as a result of fibrous tissue with hyalinisation, and often

calcification. Vascularisation of the valve cusps may still

be evident in the form of
thick-walled blood vessels with
narrowed lumina (Fig. 16.30, B). Typical Aschoff bodies are
rarely seen in the valves at this stage.2 epothog
RHEUMATICMURALENDOCARDITIS Mural endocardium
m a y also show features of rheumatic carditis though the
changes are less conspicuous as compared to valvular
S.
changes.
Grossly, the lesions are seen most commonly as MacCallum's
patch which is the region of endocardialsuriace in the
S
posterior wallofthe left atrium just above the posterior
ed leaflet (D
of the mitral valve, MacCallum's patch appears as a
nd. map-like area ofthickened, roughened and wrinkled part of
to
he
theendocardium (see Fig. 16.28).
Microscopically, the appearance of MacCallum's patch is
the similar to that seen in rheumatic valvulitis. The affected area
res. shows oedema, fibrinoid change in the collagen, and cellular
eae infiltrate of lymphocytes, plasma cells and
B) with many Anitschkow cells.
macrophages
gion
Typical Aschoff bodies may
sometimes be föund.
ous 2. RHEUMATIC MYOCARDITIS Grossly, in the
flet- early(acute)
stage, the myocardium, especially of the left ventricle, is soft
rgin and flabby. In the intermediate stage, the interstitial tissue of
the myocardium shows small foci of necrosis. Later,
tiny pale
s are foci of the Aschoff bodies may be visible throughout the
nber myocardium.
cells, Microscopically, the most characteristic feature of rheumatic
rphs. myocarditis is the presence of distinctive Aschoff bodies.
inoid These diagnostic nodules are scattered throughout the
sare interstitial tissue of the myocardium and are most
frequent
acute in the interventricular septum, left ventricle and left atrium.
cusps Derangements of the conduction system may, thus, be
ing of present. The Aschoff bodies are best identified in the
ontain intermediate stage when they appear as granulomas with
central fibrinoid necrosis and are surrounded
by palisade
B

HD

ns on the valves of the left heart. The location of

egetationS on anrtir ale (laft lmarer dinarn vegetations on mitral valve (left upper
 CS LS
A B
- Left atrium

Fibrin-platelet thrombus
ANITSCHKOw CELL
Congestion Mitral
valve

Left
ventricle

Oedema
Mononuclear cells

Fiqure 16.30 Rheumatic heart disease. A,

section. Diagrammatic
B, Rheumatic valvular endocarditis showing valve appearance rheumatic valvulitis ànd vegetation on the
of
a

an Anitschkow cell in cross section (CS) and in

longitudinal
deformed by an elevated vegetation composed of young cusp of mitral valve in sagittal
van Damjanov 2012, Jaypee Brothers Medical section (LS). (Microimage reproducted with permission granulation tissue. Inbox shows
Publishers Pvt Ltd, New Delhi). from Atlas of Histopatholoay
by
of Anitschkow cells and multinucleate Aschoff cells. There the
is infiltration by characteristic 'migratory
lymphocytes, plasma
neutrophils (see Fig. 16.27).
cells and some
In the late stage, the Aschoff
more joints at a time. polyarthritis' involving two or
bodies are gradually replaced hy small fihrquc o Histolggicalu
 HeumatiC
cell in valvular endocarditisDiagrammatic
Anitschkow showing valveappearance of rheumatic valvulitis and a
an
cross deformed by an elevated
lvan
Damjanov 2012, Jaypeesection (CS) and in vegetation the cusp of mitral valve in
Brothers Medical longitudinal section (LS). (Microimagevegetation mposed of young
on

