Rheumatic Fever and Rheumatic Heart Disease 2021

1. Rheumatic Fever
Etiology, Pathogenesis, Epidemiology,
Clinical Features, Complications, Management
Prognosis, Prevention
Prof. Imran Iqbal
Fellowship in Pediatric Neurology (Australia)
Prof of Paediatrics (2003-2018)
Prof of Pediatrics Emeritus, CHICH
Prof of Pediatrics, CIMS
Multan, Pakistan

2. Al Quran surah Al Shuaraa 26:88-89
On the day of judgement, wealth or family will not be of any benefit;
only the person who comes with a pure heart will be successful

3. Rheumatic Fever

4. Rheumatic Fever – Etiology
• Rheumatic Fever is a post-streptococcal infection
• It is seen within weeks after an episode of
Streptococcal Tonsillitis
• Repeated episodes of Rheumatic Fever are seen
after recurrent streptococcal infections
• There is inflammation of collagen tissues more
prominent in joints, heart and skin
• Permanent damage to heart valves can produce
Rheumatic Heart Disease

5. Rheumatic Fever - Pathogenesis
• Pathological lesions in Rheumatic Fever are produced by
Immune – mediated mechanisms
• Immune-mediated Cross-reactivity – antibodies produced
against Streptococcal antigens damage specific tissues in the
body because of antigenic similarity
• Immune-mediated Antigen binding – attachment of
streptococcal antigens to collagen tissues can produce an
immune response against these antigens in specific tissues

6. Epidemiology
• Incidence – 50 cases / 100,000 children / year
• Rheumatic Heart Disease is 50 % of all cardiac
diseases in children
• Rheumatic Heart Disease is a significant contributor
to child mortality in children
• Rheumatic Heart Disease is a leading cause of heart
disease among adults up to 40 years of age

7. Rheumatic Fever
Clinical Features

8. Case scenario
• A ten year old boy presents with low grade fever and joint
pains for the last 10 days. There is history of pain in elbows,
and pain and swelling in left ankle and left knee
• On examination, heart rate is 120/min, respiration is 20/min
and temperature is 99.5 F
• Child is not able to walk and his right ankle is swollen and
tender
• Cardiac examination reveals apex beat in 6th intercostal
space lateral to mid-clavicular line. Pan-systolic murmur is
heard at the apex which radiates to axilla
• Liver is palpable by 2 cm below the right costal margin
• What is your diagnosis ?

9. Case scenario
• A ten year old boy presents with low grade fever and joint pains for the last 10 days. There
is history of pain in elbows, and pain and swelling in left ankle and left knee
• On examination, heart rate is 120/min, respiration is 20/min and temperature is 99.5 F
• Child is not able to walk and his right ankle is swollen and tender
• Cardiac examination reveals apex beat in 6th intercostal space lateral to mid-clavicular line.
Pan-systolic murmur is heard at the apex which radiates to axilla
• Liver is palpable by 2 cm below the right costal margin
• What is your diagnosis ?
• Rheumatic Fever
• Mitral Regurgitation

10. Clinical Features
• Poly-arthiritis - (50%)
• Carditis (clinical or subclinical)- (50 – 75 %)
• Subcutaneous nodules (1 %)
• Erythema marginatum (1 %)
• Chorea (10 %)

11. Poly-arthiritis
• Seen in 50 % of cases
• Involves large joints
• Migratory in nature
• Pain and/or swelling of joints
• No residual damage

12. Rheumatic Carditis
(clinical / on echocardiography)
• Seen in 50 – 60 % of cases
• Pan-carditis – endocarditis, myocarditis, pericarditis
• Mitral and Aortic valves are affected
• Mitral regurgitation murmur indicates clinical Carditis
• Subclinical Carditis is detected on echocardiography
• Carditis may result in congestive cardiac failure
• Pericarditis causes pericardial friction rub
• Rheumatic Carditis can result in permanent valve damage,
more commonly after repeated infections

13. Subcutaneous Nodules
• Rarely seen
• Firm nodules on bony prominences
• Associated with severe carditis

14. Erythema Marginatum
• Rarely seen
• Serpiginous lesions
• Red margins with pale center
• Associated with severe carditis

15. Chorea
• Seen in 10 % of cases
• Emotional lability
• Late manifestation
• Occurs alone commonly
• Involuntary movements of peripheral limbs
• Semi-purposeful spontaneous movements

16. Investigations
• Inflammation –
• ESR - increased
• CBC - neutrophilia
• CRP - increased
• Carditis (clinical / on echocardiography) –
• ECG – prolonged P-R interval
• Echocardiography detects subclinical carditis
• Streptococcal infection (past or present) –
• Throat culture for beta-hemolytic Streptococci
• ASO (anti-streptolysin O) titre

17. Rheumatic Fever
Diagnosis

18. Revised Jones criteria – 2015 (Jones criteria - 1944)
2 major / 1 major and 2 minor + essential criteria
Major Criteria
• Poly-arthiritis
• Carditis ( clinical carditis / subclinical
carditis on echocardiography )
• Subcutaneous nodules
• Erythema marginatum
• Chorea
Minor Criteria
• Clinical
• Arthralgia
• Fever > 100.4F
• Laboratory
• Inflammatory markers
( ESR > 30 or CRP > 3.0 )
• Prolonged P-R interval
Essential Criteria
Positive Throat culture for beta-hemolytic Streptococci
OR
ASO (anti-streptolysin O) titre

