DR.

KANUPRIYA CHATURVEDI

To know about the epidemiology of the disease

To understand the pathogenesis of rheumatic
heart disease
To know about the clinical features: cardiac & noncardiac manifestations
To learn about the laboratory studies for RHD
To understand the principles of management

Autoimmune consequence of infection with

Group A streptococcal infection

Results in a generalised inflammatory response

affecting brains, joints, skin, subcutaneous
tissues and the heart.

The clinical presentation can be vague and

difficult to diagnose.

Currently, the modified Duckett-Jones criteria

form the basis of the diagnosis of the condition.

Rheumatic diseases are defined by the

constellation of results of the physical
examination, autoimmune marker and other
serologic tests, tissue pathology, and
imaging.

Recognition of clinical patterns remains

essential for diagnosis because there is no
single diagnostic test and results may be
positive in the absence of disease.

 Rheumatic

Heart Disease is the

permanent heart valve damage resulting
from one or more attacks of ARF.
It is thought that 40-60% of patients with
ARF will go on to developing RHD.
The commonest valves affecting are the
mitral and aortic, in that order. However all
four valves can be affected

Rheumatic fever is thought to result from an

inflammatory autoimmune response.
Rheumatic fever only develops in children
and adolescents following group A betahemolytic streptococcal pharyngitis, and
only streptococcal infections of the pharynx
initiate or reactivate rheumatic fever.
Genetic studies show strong correlation
between progression to rheumatic heart
disease and human leukocyte antigen
(HLA)-DR class II alleles and the
inflammatory protein-encoding genes MBL2
and TNFA.

Both clones of heart tissueinfiltrating T cells and

antibodies have been found to be cross-reactive with
beta-hemolytic streptococcus.
Interferon (IFN)-gamma, tumor necrosis factor (TNF)alpha, and interleukin (IL)-10-(+) cells are
consistently predominant in valvular tissue, whereas
IL-4 regulatory cytokine expression is consistently
low.
The proposed pathophysiology for development of
rheumatic heart disease is as follows:
Cross-reactive antibodies bind to cardiac tissue facilitating
infiltration of streptococcal-primed CD4+ T cells, which then
trigger an autoimmune reaction releasing inflammatory
cytokines (including TNF-alpha and IFN-gamma).
Because few IL-4producing cells are present in valvular
tissue, inflammation persists, leading to valvular lesions

In 0.3-3% of cases, infection leads to

rheumatic fever several weeks after the
sore throat has resolved.
The organism spreads by direct contact with
oral or respiratory secretions, and spread is
enhanced by crowded living conditions.
Patients remain infected for weeks after
symptomatic resolution of pharyngitis and
may serve as a reservoir for infecting
others.

Group A Streptococcus is a gram-positive coccus

that frequently colonizes the skin and
oropharynx. This organism may cause
suppurative disease, such as pharyngitis,
impetigo, cellulitis, myositis, pneumonia, and
puerperal sepsis.
It also may be associated with nonsuppurative
disease, such as rheumatic fever and acute
poststreptococcal glomerulonephritis.
Group A streptococci elaborate the cytolytic
toxins streptolysins S and O.
Of these, streptolysin O induces persistently
high antibody titers that provide a useful marker
of group A streptococcal infection and its
nonsuppurative complications

Rheumatic fever develops in some children

and adolescents following pharyngitis with
group A beta-hemolytic Streptococcus
The organisms attach to the epithelial cells
of the upper respiratory tract and produce a
battery of enzymes allowing them to
damage and invade human tissues.
After an incubation period of 2-4 days, the
invading organisms elicit an acute
inflammatory response with 3-5 days of sore
throat, fever, malaise, headache, and an
elevated leukocyte count

Group A Streptococcus, as identified using

the Lancefield classification, has a group A
carbohydrate antigen in the cell wall that is
composed of a branched polymer of Lrhamnose and N- acetyl-D-glucosamine in a
2:1 ratio.
Group A streptococci may be subserotyped
by surface proteins on the cell wall of the
organism.
The presence of the M protein is the most
important virulence factor for group A
streptococcal infection in humans

