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Rheumatic Heart Disease
DR. DIBBENDHU KHANRA
DM CARDIOLOGY
AIIMS RISHIKESH
Pathology
• Fibrinous necrosis: exudative
(bread and butter appearance)
• Proliferative (Aschoff nodules/Antishkow/
caterpiller cells) – McCallum patch
• Healing and fibrosis (milk spots)
Series of Events
SORE
THROAT
(GABHS)
ACUTE
RHEUMATIC
FEVER
RHEUMATIC
HEART
DISEASE
ACUTE
RHEUMATIC
ACTIVITY
COMPLICATIONS
PREVALENCE
5-15 YRS >15 YRS
RF 0.75/1000 (Mishra) 0.4/1000 (Verma)
RHD 4.5/1000 (Lalchandani) 4.5/1000 (Lalchandani)
5-15 YRS All age
Low risk pop <2/1 lac <1/1000
High risk pop >2/1 lac >1/1000
SORE
THROAT
(GABHS)
MODIFIED CENTOR CRITERIA
1. AGE 5-15 YRS
2. HIGH GRADE FEVER
3. ANT CERVICAL LN
4. TONSILLAR EXUDATE
5. COUGH ABSENT
0-1 +: NO AB*
2-3 +: THROAT SWAB
RAPID AG DET
AB IF POSITIVE
4-5 +: AB
SORE THROAT to ARF: 3% (epidemic) 0.3% (endemic)
THROAT SWAB: YIELD 5-10%
*AMOXICILLIN/ AZITHROMYCIN
GABHS Sore Throat
Once RF after sore throat, 50% chance of RF recurrence after another sore throat
SN 77
SP 97
ARF: Modified Jones Criteria
MAJOR
PANCARDITIS
MIGRATORY
ARTHRITIS
CHOREA
SC NODULES
ERYTHEMA
MARGINATUM
MINOR
HIGH FEVER
ARTHRALGIA
ESR>30
CRP>3
PROLONGED PR
GAS INFECTION
RAPID AG TEST
THROAT SWAB
ASO
ANTI-DNAase
H/O ARF IN RHD
BLAND & JONES 30%
PADMAVATI 30%
PAUL WOOD 60%
SB ROY 60%
Jones criteria exempted
MS
Chorea
INDIAN VS WESTERN
WESTERN
(BLAND & JONES)
INDIAN
(PADMAVATI, SANYAL)
COMMENTS
CARDITIS 2/3 1/3 LESS IN INDIANS
ARTHRITIS 1/3 2/3 ARTHALGIA > ARTHRITIS
CHOREA 50% 10% UNCOMMON
SCN 5% 1% UNCOMMON
EM 5% - RARE
PANCARDITIS
ENDOCARDITIS
Regurgitations
MC-MR
PSM
Careycoumb
EDM (AR)
Long PR/ AF
MYOCARDITIS
Cardiomegaly
S3
Parchment carditis
Vs viral carditis:
No murmer
Symp improves
PERICARDITIS
Rub
Effusion
Rare w/o
endocarditis
VALVULAR INV IN ARF
VALVE INVOLVEMENT
MITRAL 75%
MITRAL + AORTIC 20%
AORTIC 3%
TRICUSPID 2%
PULMONARY -
FATE OF MR/ PSM
1/3 DISAPPEARS
1/3 SAME
1/3 PROGRESSES
VALVULAR LOAD
SVC 5 PV 10
RA 5 LA 10
RV 25/0-5 LV 120/0-10
PA 25/10 AO 120/80
TCV 20
mmHg
MV 110
mmHg
PV 5
mmHg
AV 70
mmHg
TCVA 2
mmHg/ cm2
MVA 40
mmHg/cm2
PVA 1
mmHg/ cm2
AVA 25
mmHg/cm2
TCVA 8-10cm2 MVA 4-6cm2
PVA 2-4cm2 AVA 2-4cm2
Carditis
• Acute: Dyspnea at rest
• Subacute: DOE
• Insidious: no symps, murmer+
• Subclinical: no symp, no murmer, echo+
SEVERITY OF CARDITIS
Severity Cl/F
Mild NYHA 2-3
Mod NYHA 3-4 NO CARDIOMEGALY
Severe NYHA 3-4 CARDIOMEGALY
PERICARDIAL EFFUSION
SC NODULES
JACCOUDS ARTHOPATHY
Fulminant NYHA 3-4 CARDIOMEGALY
LV FUNCTION DEPRESSED
SUB
CLINICAL
CARDITIS
CONSEQUENCE OF CARDITIS
SANYAL ET AL
ARF
CARDITIS (60%) NO CARDITIS (40%)
2/3
RHD
(40%)
1/10
RHD
(4%)
Which murmur disappears?