19. Differential Diagnosis
• Arthritis –
• Pyogenic arthritis
• Reactive arthritis
• Juvenile idiopathic arthritis
• Carditis (clinical / on echocardiography) –
• Mitral valve prolapse
• Infective endocarditis
• Cardiomyopathy
• Chorea –
• Cerebral palsy
• Tic disorder

20. Complications
• Congestive Heart Failure (myocarditis, valve lesions)
• Rheumatic Heart disease –
• Mitral regurgitation
• Mitral stenosis
• Aortic regurgitation
• Aortic stenosis

21. Rheumatic Fever
Management

22. Management
• Rest – avoid activity to prevent worsening of carditis
• Antibiotics for Streptococcal infection –
- Benzathine (long-acting) Penicillin (IM)
OR
- Amoxicillin (Oral)
• Anti-inflammatory drugs –
- Aspirin for 4 – 6 weeks
- Corticosteroids for Congestive Heart Failure

23. Management of Complications
• Congestive Heart Failure –
diuretics
digoxin
ACE inhibitors
beta - blockers
• Chorea – sedatives

24. Prognosis
• Severity of Carditis
• Recurrences of streptococcal infection
• Adequate management of Rheumatic Fever
• Chemoprophylaxis for prevention

25. Prevention

26. Primary Prevention of Rheumatic Fever
• Prevent the first episode of Rheumatic Fever
• Treat acute tonsillitis promptly
• Give effective antibiotics (Amoxicillin) for 10 days to
eradicate Streptococcal throat infection

27. Secondary Prevention of Rheumatic Fever
• Prevent the recurrence of Rheumatic Fever
• Prevent further episodes of Streptococcal throat
infection (Acute Tonsillitis)
• Give Chemoprophylaxis – regular long-term
antibiotics for prevention of Streptococcal throat
infection (till 21 years of age)
• Benzathine Penicillin (IM) (long-acting) every 3-4
weeks
OR
• Amoxicillin (Oral) twice daily

28. Secondary Prevention of Rheumatic Fever
Duration of Chemoprophylaxis
• Rheumatic Fever – till 21 years of age
• Rheumatic Heart Disease – till 40 years of age

29. Rheumatic Heart Disease

30. Rheumatic Heart Disease – Etiology
• Rheumatic Heart Disease results from repeated
episodes of Rheumatic Fever associated with
Carditis which damage the heart valves
• Repeated episodes of Rheumatic Fever are seen
after recurrent streptococcal throat infections
• Damage to heart valves can produce Rheumatic
Heart Disease

31. Rheumatic Heart Disease
• Mitral regurgitation
• Mitral stenosis
• Aortic regurgitation
• Aortic stenosis

32. Mitral Regurgitation

33. Mitral Regurgitation
• Mitral regurgitation is the commonest valve lesion
in Rheumatic Heart Disease
• It may be asymptomatic or present with Congestive
Heart Failure

34. Mitral Regurgitation – clinical examination
• Cardiac examination reveals soft Pan-systolic
murmur at apex radiating to axilla

35. Mitral Regurgitation - management
• Echocardiography confirms the diagnosis
• Medical management
• Rheumatic Fever prophylaxis
• management of CHF
• Surgical management
• Mitral valve replacement with a prosthetic heart
valve

36. Mitral Stenosis

37. Mitral Stenosis
• Mitral stenosis can develop in damaged mitral valve after
years
• It may be associated with Mitral Regurgitation (MR+MS)
• It is seen in adolescents and adults
• It may present with dyspnea at exertion or orthopnea
(inability lie supine due to pulmonary edema)

38. Mitral Stenosis – clinical features
• Cardiac examination reveals loud S1 and rumbling
mid-diasystolic murmur at apex

39. Mitral Stenosis - management
• Echocardiography confirms the diagnosis
• Medical management
• Rheumatic Fever prophylaxis
• management of CHF
• Surgical management
• Mitral valve dilatation with a balloon catheter
• Mitral valve open repair
• Mitral valve replacement with a prosthetic heart valve

40. Aortic Regurgitation

41. Aortic Regurgitation
• Aortic Regurgitation is usually associated with mitral valve
disease
• It may be asymptomatic or present with Congestive Heart
Failure

42. Aortic Regurgitation – clinical examination
• Cardiac examination reveals soft diastolic murmur
at LUSB 3rd intercostal space

43. Aortic Regurgitation – management
• Echocardiography confirms the diagnosis
• Medical management
• Rheumatic Fever prophylaxis
• management of CHF
• Surgical management
• Aortic valve replacement with a prosthetic heart
valve

44. Aortic Stenosis

45. Aortic Stenosis
• Aortic Stenosis may be associated with Aortic Regurgitation
(AR+AS)
• It may present with palpitation and dyspnea at exertion

46. Aortic Stenosis – clinical features
• Cardiac examination reveals harsh systolic murmur
at aortic area (2nd intercostal space at right of
sternum)

47. Aortic Stenosis - management
• Echocardiography confirms the diagnosis
• Medical management
• Rheumatic Fever prophylaxis
• management of CHF
• Surgical management
• Aortic valve dilatation with a balloon catheter
• Aortic valve open repair
• Aortic valve replacement with an artificial heart valve

48. Questions ?

49. Medical Profession