Acute rheumatic heart disease often produces

a pancarditis characterized by endocarditis,
myocarditis, and pericarditis.
Endocarditis is manifested as valve
insufficiency.
The mitral valve is most commonly and
severely affected (65-70% of patients), and
the aortic valve is second in frequency (25%).
The tricuspid valve is deformed in only 10%
of patients and is almost always associated
with mitral and aortic lesions.
The pulmonary valve is rarely affected.
Pericarditis, when present, rarely affects
cardiac function or results in constrictive
pericarditis

Chronic manifestations due to residual and

progressive valve deformity occur in 9-39% of
adults with previous rheumatic heart disease.
Fusion of the valve apparatus resulting in
stenosis or a combination of stenosis and
insufficiency develops 2-10 years after an
episode of acute rheumatic fever, and recurrent
episodes may cause progressive damage to the
valves.
Fusion occurs at the level of the valve
commissures, cusps, chordal attachments, or
any combination of these.
Rheumatic heart disease is responsible for 99%
of mitral valve stenosis in adults in the United
States

At this time, rheumatic fever is uncommon

among children in the United States.
Incidence of rheumatic fever and rheumatic
heart disease has decreased in the United
States and other industrialized countries in
the past 80 years.
Decreased incidence of rheumatic fever has
been attributed to the introduction of
penicillin or a change in the virulence of the
Streptococcus.

Mortality/Morbidity

Rheumatic heart disease is the major cause

of morbidity from rheumatic fever and the
major cause of mitral insufficiency and
stenosis in the United States and the world.
Variables that correlate with severity of
valve disease include the number of
previous attacks of rheumatic fever, the
length of time between the onset of disease
and start of therapy, and sex.
The disease is more severe in females than
in males. Insufficiency from acute rheumatic
valve disease resolves in 60-80% of patients
who adhere to antibiotic prophylaxis

Race
Native Hawaiian and Maori (both of Polynesian
descent) have a higher incidence of rheumatic fever
(13.4 per 100,000 hospitalized children per year), even
with antibiotic prophylaxis of streptococcal
pharyngitis. Otherwise, race (when controlled for
socioeconomic variables) has not been documented to
influence disease incidence.

Sex
Rheumatic fever occurs in equal numbers in males and
females, but the prognosis is worse for females than
for males.

Age
Rheumatic fever is principally a disease of childhood,
with a median age of 10 years, although it also occurs
in adults (20% of cases).

 Prevalence

of RHD -0.67/1000 to
0.12/1000 children

(Periwal et al Bikaner) 2006

RHD

prevalence of 0.5 per 1000

children

(Misra et al. 2003 -2006)

The incidence of RF in Developing countries

is 27-100/1 lac /yr
(G.S.Sainani 2006)

The incidence of rheumatic fever (RF) varies

from 0.2 to 0.75/1000/ year in
schoolchildren 515 years of age (2001
Govt. Census)
(Anil Grover,Padamavati S et al, et.al INJ 2002)

A diagnosis of rheumatic heart disease is made

after confirming antecedent rheumatic fever.
The modified Jones criteria (revised in 1992)
provide guidelines for the diagnosis of
rheumatic fever.
The Jones criteria require the presence of 2
major or 1 major and 2 minor criteria for the
diagnosis of rheumatic fever.
The major diagnostic criteria include carditis,
polyarthritis, chorea, subcutaneous nodules,
and erythema marginatum.

The minor diagnostic criteria include fever,

arthralgia, prolonged PR interval on ECG,
elevated acute phase reactants (increased
erythrocyte sedimentation rate, presence of
C-reactive protein, and leukocytosis.
One of the following must be present:
-Positive throat culture or rapid
streptococcal antigen test result
-Elevated or rising streptococcal antibody
titer
-History of previous rheumatic fever or
rheumatic heart disease

 Physical

findings in a patient with

rheumatic heart disease include
cardiac and noncardiac manifestations
of acute rheumatic fever.