• No CHF/ cardiomegaly
• Low grade PSM
• Single valve
• Early penicillin
• First attack
• Male
Which ARFwill lead to RHD?
• CARDIOMEGALY / CHF
• >GR2 EDM
• OVERCROWDING
• MALNUTRITION
• NO PEN PROPHX
• RECURRENT ATTACK
HOW MANY DIES?
BLAND & JONES
10% IN 10 YRS
20% IN 20 YRS
TOTAL
30% (1/3) IN 3 YRS
CHF
CARDIOMEGALY
50% DIES
Arthralgia/ arthritis!
Fever and joint pain
1 week after sore throat
Migratory
Stereotypic
Large joints
No small joints
NOT INVOLVED: TMJ, STERNOCLAV JOINT, ATLANTOAXIAL JOINT
Back rarely involved
Severely painful/ tender/ swollen/ red/ hot
L/O function
Symp> signs
Each joint Lasts for 1 week
Dramatic response to salicylates
Total episode resolves in 4 week
No residual deformity
Arthralgia/ arthritis!DD
VS PSRA
1. Short incubation period
2. Affects small joint
3. No response to salicylates
4. Often renal involvement
5. No carditis
TO RX PENICILLIN PROPHYLAXIS FOR 1 YEAR
VS JIA
1. MP rash incl face
2. Back inv
3. Small joints inv
4. LN
5. LFT deranged
Signifies ARA
Non-erosive
Can involve lower limbs
Subcutaneous nodules
Extensor surface
Elbow forearm
Knee joints knee
Severe carditis/ active carditis
Painless
Freely mobile
Not attached to tendon
Good response to salicylate
DD
Rheumatoid nodules/JIA
-Larger
-Painful
-Attached to tendons
Osler’s node
Painful
Pulp of fingers
Smaller
Janeway lesion
Macular
Palm soles
blanching
Erythema marginatum
In crops
Painless
Axilla/ thighs+
Never on face
Annular
Evanescent
Itchy
Rare to find in indians
Carditis+
No response to salicylates
DD
Scarlet fever
Scalding
Sydenhams Chorea
Late manifestation
Never with arthritis
Carditis+
More in females
Rare in postpubertal boys
Resolves in 6m in 75% cases
Jerky speech
Pronator sign
Jack in the box
Worms in the tongue
Milkmaids grip
Spoon-like configuration
Pendular knee jerk
OCD
Poor school performance
Things fall from hands
No sensory or motor inv
Sydenhams Chorea/ DD
PANDAS
Early after sore throat
OCD
Tics
Epilepsy
TO RX PENICILLIN
TX IVIG/PLEX
WILSONS
Liver inv
No carditis
Hereditary
HUNTINGTONS
Anticipation
Psychiatric prob
Genetic/ Imaging
Antibodies
ASO > 240 TU in adults, >330 in children
ASO rises after 1 week peaks after 3 weeks
Anti DNAase B >120 TU in adults, >240 in children
Anti DNAase B rises after 2 weeks peaks after 6 weeks
Sensitivity
ASO only 65%
Anti DNAase B 85%
Together 95%
ESR>30, >50 in CHF (ESR falsely high in 50% pts of CHF)
CRP>3
Throat swab can not differentiate b/w active inf/ carrier
Multiple samples required
Yield 10%
Rapid antigen test also can not differentiate b/w active inf/ carrier
ECG features of active carditis
Heart blocks
PR prolongation despite
tachy
Relative brady
VPCs
Small voltage
DD
Dengue
Diphtheria
Progression of RHD
• Bland & Jones >20 yrs
• In india 5-10 yrs
• CMC Vellore 3months
• Depends on:
- Host factors (no penicillin prohpx)
- Environmental factors (overcrowding, malnutrition)
- Agent factors (Virulent strain, eg. Outbreak in Utah 1987)
RHD
Manjunath et el:
Mitral 60%
1/3 MS
1/3 MR
1/3 MS+MR
Mitral + aortic 25%
Aortic only 10%
Tricuspid only 10% (TR>>>TS)
Pulm valve only not reported from India
MVD 1/3
Complications of RHD
• PVH
• PAH
• LV dysfunction
• CHF
• AF
• Embolic stroke
• IE
Sudden worsening of symptoms
• Carditis/ ARA
• AF
• LV dysfunc
• Preg (carditis gravidarum)