Publishers Pvt Ltd, reproducted with ulation tissue. Inboxsagittal

of New Delhi). permission from Atlas of shows
Anitschkow cells and Histopathology by
is
infiltration by multinucleate Aschoff cells. There the
neutrophils lymphocytes,
(see Fig. plasma cells and some more
characteristic 'migratory
joints at time. polyarthritis' involving two
bodies are 16.27). In the late a or
stage,
gradually replaced by small fibrous the Aschoff
vicinity of blood vessels scars in the Histologically, the
membrane and the changes are transitory. The synovial
subsides. Presence of active and the
inflammatory infiltrate
healed lesions is
3.
Aschoff bodies
indicative of rheumatic along with old hyperaemia, oedema,periarticular connective tissue show
fibrinoid change and
RHEUMATIC PERICARDITIS
of the
activity. infiltration. Sometimes, focal
lesions neutrophilic
Intlammatory involvement
pericardium commonly accompanies bodies are observed. A
serous resembling
effusion
Aschoff
Grossly, the usual finding RHD. is commonly present. into the joint
there is loss of normal is fibrinous pericarditis in which
cavity
deposition of fibrin on shiny pericardial surface due to
its surface and
2.
SUBCUTANEOUS NODULES The subcutaneous
slight amount of fibrinous exudate accumulation of RF occur more often
in children than in nodules of
If the in the adult. These nodules are
parietal pericardium is pulled off from pericardial sac. small (0.5 to 2 cm in
pericardium, the two separated surfaces are the visceral They are attached to diameter),
deeper
spherical
structures
or ovoid and
painless.
like
thick fibrin
covering them. This appearance isshaggy
to "bread and butter
due to
often likened
fascia or tendons,
ligaments,
periosteum and therefore often remain unnoticed
by
surfaces of two slices appearance'
i.e.
resembling the buttered
in a sandwich when Newly-formed vessels
apart. If fibrinous they gently pulled
are
pericarditis fails to resolve and, instead,
Pericardium Fibrin
undergoes organisation, the two layers of the Inflammatory cells
fibrous adhesions pericardium form
resulting in chronic adhesive
Microscopically, fibrin is identified on the pericarditis.
subserosal connective tissue is surfaces. The
infiltrated by lymphocytes,
plasma cells, histiocytes and a few
neutrophils (Fig. 16.31).
Characteristic Aschoff bodies may be seen which later
undergo organisation and fibrosis.
exudate causes
Organisation of the
fibrous adhesions between the visceral and
parietal surfaces of the
pericardial sac and obliterates the
pericardial cavity.
B. Extracardiac Lesions
Patients of the
dRyn
syndrome of acute rheumatism develop lesions
in connective tissue elsewhere in
the body, chiefly the
subcutaneous tissue, arteries, brain and lungs. joints,
1.
POLYARTHRITIS Acute and painful inflammation of
the synovial membranes of some of the Figure 16.31 Rheumatic pericarditis. The surface of pericardium shows
joints, especially fibrin and a few lymphocytes while the
the larger joints of the limbs, is seen in about 90% cases of underlying tissue shows newly-
formed vessels and chronic inflammatory cells.
RE in adults and less often in children. As
pain and swelling (Reproduced with
subside in one joint, others tend to get involved, producing permission from Atlas of Histopathology by Ivan Damjanov 2012, Jaypee
Brothers Medical Publishers Pvt Ltd, New Delhi).
the patient. Characteristic locations are extensor surfaces of the preser
has w
wrists, elbows, ankles and knees.
labora
Histologically, the subcutaneous nodules of RF are criteri
representative of giant Aschoff bodies of the heart. They Dr TD
consist of 3 distinct zones: a central area with fibrinoid and m
changes, surrounded by a zone of histiocytes and fibroblasts
infect
forming a palisade arrangement, and the outermost zone of
connective tissue which is infiltrated by non-specific chronic A. Ma
inflammatory cells and proliferating blood vessels. 1. Ca

similar but 2. PC
It may be mentioned here that histologically
3. C
clinically different subcutaneous lesions appear in rheumatoid
arthritis; they are larger, painful and tender and persist for 4. Su
Er

months to years (page 896). 5.

