RHEUMATIC FEVER

• Is an inflammatory disease occurring in

response to a group A beta-haemolytic
streptococcal infection.
• Affects even the very young beginning
from the age of three.
• Peak incidence occurs between 6-15
years.
• First attacks are uncommon after
adolescence.
 AETIOLOGY
• The disease is a sequel to group A beta
haemolytic streptococcal pharyngitis.
• Development of Rheumatic fever
depends on :
1. Individual susceptibility
2. The promptness or otherwise
with
which effective treatment is
given
3. Rheumatogenicity of the
PATHOGENESIS/ PATHOLOGY
• After infection of the throat with streptococci.
The host has an autoimmune response to
epitopes in the bacterium that are
immunologically cross- reactive with host
tissues in synovium, heart, brain and skin.
• This causes proliferative and exudative
inflammatory reactions in these tissues.
• Effusions into joints, pericardial or pleural
spaces result from exudative reactions.
• Proliferative process is typified by the
Ascoff nodules most abundant in the
left atrial myocardium.
• Clusters of large multinucleated cells in
a mass of fragmented swollen
collagen, (Granulomatous collections of
leukocytes, myocytes, and interstitial
collagen)
• The cardiac lesion comprise fibrinous
pericarditis, myocarditis and
endocarditis (pancarditis).
• Endocarditis is most conspicuous in
• Varying degrees of cardiac enlargement may
occur at this acute stage.
• Mitral regurgitation is common.
• Later on as healing progresses, the
pericarditis resolves completely.
• Myocardium may become scarred.
• The valve edges become thickened and rolled
up.
• The papillary muscles become shortened thus
preventing full approximation of valve leaflet
• Commissural adhesions may
subsequently lead on to valve stenosis.
 CLINICAL FEATURES
• Involve a pharyngeal infection followed
2-6 weeks later by -a polyarthritis
- fever,
-subcutaneous nodules,
- erythema marginatum,
-tachycardia and signs of heart
failure.
- May present with chorea
 DIAGNOSIS
• Is based on Jones criteria which has major and minor manifestations.
• Need 2 major or one major and 2 minor.
• Major criteria:
- pancarditis,
- polyarthritis,
- chorea,
- erythema marginatum and
- subcutaneous nodules.
• Minor criteria:
-Arthralgia,
- fever,
- increased P-R interval
-, raised ESR,
- increased C- reactive protein,
- history of rheumatic fever,
- raised ASO titres and
- positive throat culture, Leukocytosis
• Can make a diagnosis if there is only chorea
or pancarditis.
INVESTIGATIONS
• Throat swab culture for streptococci
• ASO titres if exceeds 200i.u.
• ECG prolonged PR interval.
• Raised ESR >120mm/hr
• C- reactive protein >2g/dl.
• FBC- leucocytosis
 Differential diagnosis
• Sickle cell disease
• Septicaemia with infective endocarditis
• Juvenile rheumatoid arthritis.
TREATMENT AND PROGNOSIS
Treatment should be aimed at
• 1. Eradicating any streptococci patient
is still
harbouring.
• 2.Suppress the inflammatory reactions
• 3. Reduce cardiac workload
• These can be achieved through bed
rest, administration of penicillin and
salicylate or steroids.
• Procaine penicillin 0.3-0.6 mega units for 10
days or Benzathine penicillin 0.6-1.2 mega
units single injection.
• Aspirin 80-100mg/kg per day
• Oral Prednisolone 1-2mg/kg per day is
indicated if patient has signs of severe carditis
like extreme tarchycardia, pansystolic murmur
and rapidly progressive valve damage or
heart failure.
• Steroids may be given with salicylate until
• Usual anti-failure measures are applied
until heart failure is controlled.
• Sedating drugs such as
chlorpromazine, haloperidol and
phenobarbital should be given in severe
attacks of chorea.
• About 75% of children subsequently
develop chronic rheumatic heart
disease.
 PREVENTION
• Prevention of Rheumatic fever and
Rheumatic heart disease are interwoven.
• Primary prevention by preventing
first attack can be achieved through:
1. Prevention of overcrowding
2. Improved nutrition.
3. Health education and provision of
essential health facilities.
• 4. Prompt and effective treatment of
streptococcal infection.
• A daily dose of oral penicillin or
procaine penicillin 0.3-0.6 mega units
daily I.M. Or a single dose of
Benzathine penicillin 0.6-1.2 mega units
will achieve the same results.
 Secondary prevention:
• Aim is to prevent a recurrence of acute
rheumatic fever.
• The main thrust is prevention of
streptococcal infections by long term
antibiotic prophylaxis.
• E.g. Benzathine penicillin once monthly.
• Pen V 250mg BD.
• If allergic to penicillin give Erythromycin
250 BD daily.
• The prophylaxis can be stopped 5 years
after the last attack of Rheumatic fever
or when patient reach the age of 18
years.
• END OF PART ONE.
RHEUMATIC HEART DISEASE (
RHD)
• Sequel of Rheumatic fever.
• Mitral valve is involved in practically
every child who has RHD. Other valves
affected are the aortic and tricuspid..
Pulmonary valve involvement is very
rare .
• Majority of patients have mitral valve
regurgitation.
• Others have both mitral valve
regurgitation and stenosis.
• Pure mitral stenosis is not very
common. But it has been reported in a
number of tropical countries.
• It is believed to result from repeated
attacks of acute rheumatic fever and
 CLINICAL FEATURES
• Majority are brought with features of
cardiac failure.
• A few because of excessive precordial
activity.
• The signs are those of underlying valve
defect plus heart failure if such a
complication exist.
 INVESTIGATIONS
• CxR
• ECG
• ECHO
 TREATMENT
• Medical treatment involves taking
anti-failure measures.
• Treat infective endocarditis and use
prophylaxis before certain surgical
procedures
• Definitive treatment is surgery to
replace the damaged valves
 Cardiomyopathies
• This is a disease of the myocardium.
There is damage to heart muscle.
• There are 3 types:
• 1 dilated cardiomyopathy
• 2. Hypertrophic
• 3. Restrictive or obliterative
• Dilated cardiomyopathy (DCM)
• Involves dysfunction of the
cardiomyocytes resulting in dilatation and
impaired function of left or right ventricle.
Causes:
• Most cases are idiopathic.
• Genetic diseases e.g. mitochondrial
abnormalities, carnitine defiency, muscular
dystrophy, Pompe’s disease.
• Nutrition problems .e.g. selenium,
thiamine and calcium defiencies, Iron
overload, severe aneamia
• Infections: Post viral myocarditis (
• Toxins : mainly drugs like Doxorubicin,
Cyclophosphamide, adriamycin, AZT

