Transcript Chest X-ray

PERICARDIAL DISEASES
GENERAL CONSIDERATIONS
• The pericardium consists of two layers: the inner visceral layer,
which is attached to the epicardium, and an outer parietal layer.
• About 50 mL of serous fluid is normally present and provides
lubrication between the two layers.
• The pericardial reflection encompasses the heart and great vessels.
• The pericardium stabilizes the heart in anatomic position and
reduces contact between the heart and the surrounding structures.
• It is composed of fibrous tissue and although it will permit
moderate changes in cardiac size, it cannot stretch rapidly enough
to accommodate rapid dilation of the heart or accumulation of fluid
without increasing intrapericardial (and, therefore, intracardiac)
pressure.
• The pericardium is often involved by processes that affect the
heart, but it may also be affected by diseases of adjacent tissues
and may itself be a primary site of disease.
Physiology of the pericardium
• The normal pericardium is not essential to life:
the pericardial space is often obliterated after
open heart surgery and both layers may be
removed in patients with constrictive
pericarditis without apparent ill effect.
• Whether restraint by the normal pericardium
is of any pathophysiological importance as a
mechanism limiting stroke volume in disease
remains uncertain.
Acquired pericardial disease
• Diseases of the pericardium may be considered
from two points of view.
• The first is etiological, the second is in terms of
the physiological and clinical disturbances that
result.
• There is no fixed relation between the two, so
that an account will be given of the different
diseases affecting the pericardium and then of
the three main syndromes: acute pericarditis,
pericardial tamponade and pericardial
constriction.
Pericarditis
• Pericarditis is a
condition in which the
sac-like covering
around the heart
(pericardium) becomes
inflamed.
• Pericarditis and
cardiac tamponade
involve the potential
space surrounding the
heart or pericardium.
• Pericarditis is one
cause of fluid
accumulation in this
potential space and
cardiac tamponade is
the hemodynamic
result of fluid
accumulation.
Who gets pericarditis and what does
it feel like?
• This problem occurs most often in men ages 20 to 50.
• Chest pain is common, especially pain behind the
breastbone. Sometimes this pain spreads to the neck
and left shoulder.
• Pain from pericarditis is different from angina. Angina
feels like pressure, but pericarditis usually is a sharp,
piercing pain over the center or left side of the chest.
Often this pain gets worse if the person takes a deep
breath. Less often the pain is dull.
• A fever is also common.
• Often people with pericarditis report feeling sick.
• Some have pain when they swallow.
Acute pericarditis
• By far the most common pathologic process
involving the pericardium.
• May be classified both clinically and etiologically.
• Acute (< 2 weeks) inflammation of the
pericardium may be infectious in origin or may be
due to systemic diseases (autoimmune
syndromes, uremia), neoplasm, radiation, drug
toxicity, hemopericardium, postcardiac surgery or
contiguous inflammatory processes in the
myocardium or lung.
Acute pericarditis
• Incidence – Post mortem 1-6%, diagnosed in
only 0.1% of hospitalized patients. 5% of
patients seen in emergency rooms with CP
and no MI.
• Sequelae – Cardiac tamponade
Recurrent pericarditis
Pericardial constriction
Etiology
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Viral or idiopathic
After MI
Infectious diseases
With dissecting aortic aneurysms
Trauma
Metastasis
XRT
Uremia
After cardiac or other thoracic surgery
Autoimmune diseases
Medications
Viral infections
• especially infections with coxsackieviruses and
echoviruses but also influenza, Epstein–Barr,
varicella, hepatitis, mumps and HIV viruses are
the most common cause of acute pericarditis and
probably are responsible for many cases classified
as idiopathic.
• Males—usually under age 50 years—are most
commonly affected.
• The differential diagnosis is primarily with
myocardial infarction.
Tuberculous pericarditis
• has become rare in developed countries but
remains common in other areas
• It results from direct lymphatic or
hematogenous spread; clinical pulmonary
involvement may be absent or minor,
although associated pleural effusions are
common.
Bacterial pericarditis
• has become rare and usually results from
direct extension from pulmonary infections
• Pneumococci can cause a primary pericardial
infection.
• Borrelia burgdorferi, the organism
responsible for Lyme disease, can also cause
myopericarditis.
Uremic pericarditis
• is a common complication of renal failure
• the pathogenesis is uncertain
• it occurs both with untreated uremia and in
otherwise stable dialysis patients
Neoplastic pericarditis
• Spread of adjacent lung cancer as well as
invasion by breast cancer, renal cell
carcinoma, Hodgkin disease and lymphomas
are the most common neoplastic processes
involving the pericardium and have become
the most frequent causes of pericardial
tamponade in many countries.
Dressler syndrome
• Pericarditis may occur 2–5 days after
infarction due to an inflammatory reaction to
transmural myocardial necrosis
postmyocardial infarction or postcardiotomy
pericarditis .
Post-radiotherapy pericarditis
• Radiation can initiate a fibrinous and fibrotic
process in the pericardium, presenting as
subacute pericarditis or constriction.
• Radiation pericarditis usually follows
treatments of more than 4000 cGy delivered
to ports including more than 30% of the heart.
Other causes of pericarditis
• connective tissue diseases, such as lupus
erythematosus and rheumatoid arthritis
• drug-induced pericarditis (minoxidil,
penicillins)
• myxedema
Clinical presentation
• History
• Physical exam - 85% have audible friction rub during
the course of their disease ( the rub is high pitched
scratchy or squeaky sound best heard at the left
sternal border at end of expiration with the patient
leaning forward)
• The rub has three components- atrial systole,
ventricular systole and rapid ventricular filling during
early diastole.
Symptoms and Signs
• The presentation and course of inflammatory
pericarditis depend on its cause, but all syndromes are
often (not always) associated with chest pain, which is
usually pleuritic and postural (relieved by sitting).
• The pain is substernal but may radiate to the neck,
shoulders, back or epigastrium.