3. ERYTHEMA MARGINATUM This non-pruritic erythematous
rash is characteristic of RE The lesions occur mainly on the trunk B. Mi
and proximal parts of the extremities. The erythematous area 1. Fe
develops central clearing and has slightly elevated red margins. 2. Po
The erythema is transient and migratory. 3. Pr
4. RHEUMATIC ARTERITIS Arteritis in RF involves not only 4. La
the coronary arteries and the aorta but also occurs in arteries and le
of various other organs such as renal, mesenteric and cerebral 5.
arteries. The lesions in the coronaries are seen mainly in the
small intramyocardial branches. su
in pre
Histologically, the lesions may be like those of hypersensitivity
1. Po
angiitis (page 426), or sometimes may resemble polyarteritis
nodosa. Occasionally, foci of fibrinoid necrosis or ill-formed 2. R
Aschoff bodies may be present close to the vessel wall. O ane
anti-l

5. CHOREA MINOR Chorea minor or Sydenham's chorea or C

antec
Saint Vitus' dance is a delayed manifestation of RF as a result
of involvement of the central nervous system. The condition is in the
one n
characterised by disordered and involuntary jerky movements
of the trunk and the extremities accompanied by some degree If
of emotional instability. The condition occurs more often in have
younger age, particularly in girls. the h
recur

Histologically, the lesions are located in the cerebral life-tl

hemispheres, brainstem and the basal ganglia. They consist of the

of small haemorrhages, oedema and perivascular infiltration sequ

of lymphocytes. There may be endarteritis obliterans and espe
thrombosis of cortical and meningeal vessels. a stat
the h
6. RHEUMATICPNEUMONITISAND PLEURITIS Involvement repla
of the
of the lungs and pleura occurs rarely in RE. Pleuritis is often
T
accompanied with serofibrinous pleural effusion but definite
0acte
Aschoff bodies are not present. In rheumatic pneumonitis, the
1. C
lungs are large, firm and rubbery.
RHD
Histologically, the changes are oedema, capillary valvu
haemorrhages and focal areas of fibrinous exudate in the disea
alveoli. Aschoff bodies are generally not found. 2.
supe
Co cahvn h RH 3.
ACLINICAL FEATURES, DIAGNOSIS AND PROGNOSIS
thro
The first attack of acute RF generally appears 2 to 3 weeks after with
streptococcal pharyngitis, most oftern in children between the age the b
of 5 to 15 years. With subsequent streptococcal pharyngitis, there 4.
is reactivation of the disease and similar clinical manifestations in th
appear with each recurrent attack. The disease generally asso
 presents with migratory polyarthritis and fever. However, RF
465
has widespread systemic involvement and no single specific
laboratory diagnostic test is available. As per revised WHO
criteria (2004) based on revised Jones 'criteria (first described by
Dr TD Jones in 1944, and last revised in 1992), following major
and minor criteria and some supportive evidence of preceding
infection are included for diagnosis:
A. Major criteria
1. Carditis (50-60% cases)
t 2. Polyarthritis (60-75% cases)
d 3. Chorea (Sydenham's chorea) (2-30% cases)
r 4. Erythema marginatum (<5%)
5. Subcutaneous nodules (<5%)
us
ak B. Minor criteria
nk
ea 1. Fever
s. 2. Polyarthralgia

3. Previous history of RF
ly 4. Laboratory findings: elevated ESR, raised C-reactive protein,
es and leucocytosis
ral
5. ECGfinding of prolonged PR interval.
he
Suppòrtive evidence of greup A streptococcal.infection
in preceding 45 days

