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Rheumatic fever and
valvular vices


Aiyub medicine
Formation of the immune complexes
Proliferative arterial vasculities
Mucoide Intumescences
Fibrinoid intumescences
Fibrinoid necrosis of the
connective tissue
Inflammatory reaction stage
Rheumatic fever:
 Acute rheumatic fever: inflammatory disease with
devastating sequelae
 Link to pharyngeal infection wi...
Etiology
Immune response
Angina

Lymphatic
node
Lymphocyte
-B
Antistreptococal
antibody

Heart involvement

vegetation

Blood vesse...
Pathogenesis
􀂄 Group A strep pharyngeal infection precedes clinical
manifestations of ARF by 2 - 6 weeks
􀂄 Antibodies made...
CLINICAL FEATURES
Migratory
Polyarthritis
Myocarditis
Subcutaneous
nodules
Erythema
marginatum
Sydenham chorea
Arthritis
􀂄 Most common feature: present in 80% of
patients
􀂄 Painful, migratory, short duration, excellent
response of sa...
Subcutaneous Nodules
 Usually 0.5 - 2 cm long
 Firm, non-tender, isolated or in clusters
 Most common: along extensor s...
Erythema Marginatum
Present in 7% of patients
Highly specific to ARF
Cutaneous lesion:
Reddish pink border
Pale center
Rou...
Sydenham’s Chorea
􀂄 Extrapyramidal disorder
􀂄 Fast, clonic, involuntary movements
(especially face and
limbs)
􀂄 Muscular h...
Carditis










Most serious manifestation
May lead to death in acute phase or at later stage
Any cardiac tiss...
ACUTE:
-Inflammation
-Aschoff bodies
-Anitschkow cells
-Pancarditis
-Vegetations on
chordae tendinae at
leaflet junction

...
Acute Rheumatic vegetations:
Fish mouth Mitral stenosis:
Rheumatic endocarditis

 Diffuse
endocarditis
/valvulitis;
 Verucous acute
endocarditis;
 Fibroplastic
endocarditis;
 ...
Diffuse endocarditis
Recurrent verucous
endocarditis
McCallum plaques in the left
atrium
McCallum plaques
Aortic valve calcification
Left atrium dilation and left
ventricle hypertrophy
Shortening of the
tendineum cords

Fish mouth
mithral opening
Granulomatous stage of RF
Aschoff nodulesşi and Anitschkow cells
Rheumatic myocarditis

 Interstitial granulomatous
myocarditis;
 Exudative diffuse interstitial
myocarditis;
 Focal exu...
Interstitial granulomatous
myocarditis
Interstitial granulomatous myocarditis(H-E)
Exudative diffuse interstitial
myocarditis
Rheumatic pericarditis

EXUDATE:
 SEROUS;
 FIBRINOuS
 MIXED ;
Fibrinous pericaditis
Diagnosis: Jones Criteria
•
•
•
•
•

Major criteria
Arthritis
Carditis
Sydenham’s chorea
Erythema
marginatum
Subcutaneous
...
CONGENITAL HEART
DEFECTS
Faulty embryogenesis (week 3-8)
Usually MONO-morphic (i.e., SINGLE
lesion) (ASD, VSD, hypo-RV, ...
Incidence per Million Live
Births

%

4482

42

1043

10

Pulmonary stenosis

836 

8 

Patent ductus arteriosus

781 

7 ...
CONGENITAL HEART
DEFECTS
 LR SHUNTS: all “D’s” in their names
 NO cyanosis
 Pulmonary hypertension
 SIGNIFICANT pulmo...
LR
ASD
VSD
ASVD
PDA