• Dyspnea may also be present and the patient is often
febrile.
• A pericardial friction rub is characteristic , with or
without evidence of fluid accumulation or constriction
Clinical findings
• There are three main components to the
clinical syndrome of acute pericarditis:
1. chest pain
2. pericardial rub
3. ECG changes
Chest pain
• The pain is usually retrosternal, continuous and
sharp or 'raw' in character.
• It is frequently aggravated by sudden movements
or deep inspiration and is relieved by sitting up.
• Less commonly it may resemble angina pectoris,
or may be mild and 'atypical'.
• Painful breathing causes dyspnoea.
• The onset of the pain is usually sudden, but in
idiopathic pericarditis, it may have been preceded
by several days' malaise or other non-specific
symptoms.
Pericardial pain
• Characteristically, however, pericardial pain
may be relieved by sitting up and leaning
forward and is intensified by lying supine.
Pericardial friction rub
• the most important physical sign of acute
pericarditis
• may have up to three components per cardiac
cycle
• high-pitched, scratching and grating
• it is heard most frequently during expiration with
the patient in an upright and leaning forward
position
• the rub is often inconstant and the loud to-andfro leathery sound may disappear within a few
hours, possibly to reappear the following day
Tuberculous pericarditis
• The presentation tends to be subacute, but
nonspecific symptoms (fever, night sweats,
fatigue) may be present for days to months.
• Pericardial involvement develops in 1–8% of
patients with pulmonary tuberculosis.
Bacterial pericarditis
• Symptoms and signs are similar to those of
other types of inflammatory pericarditides,
but patients appear toxic and are often
critically ill.
Uremic pericarditis
• can present with or without symptoms
• fever is absent
Neoplastic pericarditis
• often is painless
• the presenting symptoms relate to
hemodynamic compromise or the primary
disease
Postmyocardial infarction or postcardiotomy
pericarditis (Dressler syndrome)
• Usually presents as a recurrence of pain with pleuralpericardial features
• A rub is often audible, and repolarization changes on the
ECG may be confused with ischemia.
• Large effusions are uncommon and spontaneous resolution
usually occurs in a few days.
• Dressler syndrome occurs weeks to several months after
myocardial infarction or open heart surgery, may be
recurrent and probably represents an autoimmune
syndrome.
• Patients present with typical pain, fever, malaise and
leukocytosis.
• Rarely, other symptoms of an autoimmune disorder, such
as joint pain and fever, may occur.
• Tamponade is rare with Dressler syndrome after myocardial
infarction but not when it occurs postoperatively.
Radiation pericarditis
• The clinical onset is usually within the first
year but may be delayed for many years.
PERICARDIAL EFFUSION
• Pericardial effusion can develop during any of the
processes previously discussed.
• The speed of accumulation determines the physiologic
importance of the effusion.
• Because the pericardium stretches, large effusions (>
1000 mL) that develop slowly may produce no
hemodynamic effects. Conversely, smaller effusions
that appear rapidly can cause tamponade.
• Tamponade is characterized by elevated
intrapericardial pressure (> 15 mm Hg), which restricts
venous return and ventricular filling. As a result, the
stroke volume and pulse pressure fall and the heart
rate and venous pressure rise. Shock and death may
result.
Pericardial effusion
• Differentiation from cardiac enlargement may be
difficult on physical examination, but heart sounds
tend to become faint with pericardial effusion; the
friction rub may disappear and the apex impulse may
vanish, but sometimes it remains palpable, albeit
medial to the left border of cardiac dullness.
• The base of the left lung may be compressed by
pericardial fluid, producing Ewart’s sign, a patch of
dullness beneath the angle of the left scapula.
• The chest roentgenogram may show a “water bottle”
configuration of the cardiac silhouette, but may also be
normal or almost so.
Symptoms
• Pericardial effusions may be associated with
pain if they occur as part of an acute
inflammatory process or may be painless, as is
often the case with neoplastic or uremic
effusion.
• Dyspnea and cough are common, especially
with tamponade.
• Other symptoms may result from the primary
disease.
SIGNS
• A pericardial friction rub may be present even with large
effusions .
• In cardiac tamponade, tachycardia, tachypnea, a narrow
pulse pressure and a relatively preserved systolic pressure
are characteristic.
• Pulsus paradoxus—a greater than 10 mm Hg decline in
systolic pressure during inspiration due to further
impairment of LV filling—is the classic finding, but it may
also occur with obstructive lung disease.
• Central venous pressure is elevated and there is no evident
y descent in the RA, RV or LV hemodynamic tracings.
• Edema or ascites are rarely present; these signs favor a
more chronic process.
Investigations
• Electrocardiogram
• Chest X-ray
• Echocardiography
ECG
• in acute pericarditis without massive effusion usually
displays changes secondary to acute subepicardial
inflammation
• usually there are no significant changes in QRS complexes,
except for some reduction in voltage in patients with large
pericardial effusions
• elevation of the ST segments, often with upward concavity,
involving two or three standard limb leads and V2 to V6,
with reciprocal depressions only in aVR and sometimes V
EKG
• Stages of pericarditis:
I- Diffuse ST elevation and PR segment depression
(seen in more than 80%)
II- Normalization of the ST and PR
III- Widespread T- wave inversions
IV- Normalization of the T waves
• The most reliable distinguishing feature may be the
ratio of ST segment elevation (in millimeters) to Twave amplitude in lead V6; ratio > 0.24
PERICARDIAL DISEASE
This 12-lead electrocardiogram is
representative of pericarditis.
Stage 1 electrocardiograph changes in a
patient with acute pericarditis.
Stage 2 ECG changes in patient with acute
pericarditis obtained 3 days into clinical course.
ECG criteria:
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ACUTE PERICARDITIS:
Concave (saddle-shaped) ST segment elevation;
ST may be normal or nonspecific (10% cases).
PERICARDIAL EFFUSION:
Low voltage QRS complexes;
Alternating QRS morphologies (electrical alternans).
