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Rheumatic Fever
AND RHD
Dr.Abdulelah
Mobeirek(FRCPc)
Rheumatic Fever
 Follows group A beta hemolytic
streptococcal throat infection
 It represents a delayed immune response to
infection with manifestations appearing
after a period of 2-4 weeks
 Age 5-15 yrs
 A multisystem disease
 Major effect on health is due to damage to
heart valvves
Rheumatic Fever
 A disease of poverty and low socioeconomic
status
 Rare in wealthy countries, due to improved
living conditions, less overcrowding, and better
hygiene with reduction in transmission of
GABHS
 Incidence: In Cambodia 2.2/1000 children,
Mozambique 2.3/1000, India 0.75/1000
Global Burden of RHD
 Total cases with RHD:20 Millions
 CHF: 3 Million
 Valve surgery required in 1 Million
 Annual incidence of RF: 0.5 Million,
nearly half develop carditis
 Estimated deaths from RHD: 230,000/YR
 Imposes a substantial burden on health
care systems with limited budgets
Pathologic Lesions
 Ashcoff nodules:
 Fibrinoid degeneration of
connective tissue, inflammatory
cells
Aschoff body in a patient with acute
rheumatic carditis
Clinical Features
 Onset of acute rheumatic fever is
typically characterized by an acute
febrile illness 2 to 4 weeks after an
episode of pharyngitis.
 Diagnosis is primarily clinical and is
based on a constellation of signs and
symptoms, which were initially
established as the Jones criteria
Modified Jones Criteria
Major manifestations
1. Carditis
2. Polyarthritis
3. Chorea
4. Erythema marginatum
5. Subcutaneous nodules
Modified Jones Criteria
Minor manifestations
1. Fever
2. Arthralgias
3. Previous H/O RF OR RHD
4. ↑CRP or ESR
5. Prolonged PR interval on ECG
Modified Jones Criteria
Evidence of antecedent GABHS
1. Positive throat culture or rapid
antigen test positive for GABHS
2. ↑ ASO Titer
Arthritis
 Most common feature: present in 80%
 Earliest manifestation of ARF
 Major joints: The knees and ankles,
shoulders, elbows
 “Migrating”, “Fleeting” polyarthritis
 Duration short < 1 week
 Responds well to Salicylates
 Does not progress to chronic disease
Sydenham Chorea
 Occur in 5-10% of cases
 Abrupt Purposeless involuantry
movements of muscles of face, neck,
trunk, and limbs.
 May appear even 6 months after the
attack of rheumatic fever
 Clinically manifest as-clumsiness,
deterioration of handwriting,emotional
lability or grimacing of face
13
Subcutaneous Nodules
 Occur in 10%
 Usually 0.5 – 2 cm long
 Firm non-tender
 Occur over extensor surfaces of joints, on
bony prominences, tendons, spine
 Short lived: last for few days
 Associated with severe carditis
Subcutaneous nodules
Erythema Marginatum
 Present in 5%
 Reddish border, pale center, round
or irregular serpiginous borders,
non-pruritic, transient rash
 Occurs on trunk, abdomen or
proximal limbs
 Associated with carditis
Subcutaneous nodule
Erythema marginatum
Erythematous patches
with central clearing
Carditis
 Occurs in40- 50% of cases
 Pancarditis
 Only manifestation of ARF that leaves
permanent damage
 Murmurs of MR or AR may occur in acute
stage while mitral stenosis occurs in late
stages
 Cardiomegaly and CHF may occur
Modified Jones Criteria for Diagnosis
of Acute Rheumatic Fever
A firm diagnosis requires
1) 2 Major manifestations or 1 Major and 2
Minor manifestations
and
2 ) Evidence of a recent streptococcal infection.
However, when chorea or carditis is clearly
present, evidence of an antecedent group A
streptococcal infection is not necessary.