Some

patients develop cardiac

manifestations of chronic rheumatic
heart disease

Pancarditis is the most serious and second most

common complication of rheumatic fever (50%).
In advanced cases, patients may complain of
dyspnea, mild-to-moderate chest discomfort,
pleuritic chest pain, edema, cough, or orthopnea.
Upon physical examination, carditis is most
commonly detected by a new murmur and
tachycardia out of proportion to fever.
New or changing murmurs are considered
necessary for a diagnosis of rheumatic valvulitis.
Other cardiac manifestations include congestive
heart failure and pericarditis.

The murmurs of acute rheumatic fever are typically

due to valve insufficiency.
The following murmurs are most commonly
observed during acute rheumatic fever:

Apical pansystolic murmur is a high-pitched,

blowing-quality murmur of mitral regurgitation
that radiates to the left axilla.
Apical diastolic murmur (also known as a
Carey-Coombs murmur) is heard with active
carditis and accompanies severe mitral
insufficiency..
Basal diastolic murmur is an early diastolic
murmur of aortic regurgitation and is highpitched, blowing, decrescendo, and heard
best along the right upper and mid-left sternal
border after deep expiration while the patient
is leaning forward.

Congestive heart failure

Heart failure may develop secondary to severe valve
insufficiency or myocarditis.
The physical findings associated with heart failure
include tachypnea, orthopnea, jugular venous
distention, rales, hepatomegaly, a gallop rhythm,
edema, and swelling of the peripheral extremities.

Pericarditis
A pericardial friction rub indicates that pericarditis is
present.
Increased cardiac dullness to percussion and muffled
heart sounds are consistent with pericardial effusion.
A paradoxical pulse (and accentuated fall in systolic
blood pressure with inspiration) with decreased
systemic pressure and perfusion and evidence of
diastolic indentation of the right ventricle on
echocardiogram reflect impending pericardial
tamponade.

Common noncardiac (and diagnostic) manifestations

of acute rheumatic fever include polyarthritis, chorea,
erythema marginatum, and subcutaneous nodules.
Other clinical, noncardiac manifestations include
abdominal pain, arthralgias, epistaxis, fever, and
rheumatic pneumonia.

Polyarthritis is the most common symptom and is

frequently the earliest manifestation of acute rheumatic
fever (70-75%).
The arthritis reaches maximum severity in 12-24 hours,
persists for 2-6 days (rarely more than 3 wk) at each site,
and rapidly responds to aspirin.
Sydenham chorea occurs in 10-30% of patients with
rheumatic fever.
Physical findings include hyperextended joints, hypotonia,
diminished deep tendon reflexes, tongue fasciculations

A long latency period (1-6 mo) between

streptococcal pharyngitis and the onset of
chorea is observed.
Patients with chorea often do not
demonstrate other Jones criteria.It is also
known as rheumatic chorea, Sydenham
chorea, chorea minor, and St Vitus dance.
Daily handwriting samples can be used as an
indicator of progression or resolution of
disease.
Complete resolution of the symptoms
typically occurs with improvement in 1-2
weeks and full recovery in 2-3 months.

Pediatric autoimmune neuropsychiatric disorders

associated with streptococcal infections (PANDAS) may
be associated with chorea.
Children have been identified in whom group A
streptococcal infection appears to have triggered a
relapsing-remitting symptom complex characterized by
obsessive-compulsive disorder), and neurologic
abnormalities, such as cognitive defects and motoric
hyperactivity.
The symptoms are prepubertal in onset and may
include emotional lability, separation anxiety, and
oppositional behaviors.
Streptococcal infection has been proposed to trigger the
formation of antibodies that cross-react with the basal
ganglia of genetically susceptible hosts in a manner
similar to the proposed mechanism for Sydenham
chorea, thus causing the symptom complex.