CONSTRICTIVE PERICARDITIS:
Large T wave and bifid;
Low voltage QRS complexes;
ST plan, isoelectric or depressed.
CARDIAC TAMPONADE:
Low voltage QRS complexes;
Electrical alternans.
Chest X-ray
• may suggest
enlargement of heart
tissue and can be used
to rule out other
problems within the
chest
Chest radiographs revealing markedly enlarged cardiac silhouette and normal-appearing
lung parenchyma in prepericardiocentesis (A) and postpericardiocentesis (B).
Echo
• The cardiologist looks
for the presence of fluid
in the pericardial sac,
although in many mild
cases of acute
pericarditis, there is no
pericardial fluid seen
with echocardiography.
This ultrasonogram demonstrates
a normal subcostal 4-chamber
view of the heart. The pericardium
is brightly reflective (echogenic or
white in appearance). LA = left
atrium; LV = left ventricle; RA =
right atrium; RV = right ventricle.
This is a modified subcostal ultrasonographic
view of the heart in which a thick anechoic
(dark or black) stripe is seen surrounding the
heart (H). The stripe represents a large amount
of fluid (F) in the pericardium, indicating
tamponade.
Essentials of Diagnosis
• Anterior pleuritic chest pain that is worse
supine than upright.
• Pericardial rub.
• Fever common.
• Erythrocyte sedimentation rate usually
elevated.
• ECG reveals diffuse ST segment elevation with
associated PR depression.
Cardiac tamponade
• Cardiac tamponade is a clinical syndrome
caused by the accumulation of fluid in the
pericardial space, resulting in reduced
ventricular filling and subsequent
hemodynamic compromise.
• Cardiac tamponade is a medical emergency.
• The overall risk of death depends on the
speed of diagnosis, the treatment provided
and the underlying cause of the tamponade.
Reddy et al describe 3 phases of
hemodynamic changes in tamponade
• Phase I: The accumulation of pericardial fluid causes
increased stiffness of the ventricle, requiring a higher
filling pressure. During this phase, the left and right
ventricular filling pressures are higher than the
intrapericardial pressure.
• Phase II: With further fluid accumulation, the
pericardial pressure increases above the ventricular
filling pressure, resulting in reduced cardiac output.
• Phase III: A further decrease in cardiac output occurs,
which is due to equilibration of pericardial and left
ventricular (LV) filling pressures.
Symptoms
• Patients with pericardial tamponade often
complain of shortness of breath, but the
diagnosis is most commonly made by noting the
characteristic physical examination findings
associated with pericardial tamponade.
• Dyspnea is the most common symptom of
pericardial tamponade. The pathogenesis
probably relates to a reduction in cardiac output
and, in some patients, the presence of pulmonary
edema.
Clinical findings
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Systemic arterial hypotension
Tachycardia
Elevated JVP
Pulsus paradoxus (most sensitive but not
specific)
• 15% of patients with idiopathic pericarditis or
as many as 60% of those with neoplastic,
tuberculous or purulent pericarditis can
present with cardiac tamponade.
The Beck triad or acute compression triad
• Described in 1935, this complex of physical
findings refers to increased jugular venous
pressure, hypotension and diminished heart
sounds.
• These findings result from a rapid
accumulation of pericardial fluid.
Pulsus paradoxus or paradoxical pulse
• This is an exaggeration (>12 mm Hg or 9%) of
the normal inspiratory decrease in systemic
blood pressure.
• The paradox is that while listening to the
heart sounds during inspiration, the pulse
weakens or may not be palpated with certain
heartbeats, while S1 is heard with all
heartbeats.
ECG
• ECG shows tachycardia, often with low-voltage
QRS complexes, but without Q waves or
conduction disturbances. If the effusion is
large, electrical alternans is present, when
alternate QRS complexes show differing
morphology, because the heart swings to and
fro in a large (therefore usually malignant)
effusion.
Electrocardiogram from a patient with massive malignant pericardial effusion showing electrical
alternans. Note that all are sinus beats with the same PR interval, but that the QRS axis
alternates.
Chest X-ray
• Chest radiography shows a large globular heart,
similar to that seen in dilated cardiomyopathy.
• More useful in making the diagnosis, therefore, is
the absence of any evidence of pulmonary
congestion, which would be expected if
myocardial disease were the main abnormality.
• Pulmonary oedema is most unusual in pure
tamponade: if it is present, it suggests additional
myocardial disease.
Chest X-ray
• Chest radiography
findings may show
cardiomegaly, water
bottle–shaped heart
This anteroposterior-view chest
radiograph shows a massive bottleshaped heart and conspicuous
absence of pulmonary vascular
congestion.
Echocardiography
• An echo-free space
posterior and anterior
to the left ventricle and
behind the left atrium
• Swinging of the heart in
its sac
• Inferior vena cava
plethora with minimal
or no collapse with
inspiration
Echo
• Although echocardiography provides useful information,
cardiac tamponade is a clinical diagnosis
• Conditions that may simulate pericardial effusion on 2dimensional echocardiography include the following: A
large left pleural effusion
- Any tumor surrounding the heart
- Mitral annular calcification
- A descending thoracic aorta
- A catheter in the right ventricle
- An enlarged left atrium
- An annular subvalvular LV aneurysm
- A bronchogenic cyst
Echo Findings in Pre-Tamponade
Physiology
• Diastolic right ventricular collapse
• Right atrial collapse/inversion
• Exagerated respiratory variation in inflow
velocity
• Exagerated respiratory variation in inferior
vena cava flow
• Dilated IVC in the right setting
Echo-Guided Pericardiocentesis
• Multiple Echo windows should be used to determine
the distribution of the fluid. Specifically, the
distribution and depth from the surface of the chest
at which contact with the fluid is anticipated by the
pericardiocentesis needle should be determined. If
the location of a pericardiocentesis needle is in
question, agitated saline can be injected to further
define the location of the tip.