Differential Diagnosis
 Juvenile rheumatiod arthritis
 Infective endocarditis
 Sickle cell arthropathy
 Lupus
 Myocarditis
 Reactive arthritis
 Leukemia
Investigations
 CBC, ESR, CRP
 Anti-streptococcal antibodies: ASO
titre
 Throat swab for culture
 ECG
 CXR
 ECHO
Treatment of ARF
 Salicylates : Aspirin
 75-100 mg /kg/day given as 4 divided
doses for 6 -8 weeks
 Attain a blood level 20-30 mg/dl
 Prednisolone:
 2mg/kg/day taper over 6 weeks
 Given when there is carditis
Treatment
 Bed rest
 Treat heart failure if present
 Valve replacement later in life once
symptoms develop or LV dysfunction
occurs from severe valve
regurgitation or valve stenosis
Secondary Prevention of Rheumatic Fever (Prevention of Recurrent
Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
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
Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever with carditis and 10 y since last episode
residual heart disease or until age 40y ,(which-
(persistent valvar disease*) ever is longer), sometimes
life long prophylaxis
Rhumatic fever with carditis 10 yrs or until age 21yrs
But no residual VHD (whichever is longer)
Rheumatic fever without carditis 5 y or until age 21 y,
( whichever is longer)
*
Chronic Rheumatic Heart Disease
 The normal MVA= 4-6 cm
 In severe ms <1
 High LAP
 The rise in LAP causes a similar rise
in pulmonary capillaries, veins and
artery
 Long Asymptomatic period after initial
attack of RF until onset of class 1 / 2
symptoms: 10 – 30 yrs ( latent period )
 Once symptoms develop there is another
plateau of 5 –10 yrs before onset of AF
 This followed by a period of 5-10 yrs until
onset of class III- Iv symptoms
 Dyspnea
 Fatigue
 Palpitation
 Hemoptysis (10%)
 Hoarseness ( Ortner’s syndrome)
 Dysphagia
 Storoke or peripheral
embolization
 Cyanosis (Mitral facies,malar
flush)
 Tapping apex ( S1)
 Parasternal heave
 Diastolic thrill
 Accentuated S1 , accentuated S2
 Opening snap
 Mid-diastolic rumble
 CXR
Straightening of the left heart border
Double density
Kerley B lines , CA in MV
 ECG: LAE, P Mitrale , RV dominance
 ECHO:
MVA, PAP
Mitral Stenosis
 B-Blockers ,CCB
 Digoxin ( AF )
 Warfarin
 Balloon Valvuloplasty
 Mitral valve replacement
BMV
 Asymptomatic
 Dyspnea , orthopnea, PND
 Displaced PMI, Thrill
 Soft S1,
 Pansystolic murmur
 Treatment is surgical
ECHO
 Water-hammer / collapsing pulse
 Wide pulse pressure
 Corrigan’s sign
 De Musset sign
 Muller sign
 Quincke’s pulse
 Hill’s sign
ECHO
Angina
Syncope
Dyspnea
 Arterial Pulse wave form : plateau
Small { Parvus }
Slow rise { Tardus }
 Sustained not displaced PMI
 Systolic thrill
 S4
 Late peaking of murmur
 Single S2 : Soft or absent
A2
 Paradoxical splitting of S2
Aortic Valve Disease
 Treatment is surgical: Aortic valve
Replacement

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Lecture 6-Rheumatic Heart Diseaseff.pptx

  • 2. Rheumatic Fever  Follows group A beta hemolytic streptococcal throat infection  It represents a delayed immune response to infection with manifestations appearing after a period of 2-4 weeks  Age 5-15 yrs  A multisystem disease  Major effect on health is due to damage to heart valvves
  • 3. Rheumatic Fever  A disease of poverty and low socioeconomic status  Rare in wealthy countries, due to improved living conditions, less overcrowding, and better hygiene with reduction in transmission of GABHS  Incidence: In Cambodia 2.2/1000 children, Mozambique 2.3/1000, India 0.75/1000
  • 4. Global Burden of RHD  Total cases with RHD:20 Millions  CHF: 3 Million  Valve surgery required in 1 Million  Annual incidence of RF: 0.5 Million, nearly half develop carditis  Estimated deaths from RHD: 230,000/YR  Imposes a substantial burden on health care systems with limited budgets
  • 5.