NON CYANOTIC

IRREVERSIBLE
PULMONARY
HYPERTENSION IS
THE MOST FEARED
CONSEQUENCE
ASD
NOT patent foramen ovale
Usually asymptomatic until
adulthood
SECUNDUM (90%): Defective fossa
ovalis
PRIMUM (5%): ...
VSD
 By far, most common CHD defect
 Only 30% are isolated
 Often with TETRALOGY of FALLOT
 90% involve the membranous...
PDA
90% isolated
HARSH, machinery-like murmur
LR, possibly RL as pulmonary
hypertension approaches systemic
pressure
...
AVSD
Associated with defective,
inadequate AV valves
Can be partial, or
COMPLETE (ALL 4
CHAMBERS FREELY
COMMUNICATE)
RL
Tetralogy of Fallot
Transposition of great arteries
Truncus arteriosus
Total anomalous pulmonary
venous connection...
RL SHUNTS
 TETRALOGY of FALLOT most COMMON
 1) VSD, large
 2) OBSTRUCTION to RV flow
 3) Aorta OVERRIDES the VSD
 4)...
TGA (TRANSPOSITION
of GREAT ARTERIES)
NEEDS a SHUNT for
survival
PDA or PFO (65%),
“unstable” shunt
VSD (35%), “stable”...
TRUNCUS ARTERIOSIS
TRICUSPID ATRESIA
Hypoplastic RV
Needs a shunt, ASD, VSD, or
PDA
High mortality
Total Anomalous Pulmonary
Venous Connection (TAPVC)

PULMONARY VEINS do NOT go
into LA, but into L. innominate
v. or coro...
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  1. 1. Rheumatic fever and valvular vices  Aiyub medicine
  2. 2. Formation of the immune complexes
  3. 3. Proliferative arterial vasculities
  4. 4. Mucoide Intumescences
  5. 5. Fibrinoid intumescences
  6. 6. Fibrinoid necrosis of the connective tissue
  7. 7. Inflammatory reaction stage
  8. 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. 9. Etiology
  10. 10. Immune response Angina Lymphatic node Lymphocyte -B Antistreptococal antibody Heart involvement vegetation Blood vessel Aschoff Bodies Fibrinous pericarditis
  11. 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. 12. CLINICAL FEATURES Migratory Polyarthritis Myocarditis Subcutaneous nodules Erythema marginatum Sydenham chorea
  13. 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. 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. 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. 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. 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. 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. 19. Acute Rheumatic vegetations:
  20. 20. Fish mouth Mitral stenosis:
  21. 21. Rheumatic endocarditis  Diffuse endocarditis /valvulitis;  Verucous acute endocarditis;  Fibroplastic endocarditis;  Recurrent verucous endocarditis.
  22. 22. Diffuse endocarditis
  23. 23. Recurrent verucous endocarditis
  24. 24. McCallum plaques in the left atrium
  25. 25. McCallum plaques
  26. 26. Aortic valve calcification
  27. 27. Left atrium dilation and left ventricle hypertrophy
  28. 28. Shortening of the tendineum cords Fish mouth mithral opening
  29. 29. Granulomatous stage of RF Aschoff nodulesşi and Anitschkow cells
  30. 30. Rheumatic myocarditis  Interstitial granulomatous myocarditis;  Exudative diffuse interstitial myocarditis;  Focal exudative interstitial myocarditis.
  31. 31. Interstitial granulomatous myocarditis
  32. 32. Interstitial granulomatous myocarditis(H-E)
  33. 33. Exudative diffuse interstitial myocarditis
  34. 34. Rheumatic pericarditis EXUDATE:  SEROUS;  FIBRINOuS  MIXED ;
  35. 35. Fibrinous pericaditis
  36. 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. 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. 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. 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. 40. LR ASD VSD ASVD PDA NON CYANOTIC IRREVERSIBLE PULMONARY HYPERTENSION IS THE MOST FEARED CONSEQUENCE
  41. 41. 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
  42. 42. 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
  43. 43. 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?
  44. 44. AVSD Associated with defective, inadequate AV valves Can be partial, or COMPLETE (ALL 4 CHAMBERS FREELY COMMUNICATE)
  45. 45. RL Tetralogy of Fallot Transposition of great arteries Truncus arteriosus Total anomalous pulmonary venous connection Tricuspid atresia
  46. 46. 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
  47. 47. 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”
  48. 48. TRUNCUS ARTERIOSIS
  49. 49. TRICUSPID ATRESIA Hypoplastic RV Needs a shunt, ASD, VSD, or PDA High mortality
  50. 50. 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

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