Pericardiocentesis and Biopsy
• In those with pericardial tamponade and in
those with known or suspected purulent or
neoplastic pericarditis.
• In a study involving 230 patients with acute
pericarditis in whom the cause was unknown,
pericardiocentesis and pericardial biopsy
provided a diagnosis in only 6% and 5%
respectively.
Laboratory evaluation
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Red and white cell count.
Cytology
TG
CTX
No evidence for PH, glucose, LDH and protein
measurement.
• PCR > 30 U/L for adenosine deaminase activity
may help in identifying MTB
• Clinical presentation should guide the ordering of
additional tests. Routine serologic testing, including
testing for ANA and RF, reveals a source for the
pericarditis for only 10-15% of patients.
• Plasma troponin concentrations are elevated in 3550% of patients with pericarditis. The magnitude of
the ST elevation appears to correlate with magnitude
of troponin elevation. A troponin elevation lasting
more than 2 weeks, suggests associated myocarditis.
Prognosis
Several indicators of poor prognosis:
• >38°C
• Subacute onset
• Immunosuppressed
• After trauma
• Anticoagulation use
• Myopericarditis
• Large effusion or tamponade
CHRONIC PERICARDITIS
• Chronic pericardial effusions are sometimes
encountered in patients without an
antecedent history of acute pericarditis.
• They may cause few symptoms per se, and
their presence may be detected by finding an
enlarged cardiac silhouette on chest
roentgenogram.
Tuberculosis
• It is important to consider this condition in a middleaged or elderly person with fever and enlargement of
the cardiac silhouette of undetermined origin, with or
without elevation of venous pressure.
• Weight loss, fever, and fatigability are sometimes
observed. Inasmuch as treatment is quite effective,
overlooking a tuberculous pericardial effusion may
have serious consequences.
• If the etiology of chronic pericardial effusion remains
obscure, a pericardial biopsy, preferably by a limited
thoracotomy, should be performed.
Myxedema
• Myxedema may be responsible for a pericardial
effusion that is sometimes massive but rarely, if
ever, causes cardiac tamponade.
• The cardiac silhouette is markedly enlarged and
an echocardiogram is necessary to distinguish
cardiomegaly from pericardial effusion.
• The diagnosis of myxedema is frequently
overlooked. It is important, therefore, to carry
out appropriate tests for thyroid function.
Other causes
• Neoplasms, SLE, rheumatoid arthritis, mycotic
infections, radiation therapy, pyogenic
infections, severe chronic anemia and
chylopericardium may also cause chronic
pericardial effusion and should be considered
and specifically looked for in such patients.
Tests
• Aspiration and analysis of the pericardial fluid are often
helpful in diagnosis, especially in patients with chronic
large effusions that are nonresponsive to nonsteroidal
anti-inflammatory drugs.
• Fluid should be sent for hematocrit, cell count, protein,
culture and cytology.
• In infections the organism can often be identified by
smear or culture and should lead to treatment with
appropriate systemic antibiotics.
• Grossly sanguineous pericardial fluid results most
commonly from a neoplasm, tuberculosis, uremia or
slow leakage from an aortic aneurysm.
Laboratory Findings and Diagnostic Studies
• The diagnosis of viral pericarditis is usually
clinical and leukocytosis is often present.
• Rising viral titers in paired sera may be
obtained for confirmation.
• Cardiac enzymes may be slightly elevated,
reflecting a myocarditic component.
• The echocardiogram is often normal or reveals
only a trivial amount of fluid during the acute
inflammatory process.
Laboratory Findings and Diagnostic Studies
• The diagnosis of tuberculous pericarditis can
be inferred if acid-fast bacilli are found
elsewhere.
• The tuberculous pericardial effusions are
usually small or moderate but may be large.
• The yield of organisms by pericardiocentesis is
low; pericardial biopsy has a higher yield but
may also be negative and pericardiectomy
may be required.
Laboratory Findings and Diagnostic Studies
• If bacterial pericarditis is suspected on clinical
grounds, diagnostic pericardiocentesis may be
of value.
Laboratory Findings and Diagnostic Studies
• In uremic patients not on dialysis, the
incidence of pericarditis correlates roughly
with the level of blood urea nitrogen (BUN)
and creatinine.
• The pericardium is characteristically "shaggy"
in uremic pericarditis and the effusion is
hemorrhagic and exudative.
Laboratory Findings and Diagnostic Studies
• The diagnosis of neoplastic pericarditis can
occasionally be made by cytologic
examination of the effusion or by pericardial
biopsy, but it may be difficult to establish
clinically if the patient has received
mediastinal radiation within the previous year.
• Neoplastic pericardial effusions develop over a
long period of time and may become quite
huge (> 2 L).
Laboratory Findings and Diagnostic Studies
• The sedimentation rate is high in
postmyocardial infarction or postcardiotomy
pericarditis.
• Large pericardial effusions and accompanying
pleural effusions are frequent.
• Myxedema pericardial effusions due to
hypothyroidism usually are characterized by
the presence of cholesterol crystals.
CHRONIC CONSTRICTIVE PERICARDITIS
• This disorder results when the healing of an
acute fibrinous or serofibrinous pericarditis or
a chronic pericardial effusion is followed by
obliteration of the pericardial cavity with the
formation of granulation tissue.
• The latter gradually contracts and forms a firm
scar, encasing the heart and interfering with
filling of the ventricles.
May follow
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trauma
cardiac operation of any type
mediastinal irradiation
purulent infection
histoplasmosis
neoplastic disease (especially breast cancer, lung cancer,
and lymphoma)
acute viral or idiopathic pericarditis
rheumatoid arthritis
SLE
chronic renal failure with uremia treated by chronic dialysis
Physiopathology
• The basic physiologic abnormality in symptomatic
patients with chronic constrictive pericarditis, as
in those with cardiac tamponade, is the inability
of the ventricles to fill because of the limitations
imposed by the rigid, thickened pericardium or
the tense pericardial fluid.