• Vol overload
• Bact inf
• Thyrotoxicosis
• IE
• Thrombus
Recurrences
SB Roy
1. Musical murmer
2. Rub
3. Cardiomegaly
4. CHF
5. Sleeping tachycardia
Also
1. SC nodules
2. Prolonged PR despite tachy
3. Heart blocks
4. VPCs w/o digoxin
5. Pericardial effusion
Bland & Jones
1/5 in 5 yrs
1/10 in 5-10 yrs
1/20 in 10-15 yrs
1/40 in 15-20 yrs
Sanyal
Carditis in 1st attack 30%
Vaishnab
Carditis in all attacks 90%
RHD in Young
• <5 yrs: 5% (Chockalingum)
• <12 yrs: 10% (Vaishnab) – Pediatric MS
• <20 yrs: 20% (SB Roy) – Juvenile MS
• <40 YRS: 40%
Juvenile MS (SB Roy)
- Predominant MS
- Low ca
- Less AF
- Severe PAH
- Small aorta
- Cuspal: symp> signs
- Good result to BMV
ARF: Management
• Bed rest 4-6 weeks
• Good nutrition
• Benz Pen (<27 kgs) 6lac IU (>27 kgs) 12lac IU deep IM in buttock, small needle
• OR oral Pen V 250 TDSX5d (children) 500TDSX5d (adult)
• OR Erythromycin 250 QDSx10d (2omg/kg/d upto 1 gram in 3-4 divided doses)
• OR azithromycin 500 in day and 250 ODX4d (12.5 mg/kg/d x 5d)
• Arthtitis: ASA 100mg/kg/day in 3-4 divided doses
• Carditis: ASA 100mg/kg/day in 3-4 divided doses
• Salicylism: Resp alk (hyperventilation) – paradoxical aciduria – met acidosis
• CHF: prednisolone 1mg/kg/d in two divided doses
• Review after 1 weeks, 2 weeks and 4 weeks and FU with echo/ clin
NO PROPHYLAXIS FOR
ASYMP CARRIERS/ CONTACTS
Rebound/ Recurrence?
• On treatment:
Initial recovery. But later worsening = relpase
• Treatment completed
Symptoms reappeared after completion of tx
<6wks = rebound
>8wks = recurrence
Secondary prophylaxis
Secondary prophylaxis
Penicillin
• Recurrences
- w/o pen: 10%
- With oral pen: 3%
- With IM pen: 0.5%
• Complications
- allergy: 3%
- Anaphylaxis: 0.5%
- Death: 0.05%
• Why 3wks?
Incubation period: 9 days
Achieves t1/2: 19 days
Dose: 4 weekly
For developing countries: 3 wkly
(Pen level drops after 20 days, Taiwan)
Infective endocarditis prophylaxis
ddk3987@gmail.com
9674459039
OPD: Tues/ Thurs/ Sat
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ACUTE RHEUMATIC FEVER

  • 1. Rheumatic Heart Disease DR. DIBBENDHU KHANRA DM CARDIOLOGY AIIMS RISHIKESH
  • 2.
  • 3. Pathology • Fibrinous necrosis: exudative (bread and butter appearance) • Proliferative (Aschoff nodules/Antishkow/ caterpiller cells) – McCallum patch • Healing and fibrosis (milk spots)
  • 5. PREVALENCE 5-15 YRS >15 YRS RF 0.75/1000 (Mishra) 0.4/1000 (Verma) RHD 4.5/1000 (Lalchandani) 4.5/1000 (Lalchandani) 5-15 YRS All age Low risk pop <2/1 lac <1/1000 High risk pop >2/1 lac >1/1000
  • 6. SORE THROAT (GABHS) MODIFIED CENTOR CRITERIA 1. AGE 5-15 YRS 2. HIGH GRADE FEVER 3. ANT CERVICAL LN 4. TONSILLAR EXUDATE 5. COUGH ABSENT 0-1 +: NO AB* 2-3 +: THROAT SWAB RAPID AG DET AB IF POSITIVE 4-5 +: AB SORE THROAT to ARF: 3% (epidemic) 0.3% (endemic) THROAT SWAB: YIELD 5-10% *AMOXICILLIN/ AZITHROMYCIN GABHS Sore Throat Once RF after sore throat, 50% chance of RF recurrence after another sore throat
  • 8. ARF: Modified Jones Criteria MAJOR PANCARDITIS MIGRATORY ARTHRITIS CHOREA SC NODULES ERYTHEMA MARGINATUM MINOR HIGH FEVER ARTHRALGIA ESR>30 CRP>3 PROLONGED PR GAS INFECTION RAPID AG TEST THROAT SWAB ASO ANTI-DNAase H/O ARF IN RHD BLAND & JONES 30% PADMAVATI 30% PAUL WOOD 60% SB ROY 60% Jones criteria exempted MS Chorea
