RHEUMATIC FEVER AND

RHEUMATIC HEART DISEASE

CASE
One sunny day in the PEDIA-OPD, you met Sia
Furler, 9 years old who consulted due to a
complaint of chest discomfort with occasional
associated chest pain. While interviewing her
MOM, this was your observation on SIA
A weary heart for an unrequited love leads to
misery and cause HEART BREAK

Holding on to what you know is fleeting is a

great TRAGEDY than LETTING GO..

But we should know that the GREATEST

TRAGEDY is that..
RHEUMATIC FEVER
 It is an acute, immunologically mediated,
multisystem inflammatory disease that
occurs a few weeks after an episode of
group A streptococcal pharyngitis.
 Acute rheumatic carditis is a frequent
manifestation during the active phase
of RF

May progress over time to chronic

rheumatic heart disease (RHD), of
which valvular abnormalities are key
manifestations
 EPIDEMIOLOGY
In developing countries, and in many
crowded, economically depressed urban
areas in the Western world, RHD
remains an important public health
problem, affecting an estimated 15
million people
 ETIOLOGY
66% of the patients with an acute
episode of rheumatic fever have a
history of an upper respiratory tract
infection several weeks before

Peak age and seasonal incidence of

acute rheumatic fever closely parallel
those of GABHS infections
 ETIOLOGY
Patients with acute rheumatic fever
almost always have serologic evidence of
a recent GABH S infection.
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
ETIOLOGY
 ETIOLOGY
WHO is GAS?
Classified accordingly through the M protein
M types commonly associated with
pharyngitis: Types 1, 12, 28, 3, 4, 2, and 6
M type 12 is associated with AGN and
pharyngitis
M types 49, 55, 57, and 60 are purely
nephritogenic
 ETIOLOGY
VIRULENCE:
M Protein resist phagocytosis,
stimulates SPECIFIC antibody
production
Erythrogenic Toxins (SPE-Strep
Pyrogenic Exotoxin)
SPE A, B, C resp. for Scarlet Fever
Rash
 ETIOLOGY
VIRULENCE FACTOR DESCRIPTION
Streptolysin O An exotoxin, one of the bases of
the organism's beta-hemolytic
property
Streptolysin S A cardiotoxic exotoxin, another
beta-hemolytic component, not
immunogenic and O2 stable: A
potent cell poison affecting
many types of cell including
neutrophils, platelets, and sub-
cellular organelles,
 ETIOLOGY
VIRULENCE FACTOR DESCRIPTION
Streptokinase Enzymatically activates
plasminogen, a proteolytic
enzyme, into plasmin, which in
turn digests fibrin and other
proteins
Hyaluronidase facilitate the spread of the
bacteria through tissues by
breaking down hyaluronic acid,
an important component of
connective tissue.
 VIRULENCE FACTOR DESCRIPTION
Streptodornase AKA DNases, which protect the
bacteria from being trapped in
neutrophil extracellular traps
(NETs) by digesting the NETs'
web of DNA
C5a Peptidase cleaves a potent neutrophil
chemotaxin called C5a, which is
produced by the complement
system which minimizes the
influx of neutrophils early in
infection as the bacteria are
attempting to colonize the
host's tissue.
 VIRULENCE FACTOR DESCRIPTION
Streptococcal Prevents the migration of
chemokine protease neutrophils to the spreading
infection. ScpC degrades the
chemokine IL-8, which would
otherwise attract neutrophils to
the site of infection.
 PATHOPHYSIOLOGY
ESTABLISHED PATHOGENESIS
1. Lost of Self Tolerance
2. Overwhelming Self Reactivity
(Structural)
3. Genetic Vulnerability
4. Abnormal Autoimmune Amplification
(Neuroendocrine Mediated)
PATHOPHYSIOLOGY
PATHOPHYSIOLOGY
 MORPHOLOGY
Aschoff bodies
- distinctive lesions in the heart ,
consisting of foci of lymphocytes,
occasional plasma cells, and plump
activated macrophages called
Anitschkow cells
- may be found in any of the three layers
of the heart
PATHOPHYSIOLOGY
 CLINICAL MANIFESTATIONS
The Jones criteria are intended only for
the diagnosis of the initial attack of
acute rheumatic fever and not for
recurrences

5 major and 4 minor criteria and an

absolute requirement for evidence of
recent GAS infection
 CLINICAL MANIFESTATIONS
The diagnosis of acute rheumatic fever
can be established by the Jones criteria
when a patient fulfills 2 major criteria or
1 major and 2 minor criteria and meets
the absolute requirement
CARDITIS (Review of Anatomy)
CARDITIS (Review of Anatomy)
HEART SOUNDS
 CARDIAC CYCLE
Systole S1---------- S2------S1
2 components

