1. Assessment of CVS & Murmurs
Rheumatic Fever
Assessment of CVS & Murmurs

2. Rheumatic Fever Definition:
Rheumatic fever is a systemic disease of childhood, often recurrent that follows Group A beta hemolytic streptococcal Pharyngitis * infection
It is a delayed Multisystem, Autoimmune, 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
Acute: Fever, Inflammed Skin, joint & heart
Chronic: Scarring of heart valves (mitral) dysfunction. 2

3. Introduction:
“The most important consequence of rheumatic fever is recurrent autoimmune inflammation of heart valves due to ‘GABH Strep’ causing scarring of valves leading to severe cardiac dysfunction decades later”
RHD – Mitral Stenosis. 3

4. Epidemiology Ages 5-18 yrs are most susceptible Rare <3 yrs
M:F equally except Sydenham’s chorea which is more common in girls
Common in 3rd world countries
Environmental factors--over crowding, poor sanitation, poverty,

5. Etiology: Genetic Susceptibility Environmental factor – GABH strep.
Autoimmunity – Autoantibodies ? . 5

6. Pathogenesis & Pathology
Delayed immune response to infection with group.A beta hemolytic streptococci of throat only.
After a latent period of 1-3 weeks, antibody induced immunological damage (Ashcoff nodules ) resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous tissue
-Basal gangliar lesions resulting in chorea

7. Group A Beta Hemolytic Streptococcus
Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24
Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis
Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity

8. Clinical Features: Fever 2-3 weeks following pharyngitis
Migratory polyarthritis of large joints
Pericardial friction rub
Weak heart sounds
Tachycardia, arrhythmias
Increased vulnerability to reactivation with subsequent pharyngitis.
Cumulative cardiac damage over decades 8

9. 1.Arthritis
Fleeting migratory polyarthritis, involving major joints (joints-knee, ankle, elbow & wrist )
Occur in 80% of cases
Involved joints are exquisitely tender
In children below 5 yrs arthritis usually mild but carditis more prominent
Arthritis do not progress to chronic disease

10. 2.Carditis
Manifest as pancarditis(endocarditis, myocarditis and pericarditis)
Occur in % 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)

11. 3.Sydenham Chorea 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

12. 4.Erythema Marginatum Occur in <5% of cases.
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

13. Erythema marginatum

14. 5.Subcutaneous nodules Occur in 10% of cases
Painless,pea-sized,palpable nodules
Mainly over extensor surfaces of joints,spine,scapulae & scalp
Associated with strong seropositivity
Always associated with severe carditis

15. Rheumatic Fever: Clinical Features
Polyarthritis – w/ low grade fever, large joints, (~ 80 % )
migratory - affects 1 at a time, no permanent dysfx.
Carditis - pericarditis, cardiomeagly, or valvulitis ( ~ 50%)
(valvulitis is the most serious manifestation.)
Chorea – late occurrence, months after ( ~ 10%)
infection, self-limiting, resolves in 1- 3 months.
Erythema Marginatum – “classic” truncal rash, ( ~ 5 %)
migratory - appears & disappears within hours.
(pink rash – irregular red edges – clear center)
Subcutaneous Nodules – occurs late ( months (~ 10 %)
after infection), painless small nodules over bony
prominences - elbows, knees, spine.

18. Other features (Minor features)
Fever-(upto 38.3 degree centigrade)
Arthralgia
Pallor
Anorexia
Loss of weight

19. Laboratory Investigations:
No specific laboratory investigations*
Throat culture-GABH streptococci - Cultures are usually negative.
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)
High Anti-DNAse B titres
High Acute phase reactants – 19

20. 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

21. Diagnosis Evidence of recent streptococcal infection can include:
Increased antistreptolysin O ( ASO ) or other streptococcal antibodies (anti-DNAse B)
Positive throat culture for Group A beta-hemolytic streptococci
Recent scarlet fever
Rheumatic fever is mainly a clinical diagnosis
No single diagnostic sign or specific laboratory test available for diagnosis
Diagnosis based on MODIFIED JONES CRITERIA

22. Jones clinical Criteria of Diagnosis:
Major Criteria
Migratory Polyarthritis
Carditis
Subcutaneous nodules
Erythema marginatum
Sydenham Chorea
Minor Criteria
Nonspecific symptoms
Fever
Arthralgia
High ESR / CRP
Prolonged PR Interval
Positive: 2 Major or 1 major + 2 minor
Following Group-A strep. pharyngitis. 22

24. Differential Diagnosis
Juvenile rheumatiod arthritis
Septic arthritis
Sickle-cell arthropathy
Kawasaki disease
Myocarditis
Scarlet fever
Leukemia

25. Heart Disease
Rheumatic Heart Disease – usually occurs years after initial attack. Mitral valve is more commonly involved than aortic valve. Classically, pts have mitral stenosis as a result of calcification.

26. Treatment Step I - 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)

27. Primary Prophylaxis
Timely diagnosis of GAS pharyngitis and appropriate treatment.
Treatment of choice is still Penicillin as all GAS is susceptible.
Treatment administered within 10 days of onset of illness has been shown to prevent ARF.
Alternatives – amoxicillin, erythromycin, 1st generation cephalosporin

28. STEP I: Primary Prevention of Rheumatic Fever
STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G U for patients Intramuscular Once kg (60 lb) U for patients >27 kg or
Penicillin V Children: 250 mg 2-3 times daily Oral d (phenoxymethyl penicillin) Adolescents and adults: mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 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 d (maximum 1 g/d)

29. Step II: Anti inflammatory treatment
Clinical condition Drugs

30. 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

31. Secondary Prophylaxis
Patients diagnosed with ARF need to undergo secondary prophylaxis to prevent relapses.
Prophylaxis regimens include oral Penicillin BID, Pen G IM monthly , or oral erythromycin BID.

32. STEP IV : Secondary Prevention of Rheumatic Fever
STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G U every 4 weeks* Intramuscular or
Penicillin V 250 mg twice daily Oral
Sulfadiazine g once daily for patients 27 kg Oral g once daily for patients >27 kg
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

33. Duration of Secondary Rheumatic Fever Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) 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 evidence.

34. Duration of treatment Rheumatic Fever without carditis
5yrs or until 21yo – whichever is longer
Rheumatic Fever with carditis but no valvular disease
10yrs or “well into adulthood” – whichever is longer
Rheumatic Fever with carditis and persistent valvular disease
At least 10yrs since last episode and at least until 40yo; sometimes lifelong

35. 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