Erythema marginatum, also known as erythema

annulare, is a characteristic rash that occurs in 5-13%
of patients with acute rheumatic fever.
It begins as 1-3 cm in diameter, pink-to-red
nonpruritic macules or papules located on the trunk
and proximal limbs but never on the face.
The lesions spread outward to form a serpiginous ring
with erythematous raised margins and central
clearing.
The rash may fade and reappear within hours and is
exacerbated by heat.
If the lesions are not well visualized, they can be
accentuated by the application of warm towels, a hot
bath, or the use of tangential lighting.
The rash occurs early in the course of the disease and
remains long past the resolution of other symptoms

Subcutaneous nodules are currently an infrequent

manifestation of rheumatic fever.
When present, the nodules appear over the
extensor surfaces of the elbows, knees, ankles,
knuckles, and on the scalp and spinous processes
of the lumbar and thoracic vertebrae where they
are attached to the tendon sheath.
They are firm, nontender, and free from
attachments to the overlying skin and range in
size from a few mm to 1-2 cm.
They vary in number from one to dozens (mean
3-4). Subcutaneous nodules generally occur
several weeks into the disease and resolve within
a month.
These nodules are strongly associated with
severe rheumatic carditis, and, in the absence of
carditis, the diagnosis of subcutaneous nodules
should be questioned.

Abdominal pain usually occurs at the onset of

acute rheumatic fever.
This pain resembles abdominal pain from other
conditions with acute microvascular mesenteric
inflammation and may mimic acute appendicitis.
Epistaxis may be associated with severe
protracted rheumatic carditis.
Fevers above 39C with no characteristic pattern
are initially present in almost every case of acute
rheumatic fever.
Patients with rheumatic pneumonia present with
the same signs as patients with infectious
pneumonia.
Rheumatic pneumonia should be differentiated
from respiratory distress related to congestive
heart failure

Valve deformities, thromboembolism, cardiac hemolytic

anemia, and atrial arrhythmias are the most common
cardiac manifestations of chronic rheumatic heart
disease.
Mitral stenosis occurs in 25% of patients with chronic
rheumatic heart disease and in association with mitral
insufficiency in another 40%.
Progressive fibrosis (ie, thickening and calcification of
the valve) takes place over time, resulting in
enlargement of the left atrium and formation of mural
thrombi in that chamber.
The stenotic valve is funnel-shaped, with a "fish mouth"
resemblance. Upon auscultation, S1 is initially
accentuated but becomes reduced as the leaflets
thicken. P2 becomes accentuated, and the splitting of S2
decreases as pulmonary hypertension develops.
An opening snap of the mitral valve often is heard at the
apex, where a diastolic filling murmur also is heard.

Aortic stenosis from chronic rheumatic heart

disease is typically associated with aortic
insufficiency.
The valve commissures and cusps become
adherent and fused, and the valve orifice
becomes small with a round or triangular shape.
Upon auscultation, S2 may be single because the
aortic leaflets are immobile and do not produce an
aortic closure sound.
The systolic and diastolic murmurs of aortic valve
stenosis and insufficiency are heard best at the
base of the heart.
Thromboembolism occurs as a complication of
mitral stenosis.
It is more likely to occur when the left atrium is
dilated, cardiac output is decreased, and the
patient is in atrial fibrillation

Cardiac hemolytic anemia is related to

disruption of the RBCs by a deformed valve.
Increased destruction and replacement of
platelets also may occur.
Atrial arrhythmias are typically related to a
chronically enlarged left atrium.
Successful cardioversion of atrial fibrillation
to sinus rhythm is more likely to be
successful if the left atrium is not markedly
enlarged, the mitral stenosis is mild, and
the patient has been in atrial fibrillation for
less than 6 months.

Throat culture
Throat culture findings for group A beta hemolytic
Streptococcus are usually negative by the time
symptoms of rheumatic fever or rheumatic heart
disease appear.