Diastole S1---------- S2------S1

3 components
 CARDITIS
50-60 %
Pancarditis - Most impt and serious finding in
ARF
Carditis residual of ARF that leads to chronic
changes
Endocarditis Valvulitis; universal finding of
Rheumatic Carditis
 CARDITIS
50-60 %
Pancarditis - Most impt and serious finding in
ARF
Carditis residual of ARF that leads to chronic
changes
Endocarditis Valvulitis; universal finding of
Rheumatic Carditis
 CARDITIS
Acute: Valvular Insufficiency (sometimes in
Chronic)
Chronic: Fish mouth or even stenosis
Other manifestation: aortic and mitral
regurgitation, pericarditis, pericardial effusion,
dec. ventricular contractility arrhythmias
(commonly 1st degree heart block)
 POLYARTHRITIS
Caveat?
Exquisitely tender (Nelson)
Migratory involves knees, wrists, ankle*
Symmetric or Asymmetric?
Effusions may be present Leukocytosis*
Does not lead to chronic joint disease
resolves in 12-24 hours
 POLYARTHRITIS
Synovial fluid:
10,000 100,000 WBCs/mm3 (mostly neutrophil)
Normal Glucose Level
Forms good mucin clot
Earliest manifestation and correlates with peak of
titers
Inverse Relationship bet. Severity of arthritis and
cardiac involvement
 CHOREA
10-15 %
Sydenham Chorea Unique part of ARF signs
Choreoathetoid movements occur later
Subtle beginning difficult to elicit
Best signs? (school-aged individuals)
the only symptom of ARF
Disappears in weeks and in months
 CHOREA
Characterized by emotional lability,
incoordination, poor school performance,
uncontrollable movements, and facial
grimacing. Exacerbated by stress and
disappearing with sleep
Occ. Unilateral
Longer latent period
 CHOREA
Clinical Maneuvers to elicit features of Chorea:
1. Demonstration of Milkmaids Grip (irregular
contractions of the muscles of the hands while
squeezing the examiners fingers)
2. Spooning and pronation of the hands when the
patients arms are extended
3. Wormian darting movements of the tongue upon
protrusion
4. Examination of handwriting to evaluate fine moto
movements
 ERYTHEMA MARGINATUM
<3%
Difficult to detect evanescent and occurs
infrequently; sometimes mistaken as rash of
Lymes disease
Nonspecific pink macules on the trunk that
when fully develops, blanching in the center
occurs and sometimes fusion of the borders
leading to serpinginous-looking lesion
 ERYTHEMA MARGINATUM
Worsens with heat application but does not
itch.
Spares the face
Occurs frequently with carditis*
Erythema Marginatum
Erythema Marginatum
 SUBCUTANEOUS NODULES
<1%
Seen concomitantly with severe carditis
(correlation)
Pea-size nodules, firm and non-tender
Non inflammed
Seen on extensors : knees and elbows
Subcutaneous Nodules
 MINOR CRITERIA
Arthralgia no objective
characteristic
Fever not higher than 103-104 F
Laboratory features
- Elevated erythrocyte sedimentation
rate
- Elevated C-reactive protein
- Prolonged PR interval
ANTECEDENT GAS INFECTION
Positive throat culture or rapid
streptococcal antigen test

