Diseases of the Heart - 3

Dr. Geener .K .John

Recommended reading –
Pages 425- 429 from
Robbins – Basic Pathology 10th edition
SLO# 1 : Discuss the pathogenesis, clinical features, diagnostic
criteria and morphological changes in acute rheumatic heart
disease.
SLO# 2 : Describe the changes seen in chronic rheumatic heart
disease.
SLO# 3 : Discuss the aetiology, predisposing factors, clinical
features, morphological changes and complications in
infective endocarditis.
SLO# 4 : Discuss the etiological factors and the morphological
features of Nonbacterial thrombotic endocarditis.
SLO# 5 : Discuss the etiological factors and the morphological
features of Libman-Sacks endocarditis.
 Rheumatic valvular disease
Rheumatic fever (RF) is an acute immunologically
mediated multisystem inflammatory disorder
following streptococcal pharyngitis
Cardiac manifestations are called rheumatic heart
disease (RHD)
A/c RF appear in children between 5-15 years
Incidence ↓sed due to improved socioeconomic
conditions & use of antibiotics
 What type of hypersensitivity is acute rheumatic fever?
Type II hypersensitive (autoantibodies generated via M-protein of strep pyogenes molecular mimicry)

Pathogenesis
A/c RF is a hypersensitivity reaction by Abs.
elicited by Group- A β hemolytic streptococci
Molecular mimicry – Abs. produced against
streptococcal M-protein cross react with
glycoprotein antigens in the heart, joints etc.
Cross reacting antibodies
There is a delay of 2-3 weeks after pharyngitis
Fibrosis during healing of A/c inflammation result
in valvular defects
Pathogenesis of A/c RHD
 Diagnosis
Diagnosis is clinical – based on Jones criteria
"JONES"
Major J = migratory polyarthritis (involving wrist, ankle, knees)
O = pancarditis
Carditis N = Nodes
E = erythema marginatum
S = Syndeham's chorea
Migratory poly arthritis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
What valve is always affected in acute rheumatic fever?
Mitral valve (aortic can be affected but MITRAL will always be affected)
 Diagnosis- Contd.
Minor Nonspecific: Fever, elevated ESR
Clinical-Fever, Arthralgia
Lab- Elevated A/c phase reactants like
Raised ESR & C – reactive proteins
2 major or 1 major + 2 minor
with serological evidence to support recent
streptococcal infection –Positive throat culture
Raised ASO titer & anti DNAse B
Labs to prove group A (beta-hemolytic) strep infection
 Clinical features
Predominant clinical manifestations are arthritis &
carditis
Arthritis – seen in 75 % of A/c RF
One major joint followed by another with pain &
swelling
Carditis – seen in 40-60 %
Pan carditis producing pericardial friction rubs &
arrhythmias. Even myocardial dilatation & CHF
are produced
 Morphology of cardiac lesions
In acute stage pancarditis affecting all the 3
layers inflammation of all three layers of the heart.
Focal inflammatory lesions known as Aschoff
bodies are pathognomonic Text of RF, which can
be seen in all the 3 layers
Endocardium – valvulitis & mural endocarditis
Myocardium – carditis with Aschoff bodies
Pericardium – shows fibrinous pericarditis
Most common cause of death in acute rheumatic fever
Myocarditis
 Aschoff body
Central zone of degenerating hyper - eosinophilic
matrix infiltrated by lymphocytes, plasma cells
& activated macrophages – Anitschkow cells
These cells have abundant cytoplasm central
nuclei with wavy ribbon like chromatin
“caterpillar cells”
 Endocarditis
Fibrinoid necrosis along the line of closure forming
small (1-2 mm) vegetations (verrucae)
All 4 valves can be involved.
Mitral most common followed
by aortic. Valves of Rt. Side
vegetations
are rarely involved
Rough wrinkled area in the posterior wall of Lt.
atrium on endocardial surface Mc callum’s patch
Pericarditis
Fibrinous or serofibrinous
exudate in the pericardial sac
resolve without sequelae
Described as
“bread & butter appearance”
Repeat exposure to group A beta-hemolytic strep that causes repeated acute rheumatic fever