  • 9. INDIAN VS WESTERN WESTERN (BLAND & JONES) INDIAN (PADMAVATI, SANYAL) COMMENTS CARDITIS 2/3 1/3 LESS IN INDIANS ARTHRITIS 1/3 2/3 ARTHALGIA > ARTHRITIS CHOREA 50% 10% UNCOMMON SCN 5% 1% UNCOMMON EM 5% - RARE
  • 10. PANCARDITIS ENDOCARDITIS Regurgitations MC-MR PSM Careycoumb EDM (AR) Long PR/ AF MYOCARDITIS Cardiomegaly S3 Parchment carditis Vs viral carditis: No murmer Symp improves PERICARDITIS Rub Effusion Rare w/o endocarditis
  • 11. VALVULAR INV IN ARF VALVE INVOLVEMENT MITRAL 75% MITRAL + AORTIC 20% AORTIC 3% TRICUSPID 2% PULMONARY - FATE OF MR/ PSM 1/3 DISAPPEARS 1/3 SAME 1/3 PROGRESSES
  • 12. VALVULAR LOAD SVC 5 PV 10 RA 5 LA 10 RV 25/0-5 LV 120/0-10 PA 25/10 AO 120/80 TCV 20 mmHg MV 110 mmHg PV 5 mmHg AV 70 mmHg TCVA 2 mmHg/ cm2 MVA 40 mmHg/cm2 PVA 1 mmHg/ cm2 AVA 25 mmHg/cm2 TCVA 8-10cm2 MVA 4-6cm2 PVA 2-4cm2 AVA 2-4cm2
  • 13. Carditis • Acute: Dyspnea at rest • Subacute: DOE • Insidious: no symps, murmer+ • Subclinical: no symp, no murmer, echo+
  • 14. SEVERITY OF CARDITIS Severity Cl/F Mild NYHA 2-3 Mod NYHA 3-4 NO CARDIOMEGALY Severe NYHA 3-4 CARDIOMEGALY PERICARDIAL EFFUSION SC NODULES JACCOUDS ARTHOPATHY Fulminant NYHA 3-4 CARDIOMEGALY LV FUNCTION DEPRESSED
  • 16. CONSEQUENCE OF CARDITIS SANYAL ET AL ARF CARDITIS (60%) NO CARDITIS (40%) 2/3 RHD (40%) 1/10 RHD (4%)
  • 17. Which murmur disappears? • No CHF/ cardiomegaly • Low grade PSM • Single valve • Early penicillin • First attack • Male Which ARFwill lead to RHD? • CARDIOMEGALY / CHF • >GR2 EDM • OVERCROWDING • MALNUTRITION • NO PEN PROPHX • RECURRENT ATTACK
  • 18. HOW MANY DIES? BLAND & JONES 10% IN 10 YRS 20% IN 20 YRS TOTAL 30% (1/3) IN 3 YRS CHF CARDIOMEGALY 50% DIES
  • 19. Arthralgia/ arthritis! Fever and joint pain 1 week after sore throat Migratory Stereotypic Large joints No small joints NOT INVOLVED: TMJ, STERNOCLAV JOINT, ATLANTOAXIAL JOINT Back rarely involved Severely painful/ tender/ swollen/ red/ hot L/O function Symp> signs Each joint Lasts for 1 week Dramatic response to salicylates Total episode resolves in 4 week No residual deformity
  • 20. Arthralgia/ arthritis!DD VS PSRA 1. Short incubation period 2. Affects small joint 3. No response to salicylates 4. Often renal involvement 5. No carditis TO RX PENICILLIN PROPHYLAXIS FOR 1 YEAR VS JIA 1. MP rash incl face 2. Back inv 3. Small joints inv 4. LN 5. LFT deranged
  • 22. Subcutaneous nodules Extensor surface Elbow forearm Knee joints knee Severe carditis/ active carditis Painless Freely mobile Not attached to tendon Good response to salicylate DD Rheumatoid nodules/JIA -Larger -Painful -Attached to tendons Osler’s node Painful Pulp of fingers Smaller Janeway lesion Macular Palm soles blanching
  • 23. Erythema marginatum In crops Painless Axilla/ thighs+ Never on face Annular Evanescent Itchy Rare to find in indians Carditis+ No response to salicylates DD Scarlet fever Scalding
  • 24. Sydenhams Chorea Late manifestation Never with arthritis Carditis+ More in females Rare in postpubertal boys Resolves in 6m in 75% cases Jerky speech Pronator sign Jack in the box Worms in the tongue Milkmaids grip Spoon-like configuration Pendular knee jerk OCD Poor school performance Things fall from hands No sensory or motor inv