Elevated or increasing streptococcal

antibody titer ASO (80%)
Chorea + indolent carditis
Summary of Diagnosis (Jones Criteria)
2 major criteria or 1 major + evidence of
preceding strep infection
Special Categories:
1. Chorea with other causes precluded
2. Insidious or late-onset carditis
3. Rheumatic Recurrence
No need of evidence of previous strep infection
is needed for either 1 or 2
Rheumatic Recurrence
In patients with documented rheumatic heart
disease or prior rheumatic fever, the presence of
one major criterion or of fever, arthralgia, or
elevated acute phase reactants suggests a
presumptive diagnosis of recurrence. Evidence
of previous streptococcal infection is needed
here
-Nelson
LABORATORY FINDINGS
No single specific lab test to confirm diagnosis
Throat Culture - Gold standard for
confirmation of GABHS presence
Rapid Antigen Detection Test
Streptococcal Antibody Test ASOt, ADB
ASOt peaks at 3-6 weeks post infection while
ADB peaks at 6-8 weeks post infection*
Acute and convalescent sera*
LABORATORY FINDINGS
ESR or CRP acute stage
RF, ANA , Complement Levels (C3)
Elevated IgG
ECG FINDINGS
1st Degree Heart Block (Prolonged PR Interval)
3rd or Complete Heart Block
Unremarkable in 1st attack
Left atrial enlargement
Cardiomegaly in roentnogram
DIFFERENTIAL DIAGNOSES
ARTHRITIS CARDITIS CHOREA
Rheumatoid Arthritis Viral Myocarditis Huntington Chorea
Reactive Arthritis Viral Pericarditis Wilsons Disease
(Shigella, Salmonella,
yersinia)
Serum Sickness Infective Endocarditis SLE
Sickle Cell Disease Kawasaki Disease Cerebral Palsy
Malignancy Congenital Heart Tics
Disease
SLE MVP Hyperactivity
Lyme Disease Innocent Murmurs
Gonococcal Infection
DIFFERENTIAL DIAGNOSES
Overlap Syndrome
Mixed Connective Tissue Disease
Undifferentiated Connective Tissue Disease
 TREATMENT
3 Approach Management:
1. Treat the etiology: GABHS
2. Control Clinical Manifestations
Anti-inflammatory
3. Supportive therapy CHF
 TREATMENT
10 full days of oral Antibiotic
A single IM injection of 1,200,000 units
of Benzathine Penicillin G
What if Allergic to Penicillin??
After this initial course of antibiotic
therapy, the patient should be started on
long-term antibiotic prophylaxis
 TREATMENT
Paracetamol can be used to control pain
and fever

Anti-inflammatory agents should be

withheld if arthralgia or atypical
arthritis is the only clinical manifestation
of presumed acute rheumatic fever
 TREATMENT
Typical migratory polyarthritis and carditis
without cardiomegaly or congestive heart
failure should be treated with oral salicylates
Aspirin - 100 mkday in 4 divided doses
PO for 3-5 days, followed by 75 mkday in
4 divided doses PO for 4 wk
 TREATMENT
If with carditis and cardiom egaly or
congestive heart failure, should receive
corticosteroids
Prednisone - 2 m g/kg/day in 4 divided
doses for 2-3 wk , then tapered by 5
mg/24 hr every 2-3 days
Aspirin - 75 mkday in 4 divided doses
for 6 wk, to be started during tapering
 TREATMENT
Supportive therapies for patients with
moderate to severe carditis:
Digoxin
Fluid and salt restriction
Diuretics
Oxygen
 TREATMENT
All patients with acute rheumatic fever
should be placed on bed rest and
monitored closely for evidence of
carditis

They can be allowed to ambulate as soon

as the signs of acute inflammation have
subsided
 TREATMENT
Termination of anti-inflammatory agents
depend on reappearance of s/sx and lab
abn.
For Sydenhams Chorea:
Sedatives help in the acute phase
Phenobarbital 16-32 mg every 6-8 hr PO
Haloperidol 0.01-0.03 mg/kg/24 hr BID PO
 COMPLICATIONS
The arthritis and chorea of acute
rheumatic fever resolve completely
without sequelae

Long-term sequelae of rheumatic fever

are usually limited to the heart
 PROGNOSIS
Approximately 70% of patients with
carditis during the initial episode of
acute rheumatic fever recover with no
residual heart disease; the more severe
the initial cardiac involvement, the
greater the risk is for residual heart
disease
 PROGNOSIS
Patients without carditis during the
initial episode are unlikely to have
carditis with recurrences
In contrast, patients with carditis during
the initial episode are likely to have
carditis with recurrences, and the risk for
permanent heart damage increases with
each recurrence
 PROGNOSIS
Patients who have had acute rheumatic
fever are susceptible to recurrent attacks
following reinfection of the upper
respiratory tract with GAS. Therefore,
these patients require long-term
continuous chemoprophylaxis
 PROPHYLAXIS
PRIMARY PROPHYLAXIS
Appropriate antibiotic therapy instituted
before the 9th day of symptoms of acute
GAS pharyngitis is highly effective in
preventing 1st attacks of acute
rheumatic fever from that episode
 PROPHYLAXIS
SECONDARY PROPHYLAXIS
Secondary prevention is directed at
preventing acute GAS pharyngitis in
patients at substantial risk of recurrent
acute rheumatic fever
 PROPHYLAXIS
Without carditis until 21 years old, or
5 years after since last rheumatic attack,
whichever is longer

With carditis but without residual

heart disease (no valvular disease)
until 21 years old, or 10 years after since
last rheumatic attack, whichever is
longer
 PROPHYLAXIS
With residual heart disease until 40
years old, or 10 years after since last
rheumatic attack, whichever is longer;
sometimes lifelong
 PROPHYLAXIS
Benzathine penicillin G - 600,000 IU
for children 60 lb and 1.2 million IU for
those >60 lb every 4 wk