• In constrictive pericarditis, ventricular filling is
unimpeded during early diastole but is reduced
abruptly when the elastic limit of the pericardium
is reached, while in cardiac tamponade,
ventricular filling is impeded throughout diastole.
Symptoms
• slowly progressive dyspnea
• fatigue
• weakness
Signs
• Chronic edema, hepatic congestion and ascites are
usually present.
• Ascites often seems out of proportion to the degree of
peripheral edema. The examination reveals these signs
and a characteristically elevated jugular venous
pressure.
• Kussmaul sign—a failure of the JVP to fall with
inspiration—is also a frequent finding.
• The apex may actually retract with systole and a
pericardial "knock" may be heard in early diastole.
• Pulsus paradoxus is unusual.
• Atrial fibrillation is common.
ECG
Radiographic findings
• The chest radiograph may show normal heart size
or cardiomegaly. Pericardial calcification is best
seen on the lateral view and is uncommon. It
rarely involves the LV apex and finding of
calcification at the LV apex is more consistent
with LV aneurysm.
• Cardiac CT and MRI are only occasionally helpful.
Pericardial thickening of > 4 mm must be present
to establish the diagnosis, yet no pericardial
thickening is demonstrated in 20–25% of patients
with constrictive pericarditis.
MRI fast spin echo image with blood suppression in a ventricular short-axis
plane showing circumferential thickening of the pericardium (black rim
between the epicardial and pericardial fat) (arrow).
Echocardiography
• Echocardiography rarely demonstrates a
thickened pericardium.
• A septal "bounce" reflecting the rapid early
filling is common, though.
• RV/LV interaction may be demonstrated by a
reduction in the mitral inflow pattern of >
25%, much as in tamponade.
(A) Apical four-chamber view showing pericardial effusion. (B) Modified
apical view showing a spider-web appearance formed by the fibrin
strands.
DIFFERENTIAL DIAGNOSIS
• Cor pulmonale - chronic constrictive pericarditis may be
associated with severe systemic venous hypertension but
little pulmonary congestion; the heart is usually not
enlarged and a paradoxical pulse may be present.
• Tricuspid stenosis may also simulate chronic constrictive
pericarditis; congestive hepatomegaly, splenomegaly,
ascites, and venous distention may be equally prominent,
and the manifestations of left-sided heart failure may be
inconspicuous.
• However, in tricuspid stenosis, a characteristic murmur as
well as mitral stenosis are usually present. In tricuspid
stenosis, a paradoxical pulse and a steep, deep y descent in
the jugular venous pulse do not occur, serving to
differentiate it from chronic constrictive pericarditis.
Echo
• The echocardiogram in chronic constrictive
pericarditis characteristically shows pericardial
thickening, i.e., a distinct echo posterior to the
left ventricular wall and paradoxical septal
motion.
• The left ventricular wall moves sharply outward
in early diastole on Doppler myocardial imaging.
Marked respiratory variations in atrioventricular
flow velocities on Doppler echocardiography are
also characteristic of constrictive pericarditis but
not restrictive cardiomyopathy
Effusive constrictive pericarditis
• Is a clinical hemodynamic syndrome in which
constriction of the heart by the visceral
pericardium occurs in the presence of tense
effusion in a free pericardial space.
• The hallmark is the persistence of elevated
right atrial pressure after intrapericardial
pressure has been reduced to normal levels by
removal of pericardial fluid.
Etiology
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Tubercolosis pericarditis
Hemopericardium
Viral pericarditis
Rheumatoid arthritis
Cardiac surgery
Radiation therapy
Idiopathic
Clinical features
Systemic signs of venous congestion:
-Ascities
-Hepatomegaly and raised JVP
-Dependent edema
-Anorexia and postprandial fullness.
Clinical features
• Signs of impaired ventricular filling:
-markedly raised JVP with pominent “x” and
“y” descent.
-Kussmaul’s sign.
-Friedreich’s sign: raised JVP with sharp
diastolic collapse.
-pulsus paradoxis.
-pericardial knock: early diastolic sound.
Clinical features
• Features of left sided congestion:
- dyspnea
- cough
- ortopnea
but usually are less frequent.
Essentials of Diagnosis
• Evidence of right heart failure with an elevated
JVP, edema, hepatomegaly and ascites.
• No fall or an elevation of the JVP with inspiration
(Kussmaul sign).
• Echocardiographic evidence for septal bounce
and reduced mitral inflow velocities with
inspiration.
• Catheterization evidence for RV-LV interaction, a
"square root" sign, equalization of diastolic
pressures and normal pulmonary pressure.
DIESEASES OF THE
MYOCARDIUM
Diseases of the myocardium
• CARDIOMYOPATHY
• DILATED
CARDIOMYOPATHY
(DCM)
• HYPERTROPHIC
CARDIOMYOPATHY
(HCM)
• INTERGRADE
CARDIOMYOPATHIES
• MYOCARDITIS
• INFECTIOUS CAUSES
• NON-INFECTIOUS
CAUSES
Cardiomyopathies
• The cardiomyopathies are a heterogeneous group of
entities affecting the myocardium primarily and not
associated with the major causes of cardiac disease, ie,
ischemic heart disease, hypertension, pericardial
disease, valvular disease or congenital defects.
• More recently, two additional entities have been added
to the list: a transient cardiomyopathy due to high
catecholamine discharge (Tako-Tsubo cardiomyopathy)
and an embryologic defect resulting in massive
trabeculation in the LV (ventricular noncompaction).
• Although some have specific causes, many cases are
idiopathic.
• The classification of cardiomyopathies is based on
features of presentation and pathophysiology
Dilated cardiomyopathy
• characterized by unexplained dilatation and impaired
contractile performance of the left ventricle
• Idiopathic
• Alcoholic
• Major catecholamine discharge
• Myocarditis
• Postpartum
• Doxorubicin
• Endocrinopathies
• Genetic diseases
Symptoms and Signs
• In most patients, symptoms of heart failure
develop gradually.
