Download RHEUMATIC HEART DISEASE

RHEUMATIC HEART
DISEASE
HA MWAKYOMA, MD
Introduction
• Rheumatic heart disease is the most
serious manifestation of acute rheumatic
fever and is the end result of carditis, which
affects 30% to 45% of patients with acute
rheumatic fever.
Introduction—cont-• Damage to the cardiac valves may
be chronic and progressive and
can lead to congestive heart
failure and death
• Pockets of resurgence of
rheumatic fever have occurred all
over the world in recent years
Epidemiology
• Acute rheumatic fever is a disease of the
young, occurring most commonly in
preadolescent children
• It is much rarer in children younger than 5
years and adults older than 35 years
Epidemiology—cont-• Recurrent episodes occur through
adolescence and into early adulthood, and
it is thought that the cumulative effect of
recurrent episodes of acute rheumatic fever
leads to the development of rheumatic heart
disease
• Both acute rheumatic fever and rheumatic
heart disease are more common in females
Pathogenesis
• Incompletely understood;
• Although streptococci have not been found
in the heart tissues of patients with acute
rheumatic fever, there is strong
circumstantial evidence that acute
rheumatic fever is the result of an
exaggerrated immune response to
pharyngeal infection with group A
streptococcus.
Pathogenesis-cont-• Outbreaks of acute rheumatic fever closely
follow epidemics of streptococcal
pharyngitis or scarlet fever, and adequate
treatment of documented pharyngeal
streptococcal infection clearly decreases
the incidence of subsequent rheumatic
fever.
Pathogenesis-cont-• Appropriate prophylaxis with antibiotics
can prevent recurrence in patients with
prior episodes of acute rheumatic fever
Pathogenesis—cont-• Most patients with acute rheumatic fever
also have elevated titers to one or more of
the three antistreptococcal antibodies
(streptolysin O, hyaluronidase, and
streptokinase).
• Group A streptococcal infection of the
pharynx is a necessary element for the
subsequent development of acute
rheumatic fever
Heart -Pathology
Valvular Heart Diseases
• Rheumatic Heart Disease (RHD)
• Rheumatic Fever (RF)
– Acute, immune-mediated multi-system
disease occurring a few weeks after an
episode of group A streptococcal
pharyngitis
– MC in children 5-15 yrs old
– RF  RHD (deforming fibrotic valvular disease)
– After first attack -  susceptibility to reactivation of RF with
subsequent pharyngitis
Heart -Pathology
Rheumatic Heart Diseases
Etiopathogenesis
– Hypersensitivity ( immunological) reaction to “M”
protein of Group A streptococci
– RF develops weeks after Streptococcal pharyngitis
– Antibodies against “M” protein cross react with
• Cardiolipins of Heart Rheumatic disease
• GBM of Kidney  Post – streptococcal
Glomerulonephritis (PIGN/PSGN/ Acute nephritic
syndrome)
– Morphology
• Acute (RF) Aschoff bodies (Myxoid degerantion
with surrounding inflammation), Pancarditis,
Anitschkow cells (macrophages with caterpillar like
nucleus)
• Chronic (RHD) fibrosis of valve cusps, fusion of
commisures,
Heart -Pathology
Rheumatic Heart Diseases
Clinical Features
• Onset of acute rheumatic fever is typically
characterized by an acute febrile illness 2 to 4
weeks after an episode of pharyngitis.
• Diagnosis is primarily clinical and is based on
a constellation of signs and symptoms, which
were initially established as the Jones criteria in
1944:
Jones Criteria (1992 Revision) for Diagnosis of
Acute Rheumatic Fever
• Major manifestations
1.
2.
3.
4.
5.
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Jones Criteria (1992 Revision) for Diagnosis
of Acute Rheumatic Fever– cont-Minor manifestations
1. Fever
2. Arthralgias
3. Previous rheumatic fever or rheumatic heart disease
4. Increased C- reactive protein concentrations or ESR
5. Prolonged PR interval on ECG
Evidence of antecedent group A
streptococcal infection
1. Positive throat culture or rapid antigen test positive for
group A streptococcus
2. Increased or increasing streptococcal antibody titer
The Jones Criteria for Rheumatic Fever,
Updated 1992
Major Criteria
Minor Criteria
Carditis
Clinical
Migratory polyarthritis
Fever
Sydenham's chorea
Arthralgia
Subcutaneous nodules
Erythema marginatum
Laboratory
Elevated acute phase reactants
Prolonged PR interval
plus
Supporting evidence of a recent group A streptococcal infection (e.g., positive throat
culture or rapid antigen detection test; and/ or elevated or increasing streptococcal
antibody test)
Jones Criteria (1992 Revision) for Diagnosis of
Acute Rheumatic Fever
• A firm diagnosis requires
1) 2 major manifestations or 1 major and 2 minor
manifestations
and
2 ) Evidence of a recent streptococcal infection.
However, when chorea or carditis is clearly
present, evidence of an antecedent group A
streptococcal infection is not necessary.
Heart -Pathology
Rheumatic Heart Diseases
– Types
Feature
Rheumatic Fever (RF) Rheumatic Heart Disease
(RHD
Onset
Acute
Chronic
Age
Children ( 5 – 15 yrs)
Adults
Pathology
Carditis, arthritis,
chorea
Valvular disease ( MS, MR)
Aschoff bodies
Pathognomonic
Not seen
Diagnosis
John’s criteria
Not applicable
Clinical Features
• Although the clinical usefulness of the Jones
criteria has been recently reaffirmed, the
main features have been modified or
updated several times in order to increase
the specificity of the criteria.
