Rheumatic Heart Disease

Rheumatic Fever
 Etiology
• Acute rheumatic fever is a systemic disease of
childhood,often recurrent that follows group A
beta hemolytic streptococcal infection
• It is a delayed non-suppurative sequelae to
URTI with GABH streptococci.
• It is a diffuse inflammatory disease of
connective tissue,primarily involving
heart,blood vessels,joints, subcut.tissue and
CNS
Epidemiology
• Ages 5-15 yrs are most susceptible
• Rare <3 yrs
• Girls>boys
• Common in 3rd world countries
• Environmental factors-- over crowding,
poor sanitation, poverty
Pathogenesis
• Delayed immune response to infection with
group.A beta hemolytic streptococci.
• After a latent period of 1-3 weeks, antibody
induced immunological damage occur to
heart valves,joints, subcutaneous tissue
& basal ganglia of brain
Pathologic Lesions
• Fibrinoid degeneration of connective
tissue,inflammatory edema, inflammatory cell
infiltration & proliferation of specific cells resulting
in formation of Ashcoff nodules, resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous
tissue
-Basal gangliar lesions resulting in
chorea
Clinical Features
1.Arthritis
• Flitting & fleeting migratory polyarthritis,
involving major joints
• Commonly involved joints-knee,ankle,elbow
& wrist
• Occur in 80%,involved joints are exquisitely
tender
• In children below 5 yrs arthritis usually mild
but carditis more prominent
• Arthritis do not progress to chronic disease
 Clinical Features (Contd)
2.Carditis
• Manifest as pancarditis(endocarditis,
myocarditis and pericarditis),occur in 40-
50% of cases
• Carditis is the only manifestation of
rheumatic fever that leaves a sequelae &
permanent damage to the organ
• Valvulitis occur in acute phase
• Chronic phase- fibrosis,calcification &
stenosis of heart valves (fishmouth valves)
Clinical Features
3.Sydenham Chorea(Contd)
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• May appear even 6/12 after the attack of
rheumatic fever
• Clinically manifest as-clumsiness,
deterioration of handwriting,emotional
lability or grimacing of face
• Clinical signs- pronator sign, jack in the box
sign , milking sign of hands
Clinical Features (Contd)
4.Erythema Marginatum
• Occur in <5%.
• Unique,transient,serpiginous-looking
lesions of 1-2 inches in size
• Pale center with red irregular margin
• More on trunks & limbs & non-itchy
• Worsens with application of heat
• Often associated with chronic carditis
Clinical Features (Contd)
5.Subcutaneous nodules
• Occur in 10%
• Painless,pea-sized,palpable nodules
• Mainly over extensor surfaces of
joints,spine,scapulae & scalp
• Associated with strong seropositivity
• Always associated with severe carditis
Clinical Features (Contd)
Other features (Minor features)
• Fever-(upto 101 degree F)
• Arthralgia
• Pallor
• Anorexia
• Loss of weight
 Laboratory Findings
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protien
• ASO titre >200 Todd units.
(Peak value attained at 3 weeks,then
comes down to normal by 6 weeks)
• Anti-DNAse B test
• Throat culture-GABHstreptococci
 Laboratory Findings (Contd)
• ECG- prolonged PR interval, 2nd or 3rd
degree blocks,ST depression, T
inversion
• 2D Echo cardiography- valve edema,mitral
regurgitation, LA & LV
dilatation,pericardial effusion,decreased
contractility
Diagnosis
• Rheumatic fever is mainly a clinical
diagnosis
• No single diagnostic sign or specific
laboratory test available for diagnosis
• Diagnosis based on MODIFIED JONES
CRITERIA
 Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor Supporting Evidence
Manifestations of Streptococal Infection
Carditis Clinical Laboratory
Polyarthritis Previous Acute phase
Chorea rheumatic reactants: Increased Titer of Anti-
Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO
Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O),
heart disease rate, others
Arthralgia C-reactive Positive Throat Culture
Fever protein, for Group A Streptococcus
leukocytosis Recent Scarlet Fever
Prolonged P-
R interval

*The presence of two major criteria, or of one major and two minor criteria,
indicates a high probability of acute rheumatic fever, if supported by evidence of
Group A streptococcal nfection.

