Acute Rheumatic Fever &

Rheumatic Heart Disease

By: T/haimanot Fentie (Assistant Prof. of Clinical Pharmacy)

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 Introduction
 Group A beta-haemolytic streptococci (GAS)
 Humans are exposed to GAS in the environment
 Throat & skin are common sites of infection
 GAS infections usually resolve without treatment
 Acute Rheumatic Fever (ARF)
 Delayed autoimmune response following untreated GAS
infection
 Develops after GAS infection has resolved
 Commonly affects joints, heart, CNS & skin
 Most common between the ages of 5 & 15 years
 Can recur following subsequent untreated GAS infections

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 Rheumatic mitral valve
 Rheumatic Heart Disease (RHD)
 Residual damage to heart valves
following recurrent ARF
 Valves become scarred and thickened
 Blood leaks (flows backwards over the
valves)
 RHD is the most common cause of
heart disease in adolescents & young
adults in developing countries.

3 Rheumatic aortic valve

Risk Factors
 Risk factors ARF include
 Poverty
 Poor housing, overcrowded housing
 Reduced access to health care
 Risk factor for RHD
 Recurrent episodes of ARF
 Prevention
 ARF can be prevented by prompt treatment of GAS
infections with antibiotics (primary prophylaxis)
 RHD can be prevented by long-term prevention of ARF
recurrence with antibiotics (secondary prophylaxis)

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 Natural history of disease if adequate
secondary prevention is not given
Example age timeline (years)
5 10 13 15 16

Heart
Heart failure medications
starts to are needed.
develop. The Eventually,
Leaking valves: valve surgery
heart patient may
develop may be
chambers get needed.
stretched. symptoms
The valve is including
left damaged Blocked valves:
heart muscle breathlessness.
and scarred.
May cause struggles hard
ARF leakage then to move blood
episodes later, forwards
make blockage, or
valve(s) both.
inflamed.
Disease Progression & Intervention

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Acute Rheumatic Fever Diagnosis & Management
 ARF can be confirmed if certain signs and symptoms
from the Revised Jones Criteria are present.
MAJOR Criteria MINOR Criteria
Carditis Fever
Polyarthritis Arthralgia
Sydenham’s Chorea ↑ PR interval on ECG
Erythema marginatum ESR ≥30mm/hr or CRP ≥30mg/L
Subcutaneous nodules

 MAJOR Criteria - signs & symptoms more often

associated with ARF
 MINOR Criteria - signs & symptoms that help support
the diagnosis
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 WHO guidelines set the international standard for
diagnosis of ARF.
 Two MAJOR manifestations plus evidence of preceding
Group A streptococcal infection, or
 One MAJOR and two MINOR manifestations plus
evidence of preceding Group A streptococcal infection.
 Regional modified guidelines also exist.

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Diagnosis may be missed or delayed because:
 A combination of signs & symptoms is required.
 People with ARF do not always present to the
health system.
 Symptoms may not be considered serious
 Other commitments may take priority
 Transport to the health facility may be difficult
 Health staff may not recognize the signs &
symptoms.
 ARF may be confused with other illnesses.
 ARF symptoms may be confused with a sports
injury.
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Signs & Symptoms
 Arthritis
 Painful, swollen joints (commonly knees, ankles, wrists,
elbows)
 Very common, often the first symptom
 Usually ‘migratory’ – finishes in one joint, begins in
another
 Fever
 Carditis
 May present as a heart murmur
 Chest pain and/or difficulty breathing may be present in
more severe cases

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 Sydenham’s chorea
 Twitchy, jerking movements and muscle weakness (most
obvious in the face, hands and feet)
 May occur on both sides or only one side of body
 More common in teenagers and females (rare after age
20)
 May begin up to 3-4 months after the streptococcal throat
infection, and often occurs without other symptoms
 Usually resolves within 6 weeks (may last 6 months or
more)