In certain high-risk patients, and in areas

of the world where the incidence of
rheumatic fever is particularly high, use
of Pen G every 3 wk may be necessary
 PROPHYLAXIS
Penicillin V 250 mg BID PO (7am and
1pm)

and

Sulfadiazine 0.5 g <60 lbs 1g <60lbs

OD PO
 PROPHYLAXIS
If allergic to Penicillin, Macrolide
(Erythromycin or Clarithromycin) or
Azalide (Azithromycin)
RHEUMATIC HEART DISEASE

Is it a SEQUELAE or a
COMPLICATION?
RHEUMATIC HEART DISEASE
Rheumatic heart disease is permanent
damage to the heart following rheumatic
fever.
-is a chronic heart condition
most common acquired heart disease in
children in many countries of the world,
PATHOPHYSIOLOGY
PATHOPHYSIOLOGY
 MORPHOLOGY
Inflammation of the endocardium and
the left-sided valves typically results in
fibrinoid necrosis within the cusps or
along the tendinous cords
Verrucae
- Overlying these necrotic foci are small
(1- to 2-mm) vegetations along the
lines of closure
 MORPHOLOGY
Verrucae disappears and leaves a scarred
tissue that when repeated leads to
involvement of the cordae tendinae
Subendocardial lesions, perhaps
exacerbated by regurgitant jets, may
induce irregular thickenings called
MacCallum plaques, usually in the left
atrium
 MORPHOLOGY
Mitral valve alone - 65% to 70%
Mitral + Aortic - 25% of cases.
Tricuspid valve - infrequent
Pulmonary valve - rare
 PHYSICAL ASSESSMENT

INSPECTION: heaves
PERCUSSION
PALPATION: pulsation, thrill, lifts
AUSCULATION: S1, S2, murmur
AUSCULTATION
AUSCULTATION
Assessment of Murmur
Is there a murmur?
When is it heard?
Where is it located?
Does it radiate? Where?
What is the shape?
What is the pitch?
What is the intensity/grade?
What is the quality?
What is the response to maneuver
 TIMING
Systole S1---------- S2---------------S1
2 components
Rapid Ejection
Slow Ejection

Diastole S1---------- S2---------------S1

3 components
Early (Rapid Filling)
Middle
Late
LOCATION/RADIATION
SHAPE
 PITCH
HIGH - High Pressure Gradients (VSD)

LOW Large Volume across low pressure

gradients (Mitral Stenosis)

HARSH High Pressure Gradient + Large

Volume of flow (Aortic Stenosis)
 QUALITY
Subjective

Blowing?
Musical?
Rumbling?
Harsh?
Machine Like?
RESPONSE TO MANEUVERS
INTENSITY
 Mitral Insufficiency
Loss of valvular substance
shortening and thickening of the
chordae tendineae
Mitral Insufficiency
ARF with Severe Mitral Insuff. + Pericardial,
Cardiac Involv. Myocardial, and Endocardial
Inflamm.

left ventricle high volume

enlarges load

Blood
regurgitates Inc LA pressure

Pulmonary
Congestion (LSHF)
 Mitral Insufficiency
Spontaneous improvement usually
occurs with time
Patient is asymptomatic (Quiet
Precordium)
More than half of patients with acute
mitral insufficiency no longer have the
mitral murmur 1 yr later
 Mitral Insufficiency
High-pitched holosystolic murmur at the
apex that radiates to the axilla
Heart is enlarged, with a heaving apical
left ventricular impulse and often an
apical systolic thrill
2nd heart sound may be accentuated if
pulmonary hypertension is present
3rd heart sound is generally prominent
 Mitral Insufficiency
Short mid-diastolic rumbling murmur is
caused by increased blood flow across the
mitral valve as a result of insuff.
ECG: Prominent Bifid P waves ; signs of
LVH and RVH if hypertension is present
 Mitral Insufficiency
Mild - prophylaxis against RF
Afterload-reducing agents (ACE
inhibitors or ARBs) may reduce the
regurgitant volume and preserve left
ventricular function
Surgical treatment: annuloplasty or valve
replacement
 Mitral Stenosis
Results from fibrosis of the mitral ring,
commissural adhesions, and contracture
of the valve leaflets, chordae, and
papillary muscles over time

Usually takes 10 yr or more for the

lesion to become fully established
increased pressure and enlargement
and hypertrophy of the left atrium

pulmonary venous hypertension,

increased pulmonary vascular
resistance, and pulmonary
hypertension
 Right ventricular hypertrophy and
right atrial dilatation ensue