Recommendations of the American Heart Association

Exceptions to Jones Criteria

 Chorea alone, if other causes have been

excluded
 Insidious or late-onset carditis with no
other explanation
 Treatment
• Step I - primary prevention
(eradication of streptococci)
• Step II - anti inflammatory treatment
(aspirin,steroids)
• Step III- supportive management &
management of complications
• Step IV- secondary prevention
(prevention of recurrent attacks)
STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb)
1 200 000 U for patients >27 kg
or
Penicillin V Children: 250 mg 2-3 times daily Oral 10 d
(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)

or
Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Recommendations of American Heart Association
Step II: Anti inflammatory treatment
Clinical condition Drugs
Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
3.Step III: Supportive management &
management of complications

• Bed rest
• Treatment of congestive cardiac failure:
-digitalis,diuretics
• Treatment of chorea:
-diazepam or haloperidol
• Rest to joints & supportive splinting
STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode

Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular

or
Penicillin V 250 mg twice daily Oral

or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)

For individuals allergic to penicillin and sulfadiazine

Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified and

recommended
Recommendations of American Heart Association
Duration of Secondary Rheumatic Fever
Prophylaxis
Category
Rheumatic fever with carditis and
residual heart disease
(persistent valvar disease*)
Duration
At least 10 y since last
episode and at least until
age 40 y, sometimes lifelong
prophylaxis

Rheumatic fever with carditis 10 y or well into adulthood,

but no residual heart disease whichever is longer
(no valvar disease*)

Rheumatic fever without carditis 5 y or until age 21 y,

whichever is longer

*Clinical or echocardiographic
Recommendations of evidence.
American Heart Association
 Secondary
prevention: Duration
CATEGORY DURATION OF PROPHYLAXIS
All persons with MINIMUM 10 years after most recent
ARF with no or mild
carditis episode or age 21
All persons with MINIMUM 10 years after most recent
ARF and moderate
carditis episode or age 35

All persons with MINIMUM 10 years after most recent

ARF and severe
carditis episode or age 35 and then specialist
review for need to continue. Post surgical
cases definitely lifelong.
Awareness ♦ Surveillance ♦ Advocacy ♦ Prevention
Prognosis
• Rheumatic fever can recur whenever the
individual experience new GABH
streptococcal infection,if not on
prophylactic medicines
• Good prognosis for older age group & if no
carditis during the initial attack
• Bad prognosis for younger children & those
with carditis with valvar lesions
 RHEUMATIC HEART
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.
 RHEUMATIC HEART
DISEASE

• The commonest valves affecting

are the mitral and aortic, in that
order. However all four valves
can be affected.
 RHEUMATIC HEART
DISEASE
• Sadly, RHD can go undetected
with the result that patients
present with debilitating heart
failure.
• At this stage surgery is the only
possible treatment option.
 What is the
incidence of acute
rheumatic fever and
rheumatic heart
disease?
 Incidence of ARF:
Population-based Studies
Figure 5: Trend in Incidence of First Attack of Acute
Rheumatic Fever Over Time
40
USA (all ages)
35

Incidence/100,000 population
Martinique (<20yrs)
New Zealand (<30yrs)
Kuwait (5-14yrs) 30
Iran (all ages
25

20

15

10

5
1 2 0
3 4
5 6
7 8
Time (years) 9 10 11
 What is the
prevalence of
rheumatic
heart disease?
 RHEUMATIC FEVER IS
PREVENTABLE

Costa Rica

Cuba
There was a progressive decline in the
occurrence and severity of acute RF and RHD,
with a marked decrease in the prevalence of
RHD in school children.
A marked and progressive decline was also seen
in the incidence and severity of ARF.

