1. Rheumatic fever and
valvular vices

Aiyub medicine

2. Formation of the immune complexes

3. Proliferative arterial vasculities

4. Mucoide Intumescences

5. Fibrinoid intumescences

6. Fibrinoid necrosis of the
connective tissue

7. Inflammatory reaction stage

8. Rheumatic fever:
 Acute rheumatic fever: inflammatory disease with
devastating sequelae
 Link to pharyngeal infection with group A beta hemolytic
streptocci
 Continues to be a problem worldwide:
- sporadic outbreaks in developed countries
- frequent occurrences in developing countries
 Still gaining understanding of etiology
- link between genetic predisposition and clinical
manifestations
 Best prevention still correct use of antibiotics

9. Etiology

10. Immune response
Angina
Lymphatic
node
Lymphocyte
-B
Antistreptococal
antibody
Heart involvement
vegetation
Blood vessel
Aschoff Bodies
Fibrinous
pericarditis

11. Pathogenesis
􀂄 Group A strep pharyngeal infection precedes clinical
manifestations of ARF by 2 - 6 weeks
􀂄 Antibodies made against group A strep cross-react
with human tissue
􀂄 heart valve and brain share common antigenic
sequences
with GAS bacteria
􀂄 theory of molecular mimicry
􀂄 Host immune responses may play a role in
determining who gets ARF following infection
􀂄 Virulent strains: rheumatogenic serotypes

12. CLINICAL FEATURES
Migratory
Polyarthritis
Myocarditis
Subcutaneous
nodules
Erythema
marginatum
Sydenham chorea

13. Arthritis
􀂄 Most common feature: present in 80% of
patients
􀂄 Painful, migratory, short duration, excellent
response of salicylates
􀂄 Usually >5 joints affected and large joints
preferred
􀂄 Knees, ankles, wrists, elbows, shoulders
􀂄 Small joints and cervical spine less
commonly involved

14. Subcutaneous Nodules
 Usually 0.5 - 2 cm long
 Firm, non-tender, isolated or in clusters
 Most common: along extensor surfaces of
joint
 Knees, elbows, wrists
 Also: on bony prominences, tendons, dorsi
of feet, occiput or cervical spine
 Last a few days only
 Occur in 9 - 20% of cases
 Often associated with carditis

15. Erythema Marginatum
Present in 7% of patients
Highly specific to ARF
Cutaneous lesion:
Reddish pink border
Pale center
Round or irregular shape
Often on trunk, abdomen, inner arms, or
thighs
 Highly suggestive of carditis








16. Sydenham’s Chorea
􀂄 Extrapyramidal disorder
􀂄 Fast, clonic, involuntary movements
(especially face and
limbs)
􀂄 Muscular hypotonus
􀂄 Emotional lability
􀂄 First sign: difficulty walking, talking, writing
􀂄 Usually a late manifestation: months after
infection
􀂄 Often the only manifestation of ARF

17. Carditis









Most serious manifestation
May lead to death in acute phase or at later stage
Any cardiac tissue may be affected
Valvular lesion most common: mitral and aortic
Seldom see isolated pericarditis or myocarditis
Mitral and aortic regurgitation most common
Apical systolic and basal diastolic murmurs
Pericarditis usually asymptomatic
Occasionally causes chest pain, friction rubs or distant
heart sounds

18. ACUTE:
-Inflammation
-Aschoff bodies
-Anitschkow cells
-Pancarditis
-Vegetations on
chordae tendinae at
leaflet junction
CHRONIC:
THICKENED VALVES
COMMISURAL FUSION
THICK, SHORT,
CHORDAE TENDINAE

19. Acute Rheumatic vegetations:

20. Fish mouth Mitral stenosis:

21. Rheumatic endocarditis
 Diffuse
endocarditis
/valvulitis;
 Verucous acute
endocarditis;
 Fibroplastic
endocarditis;
 Recurrent
verucous
endocarditis.

22. Diffuse endocarditis

23. Recurrent verucous
endocarditis

24. McCallum plaques in the left
atrium

25. McCallum plaques

26. Aortic valve calcification

27. Left atrium dilation and left
ventricle hypertrophy

28. Shortening of the
tendineum cords
Fish mouth
mithral opening

29. Granulomatous stage of RF
Aschoff nodulesşi and Anitschkow cells

30. Rheumatic myocarditis
 Interstitial granulomatous
myocarditis;
 Exudative diffuse interstitial
myocarditis;
 Focal exudative interstitial
myocarditis.

