
inflammatory extravascular
fluid that has a specific
gravity greater than 1.020
and contains a large
amount of protein and
cellular debris. This is an
example of an exudate
that is seen with
pericarditis and is often
called bread and butter
pericarditis due to its
appearance. Purulent is
synonymous with
suppurative and implies a
non-viral etiology.
This is a purulent pericarditis. Note the yellowish
exudate that has pooled in the lower pericardial
sac seen been opened here.
Etiology:
• A preexisting or concurrent infection such as
pneumonia with or without empyema,
meningitis, osteomyelitis, arthritis, or other
soft tissue infections.
• Patients recovering from thoracic, cardiac, or
esophageal surgery are at risk for purulent
pericarditis.
• Purulent pericarditis has been reported in
patients recovering from traumatic injury.
• The organisms that most commonly cause
purulent pericarditis are Staphylococcus
aureus, H influenzae, Neisseria meningitidis,
and Streptococcus pneumoniae.
 Less common organisms include gramnegative enteric bacilli, Pseudomonas
aeruginosa, Salmonella species,
Francisella tularensis, and anaerobic
bacteria and fungi (Histoplasma species,
coccidioidomycosis, blastomycosis,
Aspergillus species, Candida species).
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S. aureus
• This is the most common organism in
children, causing approximately 40% of
cases.
• Pericarditis occurs concomitantly with
pneumonia with empyema and less
often with acute osteomyelitis or soft
tissue abscess.
• Rarely, pericarditis is associated with S
aureus endocarditis.
• Necrotizing infection and exotoxin
production lead to increased incidence
of shock and higher mortality risk.
• Within the first 3 months after cardiac
surgery, S aureus is the most common
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H. İnfluenzae
• This is the second most common
organism listed in most reported
pediatric case series of purulent
pericarditis,
• Infection of the upper or lower
respiratory tract frequently precedes
pericarditis caused by H influenzae.
• Purulent pericarditis may occur with H
influenzae meningitis.
• H influenzae produces very thick
fibrinopurulent exudate.
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N. meningitidis
• Pericarditis occurs in approximately 5% of
young adults with meningococcemia.
• The clinical course is often milder than with
other causes of purulent pericarditis.
• Pericardial effusion can be detected at the
onset of the illness or later in the course of the
infection.
S. pneumoniae:
• At one time, S pneumoniae was the leading
cause of purulent pericarditis, but it has
become much less common perhaps because
of widespread use of antibiotics.
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Other organisms
• Gram-negative enteric bacilli and anaerobes
or fungi, are rare but should be considered
in patients who are immunocompromised.
• Aspergillus pericarditis arises as a result of
pulmonary infection in patients who are
immunocompromised.
• Mycoplasma pneumoniae pericarditis is
associated with pulmonary disease.
Complications:
• Myocardial involvement
• Heart failure
• Constrictive pericarditis
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Constrictive pericarditis is a rare complication.
increased systemic venous pressure,
hepatomegaly, dyspnea, and decreased urine
output.
Hemorrhagic Pericarditis
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An exudate composed of blood mixed
with a fibrinous or suppurative effusion is
most commonly caused by
tuberculosis
direct malignant neoplastic involvement
cardiac surgery.
The surface of the heart with hemorrhagic pericarditis
demonstrates a roughened and red appearance.
Hemorrhagic pericarditis is most likely to occur with
metastatic tumor and with tuberculosis (TB). TB can also
lead to a granulomatous pericarditis that may calcify and
produce a "constrictive" pericarditis.
Granulomatous Pericarditis
 Tuberculosis, fungal infections,
rheumatoid arthritis, and sarcoidosis.
 Caseation within the pericardial sac is
tuberculous in origin
• Infrequently mycotic infections evoke a
similar pattern.
• The pericardium is usually involved by
direct spread from tuberculous foci within
the tracheobronchial nodes.
• Caseous pericarditis is the most frequent
antecedent of disabling, fibrocalcific,
chronic constrictive pericarditis.
Chronic or Healed Pericarditis
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Organization merely produces
• plaque-like fibrous thickenings
• thin, delicate adhesions (rarely cause impairment of
cardiac function)
• complete obliteration of the pericardial sac.
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This fibrosis yields a delicate, stringy type of
adhesion between parietal and visceral
pericardium called adhesive pericarditis, which
rarely hampers or restricts cardiac action.
In some cases, however, healed pericarditis
can be clinically important, especially when it
takes the form of adhesive
mediastinopericarditis or constrictive
pericarditis.
Adhesive Mediastinopericarditis
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May follow:
• a suppurative or caseous pericarditis,
• previous cardiac surgery,
• irradiation to the mediastinum.
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The pericardial sac is obliterated, and
adherence of the external aspect of the
parietal layer to surrounding structures
produces a great strain on cardiac
function.
The increased workload causes cardiac
hypertrophy and dilatation, which
Constrictive Pericarditis
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The heart may be encased in a dense, fibrous,
or fibrocalcific scar that limits diastolic
expansion and seriously restricts cardiac
output.
History of a previous suppurative,
hemorrhagic, or caseous (tuberculous)
pericarditis, but more often the cause is
uncertain.
The pericardial space is obliterated, and the
heart is surrounded by a dense, adherent
layer of scar or calcification, often 0.5 to 1.0
cm thick, that can resemble a plaster mold in
extreme cases (concretio cordis).
Cardiac hypertrophy and dilatation cannot
occur because of the dense enclosing scar,
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Etiology:
• Chronic constrictive pericarditis develops
insidiously; frequently, an etiology is never
determined.
• In some (approximately 10%) patients, an
antecedent acute pericarditis exists.
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Tuberculosis is the most frequent known cause of
chronic constrictive pericarditis.
Other causal organisms include (among others)
staphylococci, streptococci, and fungi such as
histoplasmosis.
Rheumatoid disease
Sarcoidosis
Mediastinal radiation
Trauma (hemopericardium)
Status post–cardiac surgery (postpericardiotomy
syndrome)
Uremia
Neoplastic disease
Postinfectious (bacterial) pericarditis
Various metabolic and genetic disorders.
Pericardium in Rheumatoid arthritis
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The heart is involved in 20 to 40% of cases of
severe prolonged rheumatoid arthritis.
Fibrinous pericarditis (dense fibrous adhesions)
In the early stages of this process, rheumatoid
inflammatory granulomatous nodules resembling those
that occur subcutaneously may be identifiable deep to
the pericardial surfaces (rheumatoid nodules may
involve the myocardium, endocardium, valves of the
heart, and root of the aorta).
Amyloid involving the heart can also occur secondary
to rheumatoid arthritis.
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