There was an even more marked reduction in

recurrent attacks of RF as well as in the number
and severity of patients requiring hospitalisation
and surgical care.
What are the
clinical
features of
strep sore
throat?
 Hallmarks of STREP
sore throat
• Tender lymph nodes
• Close contact with infected person
• Scarlet fever rash
• Excoriated nares( crusted lesions) in infants
• Tonsillar exudates in older children
• Abdominal pain
• GOLD STANDARD: POSITIVE THROAT
CULTURE
 Hallmarks of VIRAL
sore throat
• Coryza: runny nose or mouth ulcers
• Other family with COLD symptoms
• Evidence of another viral infection
• Itchy watery eyes
• Hoarseness and cough: non-specific
• Fever: not specific
• Red Throat: not specific
What are the
treatment
regimens of
streptococcal
sore throat?
 Primary Prevention of
Rheumatic Fever by
treating sore throat
Antibiotic Administration Dose
Benzathine Single IM injection 1.2 MU > 30kg
benzyl penicillin 600 000 U < 30 kg
Phenoxymethyl PO for 10 days 250-500mg qds for 10 days
penicillin 125mg qds X 10 if <30 kg
(Pen VK)
Erythromycin PO for 10 days Use same dose as above.
ethylsuccinate

Oral penicillin is less efficacious than Penicillin IMI

Anaphylaxis is extremely unusual
 Is it cost-effective to
administer penicillin for all
cases of suspected strep sore
throat?
• The estimated cost of preventing one case of
rheumatic fever by a single intramuscular
injection of penicillin is US$46
• Valve replacement surgery for 1 case of RHD
is at least US$15, 000
• Cardiac surgery only available in S Africa,
Ghana and Egypt
 Rheumatic Heart Disease:
SECONDARY PREVENTION

PICTURE TAKEN
OUT FOR SPACE
ISSUES
 Secondary Prevention
Stops sore throat, prevents recurrences of ARF and
aids in regression of RHD

Antibiotic Administration Dose

Benzathine Single IM injection 1.2 MU > 30kg
benzyl penicillin monthly 600 000 U < 30 kg
Phenoxymethyl BD PO daily 250-500mg bd
penicillin
(Pen VK)
Erythromycin BD po daily Use same dose as above.
ethylsuccinate

Oral penicillin has been shown to be less effective than

Penicillin IMI
Anaphylaxis is extremely unusual
THIS IS TOO
LATE
 During an episode of
ARF, valve changes can
be minor and are still able
to regress.

After recurrent episodes of

ARF, thickening of
subvalvar apparatus,
chordal thickening and
shortening and
progression to permanent
valve damage is evident.
Secondary prevention:
specifics
PENCILLIN

Secondary prophylaxis also reduces the

severity of RHD.
It is associated with regression of heart
disease in approximately 50-70% of those
with good adherence over a decade and
reduces mortality.
Route:
BPG is most effective when given as a
deep intramuscular injection.
 Secondary prevention:
Adherence
How can we reduce the pain associated with
IM Penicillin?

• Use a 23-gauge needle- deeper is better

• Local pressure to area for 10 secs
• Warm syringe to room temperature
• First allow alcohol to dry or use ethylchloride
spray
.
Ensuring that patients
understand their disease,
are informed regarding
their future and receive
secondary prophylaxis

EDUCATION
Health education is critical at all levels
Lack of parental awareness of the causes and
consequences of ARF/RHD is a key contributor to
poor adherence amongst children on long-term
prophylaxis.
 A.S.A.P. Programme for the
Control of RHD in Africa:
Focus areas for action
• Awareness raising: public, healthcare
workers
• Surveillance: incidence, prevalence,
temporal trends
• Advocacy: appropriate funding of the
treatment and prevention programmes
• Prevention: application of existing
knowledge in primary & secondary
prevention
 Summary
• Rheumatic heart disease is the only
truly preventable chronic heart condition
• Primary prevention:
– Penicillin for suspected strep sore
throat
• Secondary prevention
– Penicillin prophylaxis
• It is a legal requirement to notify
cases of acute rheumatic fever to
the local authority in South Africa