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 Subcutaneous nodules
 Painless lumps on the outside surfaces of elbows, wrists,
knees, ankles in groups of 3-4 (up to 12)
 The skin is not red or inflamed
 Last 1-2 weeks (rarely more than 1 month)
 Nodules are more common when Carditis is also present
 Erythema marginatum
 Painless, flat pink patches on the skin that spread outward
in a circular pattern
 Usually occurs early, may last months, rarely lasts years
 Usually on the back or front of body, almost never on the
face
 Hard to see in dark-skinned people

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Treatment
 Treat the illness
 Benzathine penicillin G injection or
 Oral Penicillin for 10 days
 Relieve symptoms
 Bed rest
 Relief of arthritis, pain & fever (Paracetamol or Aspirin)
 Treat chorea (if severe)
 Anti-heart failure medication

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Management Plan
 A long-term plan should be established to prevent
recurrence of ARF and development of RHD.
 First dose of Benzathine penicillin G (secondary
prophylaxis)
 Baseline echocardiogram (if available)
 ARF alert on medical notes & computer systems (if
applicable)
 Education for person and family
 Refer to local health facility
 Dental examination
 Long-term secondary prophylaxis plan
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Management of Probable ARF
 Treat the symptoms
 Dose of Benzathine penicillin G as for ARF
 Echocardiogram (if available)
 Medical officer review after one month, and
 Repeat echocardiogram (if available)
 NOT ARF – cease Benzathine but monitor for ARF
symptoms
 ARF – continue Benzathine & manage as for ARF

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Secondary Prophylaxis to prevent recurrent
ARF
 Secondary prophylaxis is the long-term, regular
administration of antibiotics to
 Prevent Group A Streptococcal infections
 Prevent the development of ARF
 Prevent the development of RHD
 Reduce the severity of RHD
 Help reduce the risk of death from severe RHD

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Standard Treatment
 Benzathine penicillin G
 1,200,000 units for ALL people ≥27kg
 600,000 units for children <27kg
 Intramuscularly every 3 or 4 weeks
 Penicillin V
 Given if needles cannot be given due to excessive
bleeding
 250mg PO twice daily
 Erythromycin
 Given if Penicillin allergy has been confirmed
 250mg PO twice daily
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 Considerations
 When should secondary prophylaxis be considered?
 ARF confirmed by the Revised Jones Criteria
 RHD confirmed on echocardiogram
 ARF or RHD not confirmed, but considered ‘highly probable’
 Precautions
 Do not give Benzathine Penicillin G or Penicillin V if there is
a documented Penicillin allergy
 Drug reactions are rare
 Continue secondary prophylaxis during pregnancy
 Continue during anticoagulation (e.g. Warfarin)
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Guidelines for Secondary Prophylaxis
 The duration of secondary prophylaxis depends on
 Age at initial diagnosis
 Severity of disease
 Whether early carditis present
 Time (years) since last ARF
 Ongoing risk factors
 Compliance with treatment
Group Duration of secondary prophylaxis
ARF (no carditis) Minimum of 5 years after last ARF, or
Until age 18 years (whichever is longer)
Mild-moderate carditis Minimum of 10 years after last ARF, or
(or healed carditis) Until age 25 years (whichever is longer)
Severe RHD & after Surgery Continue for life
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Ceasing Secondary Prophylaxis
 Secondary Prophylaxis should only be ceased
following:
 Absence of ARF signs/symptoms for at least 5 years, and
 Medical Specialist review (Pediatrician/Cardiologist) and
 Echocardiogram to establish presence & severity of RHD
(if available)

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RHD Diagnosis & Management
 Heart valves are scarred due to healing process
following ARF.
 RHD is more likely to develop following ARF if
 The initial episode of ARF was severe
 The heart was affected with ARF
 ARF occurred at a young age
 There has been recurrent ARF

 50% of people with RHD do not remember having

ARF.