  • 6. Pathologic Lesions  Ashcoff nodules:  Fibrinoid degeneration of connective tissue, inflammatory cells
  • 7. Aschoff body in a patient with acute rheumatic carditis
  • 8. Clinical Features  Onset of acute rheumatic fever is typically characterized by an acute febrile illness 2 to 4 weeks after an episode of pharyngitis.  Diagnosis is primarily clinical and is based on a constellation of signs and symptoms, which were initially established as the Jones criteria
  • 9. Modified Jones Criteria Major manifestations 1. Carditis 2. Polyarthritis 3. Chorea 4. Erythema marginatum 5. Subcutaneous nodules
  • 10. Modified Jones Criteria Minor manifestations 1. Fever 2. Arthralgias 3. Previous H/O RF OR RHD 4. ↑CRP or ESR 5. Prolonged PR interval on ECG
  • 11. Modified Jones Criteria Evidence of antecedent GABHS 1. Positive throat culture or rapid antigen test positive for GABHS 2. ↑ ASO Titer
  • 12. Arthritis  Most common feature: present in 80%  Earliest manifestation of ARF  Major joints: The knees and ankles, shoulders, elbows  “Migrating”, “Fleeting” polyarthritis  Duration short < 1 week  Responds well to Salicylates  Does not progress to chronic disease
  • 13. Sydenham Chorea  Occur in 5-10% of cases  Abrupt Purposeless involuantry movements of muscles of face, neck, trunk, and limbs.  May appear even 6 months after the attack of rheumatic fever  Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face 13
  • 14. Subcutaneous Nodules  Occur in 10%  Usually 0.5 – 2 cm long  Firm non-tender  Occur over extensor surfaces of joints, on bony prominences, tendons, spine  Short lived: last for few days  Associated with severe carditis
  • 16. Erythema Marginatum  Present in 5%  Reddish border, pale center, round or irregular serpiginous borders, non-pruritic, transient rash  Occurs on trunk, abdomen or proximal limbs  Associated with carditis
  • 19. Carditis  Occurs in40- 50% of cases  Pancarditis  Only manifestation of ARF that leaves permanent damage  Murmurs of MR or AR may occur in acute stage while mitral stenosis occurs in late stages  Cardiomegaly and CHF may occur
  • 20. Modified Jones Criteria for Diagnosis of Acute Rheumatic Fever A firm diagnosis requires 1) 2 Major manifestations or 1 Major and 2 Minor manifestations and 2 ) Evidence of a recent streptococcal infection. However, when chorea or carditis is clearly present, evidence of an antecedent group A streptococcal infection is not necessary.
  • 21. Differential Diagnosis  Juvenile rheumatiod arthritis  Infective endocarditis  Sickle cell arthropathy  Lupus  Myocarditis  Reactive arthritis  Leukemia
  • 22. Investigations  CBC, ESR, CRP  Anti-streptococcal antibodies: ASO titre  Throat swab for culture  ECG  CXR  ECHO
  • 23. Treatment of ARF  Salicylates : Aspirin  75-100 mg /kg/day given as 4 divided doses for 6 -8 weeks  Attain a blood level 20-30 mg/dl  Prednisolone:  2mg/kg/day taper over 6 weeks  Given when there is carditis
  • 24. Treatment  Bed rest  Treat heart failure if present  Valve replacement later in life once symptoms develop or LV dysfunction occurs from severe valve regurgitation or valve stenosis
  • 25. Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) 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
  • 26. Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and 10 y since last episode residual heart disease or until age 40y ,(which- (persistent valvar disease*) ever is longer), sometimes life long prophylaxis Rhumatic fever with carditis 10 yrs or until age 21yrs But no residual VHD (whichever is longer) Rheumatic fever without carditis 5 y or until age 21 y, ( whichever is longer) *
  • 28.  The normal MVA= 4-6 cm  In severe ms <1  High LAP  The rise in LAP causes a similar rise in pulmonary capillaries, veins and artery
  • 29.  Long Asymptomatic period after initial attack of RF until onset of class 1 / 2 symptoms: 10 – 30 yrs ( latent period )  Once symptoms develop there is another plateau of 5 –10 yrs before onset of AF  This followed by a period of 5-10 yrs until onset of class III- Iv symptoms
  • 30.  Dyspnea  Fatigue  Palpitation  Hemoptysis (10%)  Hoarseness ( Ortner’s syndrome)  Dysphagia  Storoke or peripheral embolization
  • 31.  Cyanosis (Mitral facies,malar flush)  Tapping apex ( S1)  Parasternal heave  Diastolic thrill  Accentuated S1 , accentuated S2  Opening snap  Mid-diastolic rumble
  • 32.  CXR Straightening of the left heart border Double density Kerley B lines , CA in MV  ECG: LAE, P Mitrale , RV dominance  ECHO: MVA, PAP
  • 34.  B-Blockers ,CCB  Digoxin ( AF )  Warfarin  Balloon Valvuloplasty  Mitral valve replacement
  • 35. BMV
  • 36.  Asymptomatic  Dyspnea , orthopnea, PND  Displaced PMI, Thrill  Soft S1,  Pansystolic murmur  Treatment is surgical
  • 37. ECHO
  • 38.  Water-hammer / collapsing pulse  Wide pulse pressure  Corrigan’s sign  De Musset sign  Muller sign  Quincke’s pulse  Hill’s sign
  • 39. ECHO
  • 40.
  • 42.  Arterial Pulse wave form : plateau Small { Parvus } Slow rise { Tardus }  Sustained not displaced PMI  Systolic thrill  S4
  • 43.  Late peaking of murmur  Single S2 : Soft or absent A2  Paradoxical splitting of S2
  • 44. Aortic Valve Disease  Treatment is surgical: Aortic valve Replacement