• The physical examination reveals rales, an
elevated JVP, cardiomegaly, S3 gallop rhythm ,
often the murmurs of functional mitral or
tricuspid regurgitation, peripheral edema or
ascites.
• In severe CHF, Cheyne-Stokes breathing, pulsus
alternans, pallor and cyanosis may be present.
ECG
• ST–T changes, conduction abnormalities,
ventricular ectopy
• Sinus tachycardia is common.
• Other common abnormalities include left
bundle branch block and ventricular or atrial
arrhythmias.
A 12-lead ECG from a young woman showing the most common
electrocardiographic abnormality found in arrhythmogenic right ventricular
cardiomyopathy, T-wave inversion in the precordial leads V1–V4.
Chest X-ray
• The chest radiograph
reveals cardiomegaly,
evidence for left and/or
right heart failure, and
pleural effusions (right >
left).
ECHO
• An echocardiogram is indicated to exclude
unsuspected valvular or other lesions and
confirm the presence of dilated cardiomyopathy
and reduced systolic function (as opposed to
diastolic heart failure).
• Mitral Doppler inflow patterns also help in the
diagnosis of associated diastolic dysfunction.
• Color flow Doppler can reveal tricuspid or mitral
regurgitation and continuous Doppler can help
define PA pressures.
Echocardiographic appearances of a young patient with familial dilated
cardiomyopathy. Panel A: parasternal long-axis view showing significant left atrial (LA)
and biventricular dilatation with a thin intraventricular septum (IVS). Panel B: apical
four-chamber view demonstrating dilatation of all four chambers. There is failure of
the tricuspid leaflets to coapt in systole (arrow). LV, left ventricle; RA, right atrium; RV,
right ventricle.
MRI
• Cardiac MRI is particularly helpful in
infiltrative processes, such as sarcoidosis or
hemochromatosis and is the diagnostic study
of choice for RV dysplasia.
• MRI can also help define an ischemic etiology
by noting gadolinium enhancement consistent
with myocardial scar.
A transverse plane spin-echo MRI in a young woman with arrhythmogenic right
ventricular cardiomyopathy demonstrating a circumscribed area of enhanced
MR signal intensity in the right ventricular (RV) free wall (arrows) due to fatty
infiltration.
Laboratory
• The serum ferritin is an adequate screening
study for hemochromatosis.
• The erythrocyte sedimentation rate may be
low due to liver congestion.
• The serum level of BNP or pro-BNP can be
used to help quantitate the severity of CHF.
Essentials of Diagnosis
• Symptoms and signs of heart failure.
• Echocardiogram confirms LV dilation, thinning
and global dysfunction.
Hypertrophic cardiomyopathy
• Myocardial hypertrophy unrelated to any pressure or
volume overload reduces LV systolic stress, increases the EF
and can result in an "empty ventricle" at end-systole.
• The interventricular septum may be disproportionately
involved (asymmetric septal hypertrophy), but in some
cases the hypertrophy is localized to mid ventricle or to the
apex.
• The LV outflow tract is often narrowed during systole
between the bulging septum and an anteriorly displaced
anterior mitral valve leaflet, causing a dynamic obstruction
.
• The obstruction is worsened by factors that increase
myocardial contractility (sympathetic stimulation, digoxin,
postextrasystolic beat) or that decrease LV filling (Valsalva
maneuver, peripheral vasodilators). The amount of
obstruction is preload and afterload dependent and can
vary from day to day.
Epidemiology
• It has been increasingly appreciated that hypertrophic
obstructive cardiomyopathy (HOCM) is inherited as an
autosomal dominant trait with variable penetrance and
is caused by mutations of one of a large number of
genes, most of which code for myosin heavy chains or
proteins regulating calcium handling.
• The prognosis is related to the specific gene mutation.
These patients usually present in early adulthood.
• Elite athletes may demonstrate considerable
hypertrophy that can be confused with HOCM, but
generally diastolic dysfunction is not present.
Transverse short axis section through the ventricles from patients with cardiomyopathy.
Upper left shows symmetrical left ventricular hypertrophy in
hypertrophic cardiomyopathy. Upper right shows dense white fibrous tissue obliterating
the apex of both ventricles in endomyocardial fibrosis. Lower left shows a
globular, dilated left ventricle in a child with dilated cardiomyopathy. Lower right shows a
grossly dilated right ventricle with adipose infiltration of the right ventricular
free wall in arrhythmogenic right ventricular dysplasia.
Symptoms
• The most frequent symptoms are dyspnea and chest
pain.
• Syncope is also common and is typically post
exertional, when diastolic filling diminishes and
outflow obstruction increases.
• Arrhythmias are an important problem.
• Atrial fibrillation is a long-term consequence of
chronically elevated LA pressures and is a poor
prognostic sign.
• Ventricular arrhythmias are also common, and sudden
death may occur, often in athletes after extraordinary
exertion.
Signs
• Features on physical examination are a bisferiens carotid
pulse, triple apical impulse (due to the prominent atrial
filling wave and early and late systolic impulses) and a loud
S4.
• The JVP may reveal a prominent a wave due to reduced RV
compliance.
• In cases with outflow obstruction, a loud systolic murmur is
present along the left sternal border that increases with
upright posture or Valsalva maneuver and decreases with
squatting .
• These maneuvers help differentiate the murmur of HOCM
from that of aortic stenosis.
• Mitral regurgitation is frequently present as well.
ECG
• LVH is nearly universal in symptomatic
patients, though entirely normal ECGs are
present in up to 25%, usually in those with
localized hypertrophy.
• Exaggerated septal Q waves inferolaterally
may suggest myocardial infarction.
Chest X-ray
• The chest radiograph is
often unimpressive.
• Unlike aortic stenosis,
the ascending aorta is
not dilated.