• The World Health Association (WHO) has
more recently developed criteria that favor
sensitivity over specificity; they may be the
preferred guidelines in countries with
populations at high risk for acute rheumatic
fever
World Health Association for Diagnosis of
Acute Rheumatic Fever
1. First episode- same as the Jones criteria
2. Recurrent episode in a patient without
established RHD- same as for first episode
3. Recurrent episode in a patient with established
RHD- requires 2 minor Jones criteria
manifestations and evidence of an antecedent
gropu A streptococcus infection
Clinical Features
• Beyond fever, arthritis is typically the earliest
manifestation of acute rheumatic fever. In
untreated patients, the arthritis is classically
described as “migrating” from joint to joint in
quick succession.
• The knees and ankles are often the first
affected.
• The duration of joint inflammation is short (≤ 1
week), and the synovial fluid is generally sterile
when examined.
Clinical Features
• Chorea, also known as Sydenham chorea
or St. Vitus dance,
• is a neurologic movement disorder
characterized by abrupt, purposeless
involuntary movements of the muscles of
the face, neck, trunk, and limbs.
• Muscular weakness (hypotonia) and
behavioral disturbances such as
obsessive- compulsive behaviors are
considered to be additional findings of
chorea.
• .
Chorea-- Clinical Features—cont-• The course of the syndrome is variable.
Symptoms tend to develop subtly,
progressively worsen over 1 to 2 months,
and spontaneously resolve gradually after
3 to 6 months. Residual waxing and
waning of symptoms may occur for a year
or more, and 20% of patients have
recurrences within 2 years
Clinical Features
Two classic skin lesions with welldescribed identifying characteristics
occur in acute rheumatic fever:
• Subcutaneous nodules ranging
from several millimeters to 2 cm occur for
approximately 2 weeks over bony
surfaces or near tendons.
• The nodules are described as firm and
painless, and the overlying skin is not
inflamed. They are typically smaller and
shorter lived than nodules of rheumatoid
arthritis.
Subcutaneous nodule
Clinical Features—cont-• Erythema marginatum
• is a classic skin rash that occurs early in the
course of acute rheumatic fever.
• The rash is evanescent, pink to red and
nonpruritic.
• It typically occurs on the trunk or proximal
limbs.
Erythema marginatum
• The rash appears as a ring that extends
centrifugally, while the skin in the center of
the ring returns to normal. The rash can
persist or recur after other symptoms of
acute rheumatic fever have passed.
• Interestingly, erythema marginatum and
the subcutaneous nodules usually occur
only in patients with carditis.
Erytherma marginatum
Erytherma marginatum
Carditis and Rheumatic Heart Disease
• The term carditis refers to diffuse
inflammation of the pericardium,
epicardium, myocardium, and
endocardium.
• Valvular involvement, with leaflet
thickening, occurs as a rule; in addition,
the valves frequently display small rows of
vegetations called verrucae along their
appointing surfaces.
Carditis and Rheumatic Heart Disease
• Symptoms include tachycardia and mild or
moderate chest discomfort that is
commonly pleuritic in nature.
Heart -Pathology
Rheumatic Heart Diseases
Commissural fusion
Vagetations
RHD:-(thickened mitral valve, thickened chordae
tendineae, hypertrophied left ventricular
myocardium).
Carditis and Rheumatic Heart Disease
• The cardiac physical examination often
reveals the presence of pericardial friction
rub and typically, new or changing
murmurs.
• In young patients, mitral valve
regurgitation is the predominant cardiac
lesion
• A new apical systolic murmur is
characteristic
• Aortic regurgitation is less common but
can develop
• The pulmonary and tricuspid valves are
rarely involved
• Mitral stenosis becomes progressively
more common in early to mid adulthood.
• Heart block of all degrees is seen on
ECGs
• The most common radiologic finding is
cardiomegaly.
Carditis and Rheumatic Heart Disease
• Myocarditis is characterized by infiltration of
mononuclear cells,
 vasculitis, and
 degenerative changes of the insterstitial
connective tissue.
• The pathognomonic lesion is the Aschoff body in
the proliferative stage, which is present in 30%
to 40% of biopsy samples from patients with
acute rheumatic fever.
Carditis and Rheumatic Heart Disease
The use of echocardiography has contributed
much toward understanding the
pathogenesis of valvular regurgitation in
rheumatic carditis.
• At least initially, the regurgitation appears
to result from geometric changes and
stresses affecting the left ventricle rather
than from the rheumatic process directly
involving the valve leaflets.
Carditis and Rheumatic Heart Disease
• Acute rheumatic fever is also the predominant
cause of mitral stenosis; approx. 25% of
patients with rheumatic heart disease have pure
mitral stenosis.
• Mitral stenosis is characterized by progressive
thickening, fibrosis and calcification of the
leaflets and chordae tendineae.
• The leaflets show fibrous obliteration and the
mitral valve orifice becomes funnel- shaped, like
a fish- mouth.
• This feature and the classic hockey-stick
appearance of the anterior mitral leaflet in
diastole are well seen on echocardiograms
Heart -Pathology
Rheumatic Heart Diseases
? cells
pathognomonic