is 1. Positive throat culture for group A streptococci

d 2. Raised titres of streptococcal antibodies (antistreptolysin
O and S, antistreptokinase, anti-streptohyaluronidase and
anti-DNAase B).
Clinical diagnosis of RF and RHD is made in a case with
or
antecedent laboratory evidence of streptococcal throat infection
sult
in the presence of any two of the major criteria, or occurrence of
n is one major and two minor criteria.
ents
If the heart is spared in a case of acute RE the patient may
gree have complete recovery without any sequelae. However, once
n in
the heart is involved, it is often associated with reactivation and
recurrences of the disease. Myocarditis, in particular, is the most
ral life-threatening due to involvement of the conduction system
of the heart and results in serious arrhythmias. The long-term
sist
sequelae or stigmata are the chronic valvular deformities,
1on
especially the mitral stenosis, as already just explained. Initially,
and
a state of compensation occurs, while later decompensation of
the heart leads to full-blown cardiac failure. Currently, surgical
ment replacement of the damaged valves can alter the clinical course
of the disease.
often
The maior causes oth i DUD coJio folauro
 1. Fever
2. Polyarthralgia
3. Previous history of RF
4. Laboratory findings: elevated ESR, raised C-reactive protein,
and leucocytosis
5 ECG finding of prolonged PR interval.
.Suppòrtive evidence of group Astreptococcalinfection
in preceding 45 days
1. Positive throat culture for group A streptococci
2. Raised titres of streptococcal antibodies (antistreptolysin
O and S, antistreptokinase, anti-streptohyaluronidase and
anti-DNAase B).
Clinical diagnosis of RF and RHD is made in a case with
antecedent laboratory evidenceofstreptococcal throat infection
in the presence of any two ofthe major criteria, or occurrence of
one major and two minor criteria.
If the heart is spared in a case of acute RE, the patient may
have complete recovery without any sequelae. However, once
the heart is involved, it is often associated with reactivation and
recurrences of the disease. Myocarditis, in particular, is the most
life-threatening due to involvement of the conduction system
of the heart and results in serious arrhythmias. The long-term
sequelae or stigmata are the chronic valvular deformities,
especially the mitral stenosis, as already just explained. Initially,
a state of compensation occurs, while later decompensation of
the heart leads to full-blown cardiac failure. Currently, surgical
replacement of the damaged valves can alter the clinical course
of the disease.
The major causes of death in RHD are cardiac failure,
bacterial endocarditis and embolism:
1. Cardiac failure is the most common cause of death from
RHD. In young patients, cardiac failure occurs due to the chronic
valvular deformities, while in older patients coronary artery
disease may be superimposed on the old RHD.
2. Bacterial endocarditis of both acute and subacute type may
antibiotics.
supervene due to inadequate use of
from mural
3. Embolism in RHD originates most commonly
in association
thrombi in the left atrium and its appendages,
affected are
with mitral stenosis. The organs most frequently
the brain, kidneys, spleen and lungs.
thrombus
4. Sudden death may occur in RHD as a result ofball
in the left atrium or due to acute coronary insufticiency
in
association with aortic stenosis.
 Rheumatic Fever and Rheumatic
MUST-KNOW
Heart Disease
Rheumatic fever is a systemic, post-streptococcal, non-

suppurative inflammatory disease, principally affecting the

heart, joints, central nervous system, skin and subcutaneous
tissues.
Chronic stage of RF involves all the layers of the heart
(pancarditis) causing major cardiac sequelae referred to as
rheumatic heart disease (RHD).
Thedisease is more common in children between the age
of 5 to 15 years.
There are 3 groups offactors in the etiology and
pathogenesis
of RF and RHD: environmental factors,
hostsusceptibility and
immunologic evidences.
Pathognomonic feature of pancarditisin RF is the presence
of distinctive Aschoff nodules or Aschoff
bodies.
Chronic stage of RHD is characterised
by permanent
deformity one or more valves, especially the mitral (alone
of
or with other
valves) and aortic.
Patients of RF-RHD may
develop extra-cardiac lesions in
connective tissue elsewhere in the
subcutaneous tissue, arteries, brainbody,
and
chiefly the joints,
Diagnosis of RHD is made lungs.
revised Jones criteria by WHO criteria based on
minor clinical and consisting
of some
major and some
laboratory features; major criteria are
carditis, polyarthritis,
subcutaneous nodules.chorea, erythema marginatum and

NON-RHEUMATIC ENDOCARDITIS
Inflammatory involvemen
 NON-RHEUMATIC ENDOCARDITIS
Intlammatory involvement of the endocardial layer of the
heart is called endocarditis. Though in common usage, if
not specified endocarditis would mean inflammation of the
valvular endocardium, several
workers designate endocarditis
on the basis of anatomic area of
the involved endocardium such
as: valvular for
valvular
the lumina of cardiac endocardium, mural for inner lining of
of the chordae
chambers, chordal for the endocardium
of trabeculae
tendineae, trabecular for the endocardium
carneae, and papillary for the
covering the papillary muscles. Endocarditis endocardium
can be
grouped into non-infective and broadly
Most types of infective types (Table 16.6).
endocarditis are characterised
of
'vegetations'or by the presence
'verrucae' which have distinct
features.
ATYPICAL VERRUCOUS (LIBMAN-SACKS)
ENDOCARDITIS
Libman and Sacks, two
American physicians,
endocarditis in 1924 that is characterised described a form of
by sterile endocardial
Table 16.6 Classification of endocarditis.
A NON-INFECTIVE
1. Rheumatic
endocarditis (page 462)
2. Atypical verrucous (Libman-Sacks) endocarditis
3. Non-bacterial thrombotic (cachectic, marantic)
endocarditis
B. INFECTIVE