ECHO
• The echocardiogram is diagnostic, revealing
asymmetric LVH, systolic anterior motion of the mitral
valve, early closing followed by reopening of the aortic
valve, a small and hypercontractile LV and delayed
relaxation and filling of the LV during diastole.
• The septum is usually 1.3–1.5 times the thickness of
the posterior wall. Septal motion tends to be reduced.
• Doppler ultrasound reveals turbulent flow and a
dynamic gradient in the LV outflow tract and,
commonly, mitral regurgitation.
• Abnormalities in the diastolic filling pattern are present
in 80% of patients.
An echocardiogram (parasternal long axis view) of a patient with hypertrophic
obstructive cardiomyopathy demonstrating hypertrophy of the interventricular
septum (IVS), enlargement of the left atrium (LA), and systolic anterior motion of
the mitral valve, bringing it into contact with the septum (arrow).
• Myocardial perfusion imaging may suggest
septal ischemia in the presence of normal
coronary arteries.
• Cardiac MRI confirms the hypertrophy and
contrast enhancement frequently reveals
evidence for scar at the junction of the RV
attachment to the septum.
• Cardiac catheterization confirms the diagnosis
and assesses the presence of CAD.
• Frequently, coronary arterial bridging
(squeezing in systole) occurs, especially of the
septal arteries.
Prognosis
• The natural history of HOCM is highly variable.
• Several specific mutations are associated with a higher incidence of
early malignant arrhythmias and sudden death and definition of the
genetic abnormality provides the best estimate of prognosis.
• Some patients remain asymptomatic for many years or for life.
• Sudden death, especially during exercise, may be the initial event.
• The highest risk patients are those with a family history of sudden
death, those with marked hypertrophy and those that do not
increase their systemic BP with exercise.
• HOCM is the pathologic feature most frequently associated with
sudden death in athletes.
• Pregnancy is generally well tolerated.
• Endocarditis prophylaxis is indicated.
• A final stage may be a transition into dilated cardiomyopathy in 5–
10% of patients.
Essentials of Diagnosis
• May present with dyspnea, chest pain, syncope.
• Though LV outflow gradient is classic, symptoms are
primarily related to diastolic dysfunction.
• Echocardiogram shows septal hypertrophy, which is
usually asymmetric, and enhanced contractility.
Systolic anterior motion of the anterior mitral valve is
present if there is outflow tract obstruction.
• The highest risk group for sudden death includes those
with a marked LVH, with a family history for sudden
death, with ventricular ectopy, and with an abnormal
BP response to exercise.
Restrictive cardiomyopathy
• Restrictive cardiomyopathy is characterized by
impaired diastolic filling with reasonably
preserved contractile function.
• The LV systolic function may be mildly depressed
and the atria are generally enlarged; if present,
hypertrophy of the interatrial septum is a helpful
additional finding diagnostically.
• The condition is relatively uncommon, with the
most frequent causes being amyloidosis.
Causes
• endomyocardial fibrosis, a specific entity in
which there is severe fibrosis of the
endocardium, often with eosinophilia (Löffler
syndrome)
• infiltrative cardiomyopathies (eg,
hemochromatosis, sarcoidosis)
• connective tissue diseases (eg, scleroderma)
Symptoms and Signs
• Restrictive cardiomyopathy must be
distinguished from constrictive pericarditis.
• The key feature is that ventricular interaction
is accentuated with respiration in constrictive
pericarditis and that interaction is absent in
restrictive cardiomyopathy.
• Pulmonary pressure is invariably elevated in
restrictive cardiomyopathy and is normal in
uncomplicated constrictive pericarditis.
Diagnostic studies
• Conduction disturbances are frequently present. Low voltage on the
ECG combined with ventricular hypertrophy revealed by
echocardiography are suggestive.
• The echocardiogram reveals a small thickened LV with bright
myocardium, rapid early diastolic filling revealed by Doppler, and
biatrial enlargement. Atrial septal thickening may be evident.
• Cardiac MRI presents a distinctive pattern of hyper-enhancement of
the gadolinium image in amyloidosis and is a useful screening test.
• Rectal, abdominal fat, or gingival biopsies can confirm systemic
involvement, but myocardial involvement may still be present if
these are negative and requires biopsy for confirmation.
• Demonstration of tissue infiltration on biopsy specimens using
special stains followed by immunohistochemical studies and genetic
testing is essential to define which specific protein is involved.
Essentials of Diagnosis
• Right heart failure tends to dominate over left
heart failure.
• Pulmonary hypertension present.
• Amyloidosis is the most common cause.
• Echocardiography is key to diagnosis. Rapid early
filling is present with diastolic dysfunction.
Normal or near normal EF.
• MRI and cardiac catheterization are helpful.
Myocardial biopsy can confirm.
MIOCARDITIS
• Cardiac dysfunction due to primary
myocarditis is presumed to be caused by
either an acute viral infection or a postviral
immune response.
• Secondary myocarditis is the result of
inflammation caused by nonviral pathogens,
drugs, chemicals, physical agents or
inflammatory diseases such as systemic lupus
erythematosus.
Causes
• The list of infectious causes of myocarditis is
extensive and includes viruses with DNA and RNA
cores.
• The coxsackie virus is the predominant agent, but
many others have been implicated.
• Rickettsial myocarditis occurs with scrub typhus,
Rocky Mountain spotted fever and Q fever.
• Diphtheritic myocarditis is caused by the exotoxin
and is often manifested by conduction
abnormalities as well as heart failure.
Causes
• Myocarditis may also result from a
hypersensitivity to drugs or may be caused by
radiation, chemicals or physical agents.
• In an unknown number of cases, acute
myocarditis progresses to chronic dilated
cardiomyopathy.
Clinical findings
• The clinical manifestations range from an asymptomatic
state, with the presence of myocarditis inferred only by the
finding of transient electrocardiographic ST-T-wave
abnormalities, to a fulminant condition with arrhythmias,
heart failure and death.