Rapid antigen detection test

This test allows rapid detection of group A
streptococcal antigen and allows the diagnosis of
streptococcal pharyngitis and the initiation of
antibiotic therapy while the patient is still in the
physician's office.

Antistreptococcal antibodies
The clinical features of rheumatic fever begin at
the time antistreptococcal antibody levels are at
their peak.
The elevated level of antistreptococcal
antibodies is useful, particularly in patients that
present with chorea as the only diagnostic
criterion.
Sensitivity for recent infections can be improved
by testing for several antibodies.
Antibody titers should be checked at 2-week
intervals in order to detect a rising titer.
The most common extracellular
antistreptococcal antibodies tested include
antistreptolysin O (ASO), antideoxyribonuclease
(DNAse) B, antihyaluronidase, antistreptokinase,
antistreptococcal esterase, and anti-DNA.

 Antibody tests for cellular components of group A

streptococcal antigens include antistreptococcal
polysaccharide, antiteichoic acid antibody, and anti
M protein antibody.
In general, the ratio of antibodies to extracellular
streptococcal antigens rises during the first month
after infection and then plateaus for 3-6 months
before returning to normal levels after 6-12 months.
When the ASO titer peaks (2-3 wk after the onset of
rheumatic fever), the sensitivity of this test is 8085%.
The anti-DNAse B has a slightly higher sensitivity
(90%) for detecting rheumatic fever or acute
glomerulonephritis.
Antihyaluronidase results are frequently abnormal in
rheumatic fever patients with a normal level of ASO
titer and may rise earlier and persist longer than
elevated ASO titers during rheumatic fever

Acute phase reactants

The C-reactive protein and erythrocyte
sedimentation rate are elevated in rheumatic
fever due to the inflammatory nature of the
disease. Both tests have a high sensitivity but
low specificity for rheumatic fever..

Heart reactive antibodies

Tropomyosin is elevated in acute rheumatic
fever.

Rapid detection test for D8/17

This immunofluorescence technique for
identifying the B cell marker D8/17 is positive in
90% of patients with rheumatic fever

 Chest

roentgenography

Cardiomegaly, pulmonary congestion, and

other findings consistent with heart failure
may be seen on chest radiography.
When the patient has fever and respiratory
distress, chest radiography helps
differentiate heart failure from rheumatic
pneumonia.

Doppler-echocardiogram
In acute rheumatic heart disease, Dopplerechocardiography identifies and quantitates valve
insufficiency and ventricular dysfunction.
With mild carditis, Doppler evidence of mitral regurgitation
may be present during the acute phase of disease but
resolves in weeks to months.
The most important echocardiographic features of mitral
regurgitation from acute rheumatic valvulitis are annular
dilatation, elongation of the chordae to the anterior leaflet,
and a posterolaterally directed mitral regurgitation jet.
During acute rheumatic fever, the left ventricle is
frequently dilated in association with a normal or increased
fractional shortening.
In chronic rheumatic heart disease, echocardiography may
be used to track the progression of valve stenosis and may
help determine the time for surgical intervention.

Heart catheterization
In acute rheumatic heart disease, this procedure is
not indicated.
With chronic disease, heart catheterization has
been performed to evaluate mitral and aortic valve
disease and to balloon stenotic mitral valves.
Postcatheterization precautions include
hemorrhage, pain, nausea and vomiting, and
arterial or venous obstruction from thrombosis or
spasm.
Complications may include mitral insufficiency
after balloon dilation of the mitral valve,
tachyarrhythmias, bradyarrhythmias, and vascular
occlusion

On ECG, sinus tachycardia most frequently

accompanies acute rheumatic heart
disease. Alternatively, some children
develop sinus bradycardia from increased
vagal tone.
First-degree atrioventricular (AV) block
(prolongation of the PR interval) is observed
in some patients with rheumatic heart
disease.
First-degree AV block is a nonspecific finding
and should not be used as a criterion for the
diagnosis of rheumatic heart disease.