• Clinical Features
Heart failure, arrhythmias, embolism
• Investigations:
CXR shows cardiomegaly
ECG shows LVH, non specific T wave
abnormalities
Echo cardiogram shows atrial and
ventricular dilatation poorly contracting.
Reduced ejection fraction, mitral and
tricuspid regurgitation
• Metabolic screen
• Cardiac biopsy
• Treatment:
• cardiac failure and anticoagulants(
aspirin or warfarin)
• Cardiac transplant
Hypertrophic cardiomyopathy
• Characterized by hypertrophy of the
interventricular septum and left
ventricular .
• Left ventricular outflow tract obstruction
may occur.
• Causes:
Neurofibromatosis, Autosomal
dominant in some cases.
Premature infants receiving steroid for
• Clinical features:
Syncope, angina, arrhythmias, sudden
death, family history of sudden death.

• Investigation:
• CXR, ECG, Echocardiogram
• Treatment:
• Bita blockers ( propranolol)
• Amiodarone
• Cardiac pacing
• Surgical resection of the septum if
significant outflow obstruction
• Restrictive cardiomyopathy
• There is a rigid noncompliant ventricle
• Starts with cell death - fibrosis- rigid,
noncompliant heart.
• Caused by scleroderma,
hemochromatosis, Gauchers and
sarcoidosis.
• Treatment: No medical treatment
• Heart transplant
 Congestive heart failure
• Cardiac output insufficient to meet systemic
demand.
• Can be right, left or both sided failure
Causes:
• Valve disease,
• anemia,
• endocarditis,
• Cardiomyopathy
• , thyrotoxicosis,
• hypertension,
• rheumatic fever,
 Signs & symptoms are
multiorgan
• Left sided failure leads to pulmonary
oedema, dyspnea on exertion,
orthopnea, paroxysmal nocturnal
dyspnea
• Mitral regurgitation worsens the
reduction in cardiac output.
• Renal hypoperfusion- activation of
renin-angiotensin axis- increased
aldosterone- increased sodium
retention- increased water retention(
• Sweating Signs & symptoms
• Poor feeding
• Failure to thrive
• Shortness of breath
• Recurrent chest infections
• Abdominal pain (big liver)
• Collapse/shock
• Tachypnoea, intercostal and subcostal recession
• Signs Tachycardia
• Cardiomegaly
• Hepatomegally
• Gallop rhythm/murmur/muffled heart sounds
• Central cyanosis
• Cool peripheries
• In right sided failure, lack of forward
venous flow leads to liver engorgement
and lower extremity oedema
• In children they may present with only
respiratory distress, tarchycardia and
hepatomegaly

Treatment:
• Reduce physical activity
• Prop up in bed
• In right sided failure, lack of forward
venous flow leads to liver engorgement
and lower extremity oedema
• In children they may present with only
respiratory distress, tarchycardia and
hepatomegaly

Treatment:
• Reduce physical activity
• Prop up in bed
• O2
• Digoxin
• Laxis
• Slow k
• ACE inhibitors, spironolactone, Bita blockers
have been shown to improve survival.
• When giving ACE and spironolactone do not
give slow K.