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Definitions
 Valve Regurgitation suggests that heart valves
 Are thickened and sticky against the walls of the heart
 Do not meet in the middle
 Leak (the blood flows backwards over the valve)
 Valve Stenosis suggests that heart valves
 Become stuck to each other
 Do not allow blood to flow through easily (restricted
forward flow)

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 Signs & Symptoms of RHD
 Symptoms of RHD may not show for many years.
 A murmur but no symptoms usually suggests mild-
moderate disease.
 Symptoms usually suggest moderate-severe disease.
 Symptoms depend upon the type and severity of the
valve lesion, and may include
 Breathlessness with exertion or when lying down flat
 Waking at night feeling breathless
 Feeling tired & General weakness
 Leg swelling (peripheral edema)
 Palpitations if atrial fibrillation develops
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 Heart valve involvement
 Mitral valve is affected in over 90% of cases of RHD.
 Mitral regurgitation is most commonly found in children
& adolescents
 Mitral stenosis represents longer term chronic disease,
commonly in adults
 Most common complication of mitral stenosis is atrial fibrillation
 Aortic valve is next most commonly affected
 Generally associated with disease of the mitral valve.
 Tends to develop as a long term complication of aortic
regurgitation
 Tricuspid & pulmonary valves are much less commonly
affected
 Usually affected in very severe RHD when all valves are
affected
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Clinical Examination
 Mitral regurgitation
 a pansystolic murmur heard loudest at the apex and
radiating laterally to the axilla
 Mitral stenosis
 a low-pitched, diastolic rumble heard best at the apex with
the bell of the stethoscope and with the person lying in the
left lateral position.
 Aortic regurgitation
 a diastolic blowing decrescendo murmur best heard at the
left sternal border with the person sitting up and leaning
forward in full expiration.
 Aortic stenosis
 a loud, low pitched mid-systolic ejection murmur best heard
in the aortic area, radiating to the neck.
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Investigations
 Electrocardiogram (ECG)
 Determine sinus rhythm
 Detect ventricular failure
 Chest X-ray (CXR)
 Determine size and placement of heart
 Detect cardiac failure (pulmonary congestion)
 Echocardiography
 Detect heart valve damage
 Estimate severity of disease
 Useful to compare results with later follow-up.

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 Key elements in RHD Management
 Secondary prophylaxis
 Function of secondary prophylaxis with established RHD
 Prevent further Group A Streptococcal infections
 Prevent the development of recurrent ARF
 Reduce the severity of existing RHD
 Help reduce the risk of death from severe RHD
 Effective baseline consultation, referral &
education
 Appropriate surgical intervention
 Special consideration in particular circumstances
(e.g. pregnancy)

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 Routine review & structured care planning
 Education
 Adherence to secondary prophylaxis
 Regular clinical assessment and follow-up
echocardiography
 Management of cardiac failure (diuretics and ACE
inhibitors)
 Management of atrial fibrillation (digoxin and anti-
coagulation)
 Dental care and Infective endocarditis prophylaxis
 Family planning referral (for women)
 Vaccination
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 Complications of RHD
 Atrial fibrillation
 Common in RHD
 Causes irregular heart rate / palpitations, blackouts etc, causes blood
clots in atrium which can then cause stroke
 Stroke
 Ischemic stroke (blood clot)
 Due to ineffective warfarin therapy of atrial fibrillation or metal valve
 Also can complicate infective endocarditis
 Hemorrhagic stroke (bleed into brain): Due to warfarin toxicity
 Heart failure
 Symptoms: SOB, swelling in the legs, cough, fatigue, weakness
 Infective endocarditis
 bacterial infection of heart valve – targets damaged valves
 Bacteria get into blood via mouth (especially when dental hygiene is
poor), open skin etc
 People at high risk receive endocarditis prophylaxis prior to surgical
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Summary
 RHD presents as damage to the heart valves
 The mitral valve is most commonly affected, followed
by Aortic, Pulmonary and Tricuspid
 RHD can be mild, moderate or severe
 RHD may be asymptomatic
 Management of RHD includes
 Treatment of cardiac and other symptoms
 Long-term secondary prophylaxis (to prevent recurrent
ARF)
 Regular medical and cardiology review
 Management of existing pregnancy
 Dental assessment, family planning referral

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