Second-degree (intermittent) and thirddegree (complete) AV block with

progression to ventricular standstill have
been described
When acute rheumatic fever is associated
with pericarditis, ST segment elevation may
be present and is marked most in lead II, III,
aVF, and V4 -V6.
Patients with rheumatic heart disease also
may develop atrial flutter, multifocal atrial
tachycardia, or atrial fibrillation from
chronic mitral valve disease and atrial
dilation.

On ECG, sinus tachycardia most frequently

accompanies acute rheumatic heart disease.
Alternatively, some children develop sinus
bradycardia from increased vagal tone. No correlation
between bradycardia and the severity of the carditis
is noted.
First-degree atrioventricular (AV) block (prolongation
of the PR interval) is observed in some patients with
rheumatic heart disease.
This abnormality may be related to localized
myocardial inflammation involving the AV node or to
vasculitis involving the AV nodal artery. First-degree
AV block is a nonspecific finding and should not be
used as a criterion for the diagnosis of rheumatic
heart disease.
Its presence does not correlate with the development
of chronic rheumatic heart disease.

Second-degree (intermittent) and thirddegree (complete) AV block with progression

to ventricular standstill have been described.
Heart block in the setting of rheumatic fever,
however, typically resolves with the rest of
the disease process.
When acute rheumatic fever is associated
with pericarditis, ST segment elevation may
be present and is marked most in lead II, III,
aVF, and V4 -V6.
Patients with rheumatic heart disease also
may develop atrial flutter, multifocal atrial
tachycardia, or atrial fibrillation from chronic
mitral valve disease and atrial dilation.

Pathologic examination of the insufficient

valves may reveal verrucous lesions at the line
of closure.
Aschoff bodies (perivascular foci of eosinophilic
collagen surrounded by lymphocytes, plasma
cells, and macrophages) are found in the
pericardium, perivascular regions of the
myocardium, and endocardium.
Anitschkow cells are plump macrophages within
Aschoff bodies.
In the pericardium, fibrinous and serofibrinous
exudates may produce an appearance of "bread
and butter" pericarditis

Medical therapy in rheumatic heart disease

includes attempts to prevent rheumatic fever
(and thus rheumatic heart disease).
In patients who develop rheumatic heart
disease, therapy is directed toward eliminating
the group A streptococcal pharyngitis (if still
present), suppressing inflammation from the
autoimmune response, and providing
supportive treatment for congestive heart
failure.
Following the resolution of the acute episode,
subsequent therapy is directed towards
preventing recurrent rheumatic heart disease in
children and monitoring for the complications
and sequelae of chronic rheumatic heart
disease in adults.

For patients with GABHS pharyngitis, a meta-analysis

supports a protective effect against rheumatic fever
when penicillin is used following the diagnosis. [7]
Oral (PO) penicillin V remains the drug of choice for
treatment of GABHS pharyngitis, but ampicillin and
amoxicillin are equally effective.
When PO penicillin is not feasible or dependable, a single
dose of intramuscular benzathine penicillin G or
benzathine/procaine penicillin combination is
therapeutic.
For patients who are allergic to penicillin, administer
erythromycin or a first-generation cephalosporin. Other
options include clarithromycin for 10 days, azithromycin
for 5 days, or a narrow-spectrum (first-generation)
cephalosporin for 10 days. As many as 15% of patients
who are allergic to penicillin are also allergic to
cephalosporins.
Do not use tetracyclines or sulfonamides to treat GABHS
pharyngitis.