1. Bacterial endocarditis
2. Other infective types (tuberculous, syphilitic, fungal, viral,
rickettsial)
 vegetations which are distinguishable from the vegetations of
eumatic
RHD and bacterial endocarditis.
ETIOPATHOGENESIS Atypical verrucous endocarditis is one of
ococcal, non- the manifestations of 'collagen diseases. Characteristic lesions
yaffecting the of Libman-Sacks endocarditis are seen in 50% cases of acute
subcutaneous systemic lupus erythematosus (SLE); other diseases
associated
with this form of endocarditis are systemic sclerosis, thrombotic
ofthe heart thrombocytopenic purpura (TTP) and other collagen diseases
referred to as
MORPHOLOGIC FEATURES Grossly, characteristic
ween the age the mitral and tricuspid
vegetations occur most frequently on
endocarditis are
valves. The vegetations of atypical verrucous
ipathogenesis and tend to
small (1 to 4 mm in diameter), granular, multiple
sceptibilityand occur on both surfaces of
affected valves, in the valve pockets
ventricular and atrial endocardium.
and on the adjoining
s the presence Ihe vegetations are sterile unless superimposed by bacterial
dies. endocarditis. Unlike vegetations ofRHD, the healed vegetations
y permanent of Libman-Sacks endocarditis do not produce any significant
or serofibrinous
e mitral (alone valvular deformity. Frequently, fibrinous
effusion is associated.
pericarditis with pericardial endocarditis
diac lesions in verrucae of Libman-Sacks
Microscopically, the
material with superimposed
iefly the joints, are composed of fibrinoid
fibrin and platelet thrombi. The endocardium underlying
gs.
eria based on the verrucae shows characteristic histological changes
fibrinoid necrosis, proliferation of capillaries
ajor and some which include
jor criteria are and infiltration by histiocytes, plasma cells, lymphocytes,
bodies
arginatum and neutrophils and the pathognomonic haematoxylin blood.
of LE cells of the
of Gross which are counterparts found in the interstitial S M

Similar inflammatory changes may be

tissue of the myocardium. The Aschoff
bodies are
connective
never found in the endocardium or myocardium.
DITIS
ial layer of the
nmon usage, if NON-BACTERIAL THROMBOTIC(CACHECTIC
mmation of the
MARANTIC) ENDOCARDITIS
ateendocarditis marantic or terminal
docardium such Non-bacterial thrombotic, cachectic,
is an involvement of the
endocarditis or endocarditis simplex
rinner lining of
heart valves by sterile thrombotic vegetations.
e endocardium
ETIOPATHOGENESIS The exact pathogenesis oflesions in non-
endocardium
bacterial thrombotic endocarditis (NBTE) is not clear. Vegetations
endocardium
diseases and
can be broadly are found at autopsy in
0.5 to 5% of cases. Following
conditions are frequently associated with their presence
-s (Table 16.6).
state from various
the presence 1. In patients having hypercoagulable
y 50% case of NBTE) especially
ct features. etiologies e.g. advanced cancer (in
chronic tuberculosis, renal failure
mucinous adenocarcinomas,
of its association with chronic
and chronic sepsis. In view
alternate names for NBTE
debilitating and wasting diseases, endocarditis are
'marantic' and 'terminal'
such as 'cachectic,
cribed a form of
used synonymously.
ileendocardial well-nourished
lesions in young and
2. Occurrence of these
on the basis of alternative hypothesis such
patients is explained thrombosis, and
as allergy, vitamin
C deficiency, deep vein
endocardial trauma due to catheter in pulmonary artery
(e.g.
trauma to the valves).
and haemodynamic
tis MORPHOLOGICFEATURES Grossly, the verrucae of NBTE
mitral, and less often
located on cardiac valves, chiefly
) endocarditis
are small
valve. These verrucae are usually
aortic and tricuspid brownish and
single or multiple,
(1 to 5 mm in diameter), the leaflets but are more