Chronic RHD
Organization of acute inflammation &
subsequent fibrosis result in permanent
deformity of the valves especially mitral &
aortic.
Text
Mitral alone in 70%
Mitral & aortic 25%
Healed mitral valve is
described as “ fish mouth” or “ buttonhole”
1- Valvular stenosis
stenosis 2- Fish-mouth
appearance
Infective endocarditis
One of the most serious infections characterized by
microbial invasion of heart valves & mural
endocardium, resulting in bulky friable vegetations
associated with destruction of underlying cardiac
tissue
Causes –
Extra cellular bacteria, fungi, rickettsiae,
chlamydiae
Infection occurs when organisms are implanted on
the endocardial surface during episodes of
bacteremia. secondary to bacteremia.
A/c Vs Sub A/c endocarditis
Acute endocarditis Sub acute endocarditis
Severe destructive form Insidious onset
Highly virulent organism Organism of low virulence
Usually attacks a deformed
Attacks previously valve
normal valve Protracted course, most
Death within weeks in patients recover with
> 50% cases antibiotic therapy

Subacute = small vegetations that do not destroy valve

Acute = massive vegetations that destroy the valve

Etiology
Previously damaged or deformed valves most
commonly (50-60%) infected by
Streptococcus viridans  sub A/c course
Healthy or deformed valves (10-20%) by
virulent organism like Staph.aureus A/c
course. This is the major organism in I/V drug
abusers
Rarely other organisms like Haemophilus and
Enterococci are also involved
 Predisposing factors
Source of bacteremia – dental or surgical procedures
 transient bacteremia, occult infection in GIT,
urinary tract or oral cavity,
injection of contaminated material
in Streptococcus viridans
Underlying heart disease – C/c RHD, VSD, PDA,
bicuspid aortic valve, mitral valve prolapse,
prosthetic heart valves
Impaired host defense – neutropenia, DM,
immunodeficiency, malignancy, alcoholism
 vegetations: thrombus attached to the valve

Morphology
In both A/c & sub A/c form- destructive, bulky,
friable vegetations are seen on heart valves.
Vegetation is a mass of platelets, fibrin,
microorganism & inflammatory cells
septic thrombus/emboli

Vegetations may be
small or large
single or multiple
involves one or more valves
Morphology - Contd.
Valvular involvement most active

Aortic & mitral valves are most common

In I/V drug abusers valves of Rt. side mainly
tricuspid valve is involved
Vegetations can erode into
myocardium producing –
ring abscess
 "FROM JANE"
F = fever
R = Roth spots (retinal hemorrhages from bacterial emboli)
Clinical features O = Osler nodes (painful nodes on finger pads from emboli)
M = murmur (vegetations on heart valve)
Fever is the most consistent sign.
J = Janeway lesions (painless red lesions on hands/feet
A = anemia (of chronic disease)
N = Nail hemorrhages (splinter nail)
Murmurs are present in 90% cases
E = Emboli to brain (stroke) to kidney (renal failure) etc.

A/c IE – rapidly developing fever, chills, rigor &

weakness
Sub A/c IE – fever may be absent, nonspecific
fatigue & weight loss
Diagnosis based on blood culture, ECG findings,
clinical & laboratory findings
Clinical findings due to microemboli – petechiae,
bleeding under nail pit
splinter hemorrhage, Roth spots, Janeway
lesions, Osler nodes.
Complications
immunological
GN due to trapping of antigen Ab. complexes
 hematuria, albuminuria & renal failure
Septicemia
non- immunological
Arrhythmias
Septic emboli – vegetations are friable, tends to
dislodge easily & contain large no. of
organism  mycotic aneurysms
From left side of heart  septic infarcts /
abscess in spleen, kidney & brain. From right
side  in lungs
Nonbacterial thrombotic endocarditis
Deposition of small masses of fibrin, platelets
etc. on the heart valves
They are sterile no microorganism
Seen in debilitated individuals – marantic
causes:
endocarditis Malignancy
Hypercoagulability state
Underlying mechanism is hypercoagulable
states – DIC, APML, Mucinous
adenocarcinoma
Marantic endocarditis is characterized by the presence of sterile vegetations
in the heart valves, and is associated with hypercoagulability states (cancer,
autoimmune diseases, HIV).
 Morphology
Sterile, nondestructive, small vegetations seen
along the line of closure of the leaflets or
cusps
Clinical significance
Local effect on the valve is trivial
Can produce emboli to
brain, heart etc.  infarcts
Its main complications are stroke, pulmonary
thromboembolism, acute intestinal ischemia and
splenic, renal and hepatic infarcts.
 Libman – Sacks endocarditis
Seen in patients with SLE
Occurs due to immune complex deposition &
associated inflammation
Small granular pink friable vegetations
No predilection for line
of valve closure
Cause of vegetations on both sides of mitral valve

Mitral valve —> most common

 Comparison of vegetations
Pulmonary embolism is caused by
endocarditis of what valve?
Tricuspid = because bacterial
vegetation embolizes to lungs

Acute rheumatic fever = mitral

regurgitation (tiny vegetations)
Chronic rheumatic fever = mitral
stenosis