For recurrent group A streptococci (GAS) pharyngitis, a

second 10-day course of the same antibiotic may be
repeated. Alternate drugs include narrow-spectrum
cephalosporins, amoxicillin-clavulanate, dicloxacillin,
erythromycin, or other macrolides.
Control measures for patients with GABHS pharyngitis
are as follows:
Hospitalized patients: Place hospitalized patients with
GABHS pharyngitis of pneumonia on droplet precautions,
as well as standard precautions, until 24 hours after
initiation of appropriate antibiotics.
Exposed persons: People in contact with patients having
documented cases of streptococcal infection first should
undergo appropriate laboratory testing if they have
clinical evidence of GABHS infection and should undergo
antibiotic therapy if infected.
School and childcare centers: Children with GABHS
infection should not attend school or childcare centers
for the first 24 hours after initiating antimicrobial therapy.

For recurrent group A streptococci (GAS) pharyngitis,

a second 10-day course of the same antibiotic may
be repeated.
Alternate drugs include narrow-spectrum
cephalosporins, amoxicillin-clavulanate, dicloxacillin,
erythromycin, or other macrolides.
Control measures for patients with GABHS
pharyngitis are as follows:

Hospitalized patients: Place hospitalized patients with

GABHS pharyngitis of pneumonia on droplet precautions, as
well as standard precautions, until 24 hours after initiation of
appropriate antibiotics.
Exposed persons: People in contact with patients having
documented cases of streptococcal infection first should
undergo appropriate laboratory testing if they have clinical
evidence of GABHS infection and should undergo antibiotic
therapy if infected.
School and childcare centers: Children with GABHS infection
should not attend school or childcare centers for the first 24
hours after initiating antimicrobial therapy.

Therapy is directed towards eliminating the

GABHS pharyngitis (if still present), suppressing
inflammation from the autoimmune response,
and providing supportive treatment of congestive
heart failure.
Treat residual GABHS pharyngitis as outlined
above, if still present.
Treatment of the acute inflammatory
manifestations of acute rheumatic fever consists
of salicylates and steroids.
Aspirin in anti-inflammatory doses effectively
reduces all manifestations of the disease except
chorea, and the response is typically dramatic.
If rapid improvement is not observed after 24-36
hours of therapy, question the diagnosis of
rheumatic fever.

Attempt to obtain aspirin blood levels from 20-25

mg/dL, but stable levels may be difficult to achieve
during the inflammatory phase because of variable
GI absorption of the drug.
Maintain aspirin at anti-inflammatory doses until
the signs and symptoms of acute rheumatic fever
are resolved or residing (6-8 wk) and the acute
phase reactants (APRs) have returned to normal.
Anti-inflammatory doses of aspirin may be
associated with abnormal liver function tests and GI
toxicity, and adjusting the aspirin dosage may be
necessary.
When discontinuing therapy, withdraw aspirin
gradually over weeks while monitoring the APRs for
evidence of rebound.
Chorea is most frequently self-limited but may be
alleviated or partially controlled with phenobarbital
or diazepam

If moderate to severe carditis is present as

indicated by cardiomegaly, third-degree heart
block or congestive heart failure, substitute PO
prednisone for salicylate therapy.
Continue prednisone for 2-6 weeks depending
on the severity of the carditis, and taper
prednisone during the final week(s) of therapy.
Weaning prednisone therapy after a shorter
period (2-4 weeks) while initiating and
maintaining salicylates for several weeks can
minimize adverse effects of the steroids while
preventing rebound of the carditis.

Include digoxin and diuretics, afterload reduction,

supplemental oxygen, bed rest, and sodium and
fluid restriction as additional treatment for patients
with acute rheumatic fever and heart failure.
The diuretics most commonly used in conjunction
with digoxin for children with heart failure include
furosemide and spironolactone.
Initiate digoxin only after checking electrolytes and
correcting hypokalemia.
Include digoxin and diuretics, afterload reduction,
supplemental oxygen, bed rest, and sodium and
fluid restriction as additional treatment for patients
with acute rheumatic fever and heart failure.
The diuretics most commonly used in conjunction
with digoxin for children with heart failure include
furosemide and spironolactone.
Initiate digoxin only after checking electrolytes and
correcting hypokalemia

The total digitalizing dose is 20-30

mcg/kg PO, with 50% of the dose
administered initially, followed by 25%
of the dose 12 hours and 24 hours after
the initial dose.
Maintenance doses typically are 8-10
mcg/kg/d PO in 2 divided doses.
For older children and adults, the total
loading dose is 1.25-1.5 mg PO, and the
maintenance dose is 0.25-0.5 mg PO
every day.
Therapeutic digoxin levels are present at
trough levels of 1.5-2 ng/mL.