occur along
the line of closure of healed
of RHD. Organised and
fungal,viral, friable than the vegetations
 vegetations appear as fibrous nodules. Normal age-related
appearance of tag-like appendage at the margin of the valve
cusps known as 'Lambl's excrescences' is an example of such
healed lesions.
Microscopically, the vegetations in NBTE are composed
of fibrin along with entangled RBCs, WBCs and platelets.
Vegetations in NBTE are sterile, bland and do not cause tissue
destruction. The underlying valve shows swollen collagen,
fibrinoid change and capillary proliferation but does not show
any inflanmmatory infiltrate.

Embolic phenomenon is seen in many cases of NBTE and

results in infarcts in the brain, lungs, spleen and kidneys. The
bland vegetations of NBTE on infection may produce infective
endocarditis.

INFECTIVE(BACTERIAL) ENDOCARDITIS
DEFINITION Infective or bacterial endocarditis (IE or BE) is
serious infection of the valvular and mural endocardium caused
by different forms of microorganisms and is characterised by
typical infected and friable vegetations..A few specific forms
of IE are named by the microbial etiologic agent causing them
e.g. tubercle bacilli, fungi etc. Depending upon the severity of
infection, BE is subdivided into 2 clinical forms:
1. Acute bacterial endocarditis (ABE) is fulminant and
destructive acute infection of the endocardium by highly virulent
bacteria in a previously normal heart and almost invariably runs
a rapidly fatal course in a period of 2-6 weeks.
SM

2. Subacute bacterial endocarditis (SABE) or endocarditis

lenta (lenta = slow) is caused by less virulent bacteria in a
previously diseased heart and has a gradual downhill course in
a period of 6 weeks to a few months and sometimes years.
Although classification of bacterial endocarditis into acute
and subacute forms has been largely discarded because the
clinical course is altered by antibiotic treatment, still a few
important distinguishing features are worth noting (Table l6.7).
However, features of the vegetations in the two forms of BE are
difficult to distinguish.
INCIDENCE Introduction of antibiotic drugs has helped greatly
in lowering the incidence of BË as compared with its incidence
in the pre-antibiotic era. Though BE may occur at any age, most
cases of ABE as well as SABE occur over 50 years of age. Males
are affected more often than females.

Table 16.7 Distinguishing features of acute and subacute

bacterial endocarditis.
 Although classification of bacterial endocarditis into acute
and subacute forms has been largely discarded because the
elinical course is altered by antibiotic treatment, still a few
important distinguishing features are worth noting (Table 16.7).
However, features of the vegetations in the two forms of BE are
difficult to distinguish.
INCIDENCE Introduction of antibiotic drugs has helped greatly
in lowering the incidence of BE as compared with its incidence
in the pre-antibiotic era. Though BE may occur at any age, most
cases of ABE as well as SABE occur over 50 years of age. Males
are affected more often than females.
Table 16.7 Distinguishing features of acute and subacute
bacterial endocarditis.
FEATURE ACUTE SUBACUTE
1. Duration <6 weeks >6 weeks

2 Most common Staph. aureus, Streptococcus

organisns B-streptococci viridians
3 Virulence of Highly virulent Less virulent
organisms
4. Previous condition Usually previously Usually previously
of valves normal damaged
5. Lesion on valves Invasive Usually not invasive
destructive, or suppurative
suppurative
6. Clinical features Features of acute Splenomegaly,
systemic infection clubbing of fingers,
petechiae
 ETIOLOGY All cases of BE are caused by infection with
microorganisms in patients having certain predisposingfactors.
467
A. Infective agents About 90% cases of BE are caused by
streptococci and staphylococci.
I n ABE, the most common causative organisms are
virulent strains of staphylococci, chiefly Staphylococcus
aureus. Others are pneumococci, gonococci, B-streptococci
and enterococci.
I n SABE, the commonest causative organisms are the
streptococci with low virulence, predominantly Streptococcus
viridans, which forms part of normal flora of the mouth and
pharynx. Other less common etiologic agents include other
strains of streptococci and staphylococci (e.g. Streptococcus
bovis which is the normal inhabitant of gastrointestinal tract,
Streptococcus pneumoniae, and Staphylococcus epidermidis
which is a commensal of the skin), gram-negative enteric
bacilli (e.g. E. coli, Klebsiella, Pseudomonas and Salmonella),
pneumococci, gonococci and Haemophilus influenzae.
B. Predisposing factors There are 3 main types offtactors which
predispose to the development of both forms of BE:
Conditions initiatingtransient bacteraemia, septicaemia
and pyaemia.
Underlying heart disease.
f
Impaired host defenses.
1. Bacteraemia, septicaemia and pyaemia Bacteria gain