31. Interstitial granulomatous
myocarditis

32. Interstitial granulomatous myocarditis(H-E)

33. Exudative diffuse interstitial
myocarditis

34. Rheumatic pericarditis
EXUDATE:
 SEROUS;
 FIBRINOuS
 MIXED ;

35. Fibrinous pericaditis

36. Diagnosis: Jones Criteria
•
•
•
•
•
Major criteria
Arthritis
Carditis
Sydenham’s chorea
Erythema
marginatum
Subcutaneous
nodues
Minor criteria
•Fever
•Arthralgia
•Elevated c-reactive
protein or
•Erythrocyte
sedimentation rate
•Prolonged PR
interval on EKG

37. CONGENITAL HEART
DEFECTS
Faulty embryogenesis (week 3-8)
Usually MONO-morphic (i.e., SINGLE
lesion) (ASD, VSD, hypo-RV, hypo-LV)
May not be evident until adult life
(Coarctation, ASD)
Overall incidence 1% of USA births
INCREASED simple early detection via
non invasive methods, e.g., US, MRI,
CT, etc.

38. Incidence per Million Live
Births
%
4482
42
1043
10
Pulmonary stenosis
836
8
Patent ductus arteriosus
781
7
Tetralogy of Fallot
577
5
Coarctation of aorta
492
5
Atrioventricular septal defect
Aortic stenosis
396
4
388
4
Transposition of great arteries
Truncus arteriosus
Total anomalous pulmonary venous connection
Tricuspid atresia
388
4
136
1
120
1
Malformation
Ventricular septal defect
Atrial septal defect

39. CONGENITAL HEART
DEFECTS
 LR SHUNTS: all “D’s” in their names
 NO cyanosis
 Pulmonary hypertension
 SIGNIFICANT pulmonary hypertension is
IRREVERSIBLE
 RL SHUNTS: all “T’s” in their names
 CYANOSIS
 VENOUS EMBOLI become SYSTEMIC
 OBSTRUCTIONS

40. LR
ASD
VSD
ASVD
PDA
NON CYANOTIC
IRREVERSIBLE
PULMONARY
HYPERTENSION IS
THE MOST FEARED
CONSEQUENCE

42. ASD
NOT patent foramen ovale
Usually asymptomatic until
adulthood
SECUNDUM (90%): Defective fossa
ovalis
PRIMUM (5%): Next to AV valves,
mitral cleft
SINUS VENOSUS (5%): Next to
SVC with anomalous pulmonary
veins draining to SVC or RA

43. VSD
 By far, most common CHD defect
 Only 30% are isolated
 Often with TETRALOGY of FALLOT
 90% involve the membranous septum
 If muscular septum is involved, likely to
have multiple holes
 SMALL ones often close spontaneously
 LARGE ones progress to pulmonary
hypertension

45. PDA
90% isolated
HARSH, machinery-like murmur
LR, possibly RL as pulmonary
hypertension approaches systemic
pressure
Closing the defect may be life
saving
Keeping it open may be life saving
(Prostaglandin E). Why?

46. AVSD
Associated with defective,
inadequate AV valves
Can be partial, or
COMPLETE (ALL 4
CHAMBERS FREELY
COMMUNICATE)

47. RL
Tetralogy of Fallot
Transposition of great arteries
Truncus arteriosus
Total anomalous pulmonary
venous connection
Tricuspid atresia

48. RL SHUNTS
 TETRALOGY of FALLOT most COMMON
 1) VSD, large
 2) OBSTRUCTION to RV flow
 3) Aorta OVERRIDES the VSD
 4) RVH
 SURVIVAL DEPENDS on SEVERITY of
SUBPULMONIC STENOSIS
 Can be a “PINK” tetrology if pulmonic
obstruction is small, but the greater the obstruction,
the greater is the RL shunt

50. TGA (TRANSPOSITION
of GREAT ARTERIES)
NEEDS a SHUNT for
survival
PDA or PFO (65%),
“unstable” shunt
VSD (35%), “stable”
shunt
RV>LV in thickness
Fatal in first few
months
Surgical “switching”

51. TRUNCUS ARTERIOSIS

52. TRICUSPID ATRESIA
Hypoplastic RV
Needs a shunt, ASD, VSD, or
PDA
High mortality

53. Total Anomalous Pulmonary
Venous Connection (TAPVC)
PULMONARY VEINS do NOT go
into LA, but into L. innominate
v. or coronary sinus
Needs a PFO or a VSD
HYPOPLASTIC LA