Afterload reduction (ie, using ACE inhibitor

captopril) may be effective in improving
cardiac output, particularly in the presence of
mitral and aortic insufficiency.
Start these agents judiciously. Use a small,
initial test dose (some patients have an
abnormally large response to these agents),
and administer only after correcting
hypovolemia.
When heart failure persists or progresses
during an episode of acute rheumatic fever in
spite of aggressive medical therapy, surgery is
indicated and may be life-saving for severe
mitral and/or aortic insufficiency.

 Treatment

for patients following

rheumatic heart disease (RHD)
Preventive and prophylactic therapy is
indicated after rheumatic fever and acute
rheumatic heart disease to prevent further
damage to valves.
Primary prophylaxis (initial course of
antibiotics administered to eradicate the
streptococcal infection) also serves as the
first course of secondary prophylaxis
(prevention of recurrent rheumatic fever and
rheumatic heart disease).
.

 An injection of 0.6-1.2 million units of

benzathine penicillin G intramuscularly every
4 weeks is the recommended regimen for
secondary prophylaxis for most US patients.
Administer the same dosage every 3 weeks in
areas where rheumatic fever is endemic, in
patients with residual carditis, and in high-risk
patients.
Although PO penicillin prophylaxis is also
effective, data from the World Health
Organization indicate that the recurrence risk
of GABHS pharyngitis is lower when penicillin
is administered parentally

The duration of antibiotic prophylaxis is

controversial.
Continue antibiotic prophylaxis
indefinitely for patients at high risk (eg,
health care workers, teachers, daycare
workers) for recurrent GABHS infection.
Patients with rheumatic fever with
carditis and valve disease should
receive antibiotics for at least 10 years
or until age 40 years.

Patients with rheumatic heart disease and

valve damage require a single dose of
antibiotics 1 hour before surgical and dental
procedures to help prevent bacterial
endocarditis.
Patients who had rheumatic fever without
valve damage do not need endocarditis
prophylaxis.
Do not use penicillin, ampicillin, or amoxicillin
for endocarditis prophylaxis in patients already
receiving penicillin for secondary rheumatic
fever prophylaxis (relative resistance of PO
streptococci to penicillin and aminopenicillins.

Alternate drugs recommended by the American

Heart Association for these patients include PO
clindamycin (20 mg/kg in children, 600 mg in
adults) and PO azithromycin or clarithromycin (15
mg/kg in children, 500 mg in adults).
The guidelines for endocarditis prophylaxis in
patients with valve damage from rheumatic heart
disease have changed. Antibiotic prophylaxis is no
longer recommended.
A recent study investigated the difference in
clinical manifestations and outcomes between
first episode and recurrent rheumatic fever.
The study concluded that subclinical carditis
occurred only in patients experiencing the first
episode, and that all deaths occurred in patients
with recurrent rheumatic fever, emphasizing the
need for secondary prophylaxis.

When heart failure persists or worsens after

aggressive medical therapy for acute rheumatic
heart disease, surgery to decrease valve
insufficiency may be life-saving.
Forty percent of patients with acute rheumatic
heart disease subsequently develop mitral
stenosis as adults.
In patients with critical stenosis, mitral
valvulotomy, percutaneous balloon valvuloplasty,
or mitral valve replacement may be indicated.
Due to high rates of recurrent symptoms after
annuloplasty or other repair procedures, valve
replacement appears to be the preferred surgical
option