entry to the blood stream causing transient and clinically silent

bacteraemia in a variety of day-to-day procedures as well as from
S other sources of infection. Some of the common examples are:
i) Periodontal infections such as trauma from vigorous
brushing of teeth, hard chewing, tooth extraction and er
adental procedures. in catheterisation,
"ii) Infections ofthe genitourinary tract such as
cystoscopy, obstetrical procedures including normal delivery
and abortions.

ii) Infections of gastrointestinal and biliary

tract.

and genitourinary tracts.

iv) Surgery of the bowel, biliary tract abscesses.
carbuncles and
e v)Skin infections such as boils,
tract infections including
vi) Upper and lower respiratory
bacterial pneumonias.
e vii) Intravenous drug abuse.
cardiac surgery for implantation
st vii) Cardiac catheterisation and
es of prosthetic valves.
2. Underlying heart disease SABE occurs much more frequently
whereas the ABE is common
in previously diseased heart valves,
the commonly associated
in previously normal heart. Amongst
the following:
underlying heart diseases are
valvular disease in about 50% cases.
i) Chronic rheumatic
in about 20% cases. These include
ii) Congenital heart diseases aortic
stenosis, bicuspid
VSD, subaortic stenosis, pulmonary
and PDA.
valve, coarctation of the aorta,
aortic valve disease, atherosclerotic
ii) Othercauses syphilitic
are

and prosthetic heart valves.

valvular disease, floppy mitral valve,
is
All conditions in which there
3. Impaired host defenses and
of complement
depression of specilic immunity, deficiency BE. Following are
to
defective phagocyticfunction, predispose
conditions:
some of the examples of such
i) Impaired specific immunity in lymphomas.
ii) Leukaemias.
forms of cancers and transplant
ii) Cytotoxic therapy for various
patients.
and macrophages
iv) Deficient functions of neutrophils
468 PATHOGENESIS Bacteria causing BE on entering the blood found o n previously normal
stream from any of the above-mentioned routes are implanted vegetations of ABE are often
are often located on the
on the cardiac valves or mural endocardium because they have valves. Like in RHD, the vegetations
and ventricular surface
surface adhesion molecules which mediate their adherence to atrial surface of atrioventricularvalves
from the contact areas of
injured endocardium. There are several predisposing conditions ofthe semilunar valves. They begin the valves and
which explain the development of bacterial implants on the valves: the valve and may extend along the surface of
16.32).
1. The circulating bacteria are lodged much more frequently on to the adjacent endocardium (Fig.
millimeters
on previously damaged valves from diseases, chiefly RHD, The vegetations of BE vary in size from a few
congenital heart diseases and prosthetic valves, than on healthy to several centimeters, grey-tawny to greenish, irregular,

