RHEUMATIC FEVER and RHD 2 FOR MLS.ppt rule

This slide contains information regarding Rheumatic fever and other

1. 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.
•Slightly more common in girl than boys.

2. 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 infecting
streptococci

3. 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.

4. •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 the left
ventricle and mitral valves.
Mitral valves becomes roughened and oedematous.

5. •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
during ventricular systole.

6. •Commissural adhesions may subsequently
lead on to valve stenosis.

7. CLINICAL FEATURES
•Involve a pharyngeal infection followed 2-6
weeks later by a polyarthritis, fever,
subcutaneous nodules, erythema
marginatum, tarchyardia and signs of heart
failure. May present with chorea
•Exact symptoms depend on the organs
involved.

8. 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.

9. •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.

10. Differential diagnosis
•Sickle cell disease
•Septicaemia with infective endocarditis
•Juvenile rheumatoid arthritis.

11. 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.

12. •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 signs
have subsided.

13. •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.
•

14. PREVENTION
•Prevention of Rheumatic fever and
Rheumatic heart disease are interwoven.
•Primary preventionby preventing first
attack can be achieved through:
1. Prevention of overcrowding
2. Improved nutrition.
3. Health education and provision of
essential health facilities.

15. •4. Prompt and effective treatment of
streptococcal infection.
•A 7-10 day course 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.

16. 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.

17. •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.

18. •END OF PART ONE.

19. RHEUMATIC HEART
DISEASE ( RHD)
•Sequel of Rheumatic fever.
•No complications on all other parts of the
body affected.
•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 .

20. •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 associated intense host
reaction.

21. 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.

22. INVESTIGATIONS
•CxR
•ECG
•ECHO

23. 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

24. 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

25. •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(Acid alfa glucosidase GAA deficient disease)
•Nutrition problems .e.g. selenium, thiamine and
calcium defiencies, Iron overload, severe aneamia
•Infections: Post viral myocarditis ( coxsackie,
echovirus), sepsis, diphtheria, rheumatic fever, HIV

26. •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

27. •Metabolic screen
•Cardiac biopsy
•Treatment:
•cardiac failure and anticoagulants( aspirin
or warfarin)
•Cardiac transplant

28. 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 lung
disease( transient)

29. •Clinical features:
Syncope, angina, arrhythmias, sudden death,
family history of sudden death.
•Investigation:
•CXR, ECG, Echocardiogram

30. •Treatment:
•Bita blockers ( propranolol)
•Amiodarone –antiarrhythmias K+/NA+
channel blocker
•Cardiac pacing
•Surgical resection of the septum if
significant outflow obstruction

31. •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

32. 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, congenital heart diseases.

33. 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(
increased in total body fluid load)

34. •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

35. •Digoxin
•Laxis
•Slow k
•ACE inhibitors, spironolactone, Beta blockers
have been shown to improve survival.
•When giving ACE and spironolactone do not give
slow K.