• In some patients, myocarditis simulates acute myocardial
infarction, with chest pain, electrocardiographic changes
and elevated serum levels of myocardial enzymes.
• Patients with myocarditis and pulmonary hypertension are
at a particularly high risk of death.
• Clinical examination may reveal signs of cardiac failure. The
onset of heart failure may be gradual or may be abrupt and
fulminant. Pleural-pericardial chest pain is common.
Signs
• The physical examination is often normal,
although more severe cases may show a muffled
first heart sound, along with a third heart sound
and a murmur of mitral regurgitation.
• A pericardial friction rub may be audible in
patients with associated pericarditis.
• Examination may reveal tachycardia, gallop
rhythm and other evidence of heart failure or
conduction defect.
Natural history
• viral myocarditis is most often self-limited and
without sequelae
• severe involvement may recur
• it is likely that acute viral myocarditis
occasionally progresses to a chronic form and
to dilated cardiomyopathy
BACTERIAL MYOCARDITIS
• Bacterial involvement of the heart is uncommon, but
when it does occur, it is usually as a complication of
endocarditis.
• Myocardial abscess formation may involve the valve
rings and interventricular septum.
• Diphtheritic myocarditis develops in over one-quarter
of the patients with diphtheria, is one of the most
serious complications and is the most common cause
of death.
• Cardiac damage is due to the liberation of a toxin that
inhibits protein synthesis and leads to a dilated, flabby,
hypocontractile heart; the conduction system is
frequently involved as well.
Investigations
• ECG
• Echocardiography
• Chest X-ray
• CT
• MRI
• Scinti
ECG
• ECG may show sinus tachycardia, other
arrhythmias, nonspecific repolarization
changes and intraventricular conduction
abnormalities.
• Ventricular ectopy may be the initial and only
clinical finding.
High troponin in first set of lab was highly indicative of myocarditis. During initial
evaluation, the patient suddenly developed third degree heart block on the monitor
but he remained asymptomatic
This young man came to the emergency department with 12 hours of continuing anterior chest
pain. His pain was precipitated by exertion and movement. There was no significant pleuritic
component. He is not a smoker and denied history of illicit drug or cocaine use. His CPK-MB and
Troponin were both abnormal. The ECG shown above was the first one taken. At this point, he
was having 10 of 10 chest pain with minimal relief from nitroglycerin and morphine. Fearing an
Acute Myocardial Infarction (Heart Attack), the patient was taken emergently for cardiac
catheterization. Fortunately, coronary arteriography showed normal coronary arteries without
any evidence of atherosclerosis. His left ventricular function was abnormal and the left
ventricular cavity appeared slightly dilated. His pain persisted, off and on, for the next 2-3 days.
During this time, his cardiac enzymes continued to rise and the ECG abnormalities worsened.
Five days later, echocardiography showed global left ventricular dysfunction.
The patient left the hospital with a diagnosis of Pericarditis/Myocarditis. Eight weeks later, his
echocardiogram showed normal left ventricular function. His pain disappeared completely 96
hours after admission to the hospital.
Chest X-ray
• Chest radiograph is
nonspecific, but
cardiomegaly is
frequent, though not
universal.
• Evidence for pulmonary
venous hypertension is
common and frank
pulmonary edema may
be present.
Diagnostic studies
• There is no specific laboratory study that is consistently
present, though the white blood cell count is usually
elevated and the sedimentation rate may increase.
• Troponin I levels are elevated in about one-third of
patients, but CK-MB is elevated in only 10%.
• Echocardiography provides the most convenient way of
evaluating cardiac function and can exclude many other
processes.
• Gallium-67 scintigraphy may reveal increased cardiac
uptake in acute or subacute myocarditis, but it is not very
sensitive.
• MRI with gadolinium enhancement reveals spotty areas of
injury throughout the myocardium.
• Paired serum viral titers and serologic tests for other
agents may indicate the cause.
Two-dimensional parasternal long-axis view depicting disproportionate thickening,
increased echogenicity, and dyskinesis of the inferolateral wall relative to the septum;
findings are consistent with tissue oedema.
Acute myocarditis in a 35-year old male presenting with acute chest pain,
showing normal coronary arteries. Delayed enhancement MRI shows a strong
subepicardial enhancement in the anterolateral LV wall as shown in the shortaxis view (white arrows), b the spread in longitudinal direction can be well
appreciated on the vertical long-axis view (white arrows)
Endomyocardial biopsy
• Pathologic examinations may reveal a lymphocytic
inflammatory response with necrosis, but the patchy
distribution of abnormalities makes the test relatively
insensitive.
• By biopsy, the diagnosis of myocarditis has been
established by the 1986 "Dallas" criteria. The diagnosis is
dependent on describing the severity of an inflammatory
infiltrate with necrosis and degeneration of adjacent
myocytes.
• The type of infiltrate is dependent on the causal agent;
usually this is lymphocytic in viral disease, but it may be
neutrophilic, eosinophilic, giant cell, granulomatous or
mixed.
Caption: Myocarditis. Light micrograph of human cardiac muscle with chronic myocarditis
(inflammation). In this longitudinal section, muscle fibres run diagonally (purple). Abnormal
features are a loss of organisation of the muscle fibres which are normally closely spaced, with
the spaces between fibres (white, blue) representing oedema. There are neutrophils (white
blood cells, purple dots) infiltrating the oedematous areas, as an early inflammatory response
and to prevent infection. Chronic myocarditis is caused by rheumatic fever and virus infections,
with enlargement of the heart. Magnification: x50 at 35mm size.
Essentials of Diagnosis
• Often follows an upper respiratory infection.
• May present with chest pain (pleuritic or
nonspecific) or signs of heart failure.
• Echocardiogram documents cardiomegaly and
contractile dysfunction.
• Myocardial biopsy, though not sensitive, may
reveal a characteristic inflammatory pattern.