valves. single or multiple, and typically friable. They may appear

2. Conditions flat, filiform, fungating or polypoid. The vegetations in ABE
producing haemodynamic stress on the valves tend to be bulkier and globular than those of SABE and are
are liable to cause damage to the endothelium, favouring the
formation of platelet-fibrin thrombi which get infected from located more often on previously normal valves, may cause
circulating bacteria. ulceration or perforation of the underlying valve leaflet, or
3. Another alternative hypothesis is the occurrence of non- may produce myocardial abscesses.
bacterial thrombotic endocarditis from prolonged stress which Microscopically, the vegetations of BE consist of 3 zones
is followed by bacterial contamination. (Fig. 16.33):
i) The outer layer or cap consists of eosinophilic material
MORPHOLOGIC FEATURES The characteristic pathologic composed of fibrin and platelets.
feature in both ABE and SABE is the presence of typical ii) Underneath this layer is the basophilic zone containing
or leaflets, and
vegetations or v e r r u c a e on the valve cusps colonies of bacteria. However, bacterial component of the
distinct
less often, on mural endocardium, which are quite vegetations may be lacking in treated cases.
forother types. A summary of the distinguishing features of ii) The deeper zone consists of non-specific inflammatory
Table 16.8.
the principal types ofvegetations is presented in the valves reaction in the cusp itself, and in the case of SABE there may
Grossly, the lesions are found commonly
on
be evidence of repair.
of the left heart, most frequently on the mitral, followed
simultaneous
in descending frequency, by the aortic, In the acute fulminant form of the disease, the
involvement of both mitral and aortic valves,
and quite rarely
inflammatory cell intiltrate chiefly consists of neutrophils and
the right heart. The vegetations in SABE are
on the valves of diseased valves, whereas the
is accompanied with tissue necrosis and
abscesses in the valve
more often s e e n on previously rings and in the myocardium. In the subacute form, there is
 ).0, the case
Grossly, the lesions are found reaction in thecusp itself, and in ol.
of the left heart, most commonly on the valves be evidence of repair.
in descending
frequently on the mitral, followed
frequency, by the aortic, simultaneous In the acute fulminant form
of the disease, the
involvement of both mitral and aortic valves, and quite
the valves of the right heart. The
on
rarely inflammatory cell infiltrate chiefly
consists of neutrophils and
vegetations in SABE are IS accompanied with tissue necrosis
and abscesses in the valve
more often seen on
previously diseased valves, whereas the subacute form, thereis
rings and in the myocardium. In the
Table 16.8 Distinguishing features of vegetations in major forms of endocarditis.
FEATURE RHEUMATIC LIBMAN-SACKS NON-BACTERIAL BACTERIAL (INFECTIVE)
THROMBOTIC
1. Mitral; aortic; combined
Valves commonly Mitral alone; mitral and Mitral, tricuspid Mainly mitral; less often
affected aortic combined aortic and tricuspid mitral and aortic

2. Location on valve Occur along the line of Occur on both surfaces of Occur along the line of SABE more often on
cusps or leaflets closure, atrial surface of valve leaflets or cusps, in closure diseased valves: ABE on

atrioventricular valves the valve pockets previously normal valves;

and ventricular surface of location same as in RHD

semilunar valves
3 Gross appearance

Smal, multiple, warty,
grey brown, translucent,
firmly attached, generally
produce permanent
valvular deformity
A. MicroscopPy i) Composed of fibrin with
superimposed platelet
thrombi and no bacteria
ii) Adjacent and
underlying endocardium
shows oedema,
proliferation of capillaries,
mononuclear inflammatory
infiltrate and occasional
Aschoff bodies.
subacute bacterial
a c u t e
hacterial endocarditis; SABE,
r
Medium-sized, multiple,
generally do not produce
significant valvular
deformity
i) Composed of
fibrinoid material with
superimposed fibrin and
platelet thrombi and no
bacteria.
ii) The underlying
endocardium shows
fhbrinoid necrosis,
proliferation of capillaries
and acute and chronic
inflammatory infiltrate
including the haematoxylin
bodies of Gross.
endocarditis; RHD, rheumatic heart disease).
Small but larger than those
of rheumatic, single or
multiple, brownish, firm,
but more friable than those
of rheumatic
i) Composed of
degenerated valvular
tissue, fibrin platelets
thrombi and no bacteria.
ii) The underlying
valve shows swelling of
collagen, fibrinoid change,
proliferation of capillaries
but no significant
inflammatory cell infiltrate.
Often large, grey-tawny to
greenish, iregular, single
or multiple, typically friable
i) Composed of outer
eosinophilic zone of fibrin
and platelets, covering
colonies of bacteria and
deeper zone of non-
speciñc acute and chronic
inflammatory cells.
ii) The underlying
endocardium may show
abscesses in ABE and
inflammatory granulation
tissue in the SABE.