RV dilation, tricuspid regurgitation,

and clinical signs of right-sided heart
failure
 Mitral Stenosis
Mild - asymptomatic
Severe - exercise intolerance and
dyspnea;
Critical - orthopnea, paroxysmal
nocturnal dyspnea, overt pulmonary
edema, atrial arrhythmias.
pulmonary hypertension/right
ventricular dilatation - functional
tricuspid insufficiency, hepatomegaly,
ascites, and edema
 Mitral Stenosis
Hemoptysis caused by rupture of
bronchial or pleurohilar veins and,
occasionally , by pulmonary infarction
may occur
 Mitral Stenosis
Parasternal right ventricular lift

Loud 1st heart sound, an opening snap

of the mitral valve, and a long, low-
pitched, rumbling mitral diastolic
murmur with presystolic accentuation at
the apex (Absent in significant heart
failure)
 Mitral Stenosis
A holosystolic murmur sec to tricuspid
insuff. may be audible
Pulmonic component of S2 is
accentuated due to presence of pul.
HPN
An early diastolic murmur may be caused
by associated aortic insuff. or pulmonary
valvular insuff sec. to pulm. HPN
 Mitral Stenosis
ECG
1. Severe: Notched P waves and Varying
degrees of RVH
2. Atrial Fibrillation is a common late
manifestation
 Mitral Stenosis
Symptomatic
Surgical valvotomy or balloon catheter
mitral valvuloplasty
Valve replacement is avoided unless
absolutely necessary
Balloon valvuloplasty is indicated for
symptomatic, stenotic, pliable,
noncalcified valve of patients without
atrial arythmias or thrombi
 Aortic Insufficiency
Sclerosis of the aortic valve results in
distortion and retraction of the cusps
Regurgitation of blood leads to volume
overload with dilatation and hypertrophy
of the left ventricle
Combined mitral and aortic insuff >
aortic involvement alone
 Aortic Insufficiency
Symptoms are unsual except in severe

Palpitations, sweating and heat

intolerance

Dyspnea on exertion
 Aortic Insufficiency
Wide pulse pressure, bounding
peripheral pulses

Left ventricular apical heave and

diastolic thrill
 Aortic Insufficiency
Typical murmur begins immediately
with the 2nd heart sound and continues
until late in diastole; heard over the
upper and midleft sternal border with
radiation to the apex and upper right
sternal border
high-pitched blowing quality, easily
audible in full expiration
 Aortic Insufficiency
Aortic Systolic Ejection Murmur is
frequent because of Increased Stroke
Volume
An apical presystolic murmur (Austin
Flint Murmur) resembling that of mitral
stenosis is sometimes heard and is a
result of the large regurgitant aortic
flow in diastole preventing the mitral
valve from opening fully
 Sign Description
Becker Sign Visible systolic pulsations of the retinal arterioles

Corrigan pulse ("water- Abrupt distention and quick collapse on palpation

hammer" pulse) of the peripheral arterial pulse

de Musset sign Bobbing motion of the patient's head with each

heartbeat
Hill sign Popliteal cuff systolic blood pressure 40 mm Hg
higher than brachial cuff systolic blood pressure

Duroziez sign Systolic murmur over the femoral artery with

proximal compression of the artery, and diastolic
murmur over the femoral artery with distal
compression of the artery

Mller sign Visible systolic pulsations of the uvula

Quincke sign Visible pulsations of the fingernail bed with light
compression of the fingernail

Traube sign ("pistol-shot" Booming systolic and diastolic sounds auscultated

pulse) over the femoral artery
 Aortic Insufficiency
ECG:
LVH
Strain with prominent P waves
 Aortic Insufficiency
does not regress; combined lesions
during the episode of acute rheumatic
fever may have only aortic involvement
1-2 yr later
afterload reducers and prophylaxis
against recurrence of acute rheumatic
fever
surgical intervention before
complications
 Tricuspid Valve Diseases
Rare after rheumatic fever

More common secondary to right

ventricular dilatation resulting from
unrepaired left-sided lesions
 Tricuspid Valve Diseases
Prominent pulsations of the jugular
veins, systolic pulsations of the liver, and
a blowing holosystolic murmur at the
lower left sternal border that increases in
intensity during inspiration.

Tricuspid valvuloplasty m ay be required

in rare cases.
 Pulmonary Valve Diseases
Pulmonary insufficiency usually occurs
on a functional basis secondary to
pulmonary hypertension

Late finding with severe mitral stenosis

 Pulmonary Valve Diseases
Murmur (Graham Steell murmur) is
similar to that of aortic insufficiency ,
but peripheral arterial sign are absent.

The correct diagnosis is confirmed by

two-dimensional echocardiography and
Doppler studies