  • 25. Sydenhams Chorea/ DD PANDAS Early after sore throat OCD Tics Epilepsy TO RX PENICILLIN TX IVIG/PLEX WILSONS Liver inv No carditis Hereditary HUNTINGTONS Anticipation Psychiatric prob Genetic/ Imaging
  • 26. Antibodies ASO > 240 TU in adults, >330 in children ASO rises after 1 week peaks after 3 weeks Anti DNAase B >120 TU in adults, >240 in children Anti DNAase B rises after 2 weeks peaks after 6 weeks Sensitivity ASO only 65% Anti DNAase B 85% Together 95% ESR>30, >50 in CHF (ESR falsely high in 50% pts of CHF) CRP>3 Throat swab can not differentiate b/w active inf/ carrier Multiple samples required Yield 10% Rapid antigen test also can not differentiate b/w active inf/ carrier
  • 27. ECG features of active carditis Heart blocks PR prolongation despite tachy Relative brady VPCs Small voltage DD Dengue Diphtheria
  • 28. Progression of RHD • Bland & Jones >20 yrs • In india 5-10 yrs • CMC Vellore 3months • Depends on: - Host factors (no penicillin prohpx) - Environmental factors (overcrowding, malnutrition) - Agent factors (Virulent strain, eg. Outbreak in Utah 1987)
  • 29. RHD Manjunath et el: Mitral 60% 1/3 MS 1/3 MR 1/3 MS+MR Mitral + aortic 25% Aortic only 10% Tricuspid only 10% (TR>>>TS) Pulm valve only not reported from India MVD 1/3
  • 30. Complications of RHD • PVH • PAH • LV dysfunction • CHF • AF • Embolic stroke • IE
  • 31. Sudden worsening of symptoms • Carditis/ ARA • AF • LV dysfunc • Preg (carditis gravidarum) • Vol overload • Bact inf • Thyrotoxicosis • IE • Thrombus
  • 32. Recurrences SB Roy 1. Musical murmer 2. Rub 3. Cardiomegaly 4. CHF 5. Sleeping tachycardia Also 1. SC nodules 2. Prolonged PR despite tachy 3. Heart blocks 4. VPCs w/o digoxin 5. Pericardial effusion Bland & Jones 1/5 in 5 yrs 1/10 in 5-10 yrs 1/20 in 10-15 yrs 1/40 in 15-20 yrs Sanyal Carditis in 1st attack 30% Vaishnab Carditis in all attacks 90%
  • 33. RHD in Young • <5 yrs: 5% (Chockalingum) • <12 yrs: 10% (Vaishnab) – Pediatric MS • <20 yrs: 20% (SB Roy) – Juvenile MS • <40 YRS: 40% Juvenile MS (SB Roy) - Predominant MS - Low ca - Less AF - Severe PAH - Small aorta - Cuspal: symp> signs - Good result to BMV
  • 34. ARF: Management • Bed rest 4-6 weeks • Good nutrition • Benz Pen (<27 kgs) 6lac IU (>27 kgs) 12lac IU deep IM in buttock, small needle • OR oral Pen V 250 TDSX5d (children) 500TDSX5d (adult) • OR Erythromycin 250 QDSx10d (2omg/kg/d upto 1 gram in 3-4 divided doses) • OR azithromycin 500 in day and 250 ODX4d (12.5 mg/kg/d x 5d) • Arthtitis: ASA 100mg/kg/day in 3-4 divided doses • Carditis: ASA 100mg/kg/day in 3-4 divided doses • Salicylism: Resp alk (hyperventilation) – paradoxical aciduria – met acidosis • CHF: prednisolone 1mg/kg/d in two divided doses • Review after 1 weeks, 2 weeks and 4 weeks and FU with echo/ clin NO PROPHYLAXIS FOR ASYMP CARRIERS/ CONTACTS
  • 35. Rebound/ Recurrence? • On treatment: Initial recovery. But later worsening = relpase • Treatment completed Symptoms reappeared after completion of tx <6wks = rebound >8wks = recurrence
  • 38. Penicillin • Recurrences - w/o pen: 10% - With oral pen: 3% - With IM pen: 0.5% • Complications - allergy: 3% - Anaphylaxis: 0.5% - Death: 0.05% • Why 3wks? Incubation period: 9 days Achieves t1/2: 19 days Dose: 4 weekly For developing countries: 3 wkly (Pen level drops after 20 days, Taiwan)