Rheumatic Heart Disease
Pathology
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? Fibrinous necrosis: exudative(bread and butter appearance)
? Proliferative (Aschoff nodules/Antishkow/
caterpiller cells) ? McCallum patch
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? Healing and fibrosis (milk spots)
Series of Events
SORE
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ACUTE
RHEUMATIC
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ACUTETHROAT
RHEUMATIC
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HEART
RHEUMATIC
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(GABHS)FEVER
DISEASE
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ACTIVITY
COMPLICATIONS
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PREVALENCE5-15 YRS
>15 YRS
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RF
0.75/1000 (Mishra)
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0.4/1000 (Verma)RHD
4.5/1000 (Lalchandani)
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4.5/1000 (Lalchandani)
5-15 YRS
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Al ageLow risk pop
<2/1 lac
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<1/1000
High risk pop
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>2/1 lac>1/1000
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GABHS Sore ThroatMODIFIED CENTOR CRITERIA
1. AGE 5-15 YRS
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0-1 +: NO AB*
SORE
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2. HIGH GRADE FEVER2-3 +: THROAT SWAB
THROAT
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3. ANT CERVICAL LN
RAPID AG DET
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(GABHS)4. TONSILLAR EXUDATE
AB IF POSITIVE
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5. COUGH ABSENT
4-5 +: AB
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SORE THROAT to ARF: 3% (epidemic) 0.3% (endemic)Once RF after sore throat, 50% chance of RF recurrence after another sore throat
THROAT SWAB: YIELD 5-10%
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*AMOXICILLIN/ AZITHROMYCIN
SN 77
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SP 97ARF: Modified Jones Criteria
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MAJORPANCARDITIS
MINOR
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MIGRATORY
HIGH FEVER
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GAS INFECTIONARTHRITIS
ARTHRALGIA
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RAPID AG TEST
CHOREA
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ESR>30THROAT SWAB
SC NODULES
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CRP>3
ASO
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ERYTHEMAPROLONGED PR
ANTI-DNAase
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MARGINATUM
H/O ARF IN RHD
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Jones criteria exemptedBLAND & JONES 30%
MS
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PADMAVATI 30%
Chorea
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PAUL WOOD 60%SB ROY 60%
INDIAN VS WESTERN
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WESTERN
INDIAN
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COMMENTS(BLAND & JONES)
(PADMAVATI, SANYAL)
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CARDITIS
2/3
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1/3LESS IN INDIANS
ARTHRITIS
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1/3
2/3
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ARTHALGIA > ARTHRITISCHOREA
50%
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10%
UNCOMMON
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SCN5%
1%
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UNCOMMON
EM
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5%-
RARE
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PANCARDITISENDOCARDITIS
MYOCARDITIS
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PERICARDITIS
Regurgitations
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CardiomegalyRub
MC-MR
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S3
Effusion
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Parchment carditisRare w/o
PSM
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endocarditis
Careycoumb
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Vs viral carditis:EDM (AR)
No murmer
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Symp improves
Long PR/ AF
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VALVULAR INV IN ARFVALVE
INVOLVEMENT
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MITRAL
75%
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MITRAL + AORTIC20%
AORTIC
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3%
TRICUSPID
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2%PULMONARY
-
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FATE OF MR/ PSM
1/3 DISAPPEARS
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1/3 SAME1/3 PROGRESSES
VALVULAR LOAD
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SVC 5PV 10
RA 5
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LA 10
RV 25/0-5
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LV 120/0-10PA 25/10
AO 120/80
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TCV 20
MV 110
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mmHgmmHg
PV 5
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AV 70
mmHg
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mmHgTCVA 2
MVA 40
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TCVA 8-10cm2
MVA 4-6cm2
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mmHg/ cm2mmHg/cm2
PVA 2-4cm2
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AVA 2-4cm2
PVA 1
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AVA 25mmHg/ cm2
mmHg/cm2
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Carditis
? Acute: Dyspnea at rest
? Subacute: DOE
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? Insidious: no symps, murmer+? Subclinical: no symp, no murmer, echo+
? In jones criteria: No role of Murmer
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SEVERITY OF CARDITIS
Severity
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Cl/FMild
NYHA 2-3
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Mod
NYHA 3-4 NO CARDIOMEGALY
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SevereNYHA 3-4 CARDIOMEGALY
PERICARDIAL EFFUSION
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SC NODULES
JACCOUDS ARTHOPATHY
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Fulminant NYHA 3-4 CARDIOMEGALYLV FUNCTION DEPRESSED
SUB
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CLINICAL
CARDITIS
CONSEQUENCE OF CARDITIS
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SANYAL ET AL
ARF
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CARDITIS (60%)NO CARDITIS (40%)
2/3
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1/10
RHD
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RHD(40%)
(4%)
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Which murmur disappears?
Which ARFwil lead to RHD?
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? No CHF/ cardiomegaly? CARDIOMEGALY / CHF
? Low grade PSM
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? >GR2 EDM
? Single valve
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? OVERCROWDING? Early penicillin
? MALNUTRITION
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? First attack
? NO PEN PROPHX
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? Male? RECURRENT ATTACK
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HOW MANY DIES?BLAND & JONES
10% IN 10 YRS
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20% IN 20 YRS
TOTAL
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30% (1/3) IN 3 YRSCHF
CARDIOMEGALY
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50% DIES
Arthralgia/ arthritis!
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Fever and joint pain1 week after sore throat
Migratory
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Stereotypic
Large joints
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No small jointsNOT INVOLVED: TMJ, STERNOCLAV JOINT, ATLANTOAXIAL JOINT
Back rarely involved
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Severely painful/ tender/ swollen/ red/ hot
L/O function
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Symp> signsEach joint Lasts for 1 week
Dramatic response to salicylates
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Total episode resolves in 4 week
No residual deformity
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Arthralgia/ arthritis!DD
VS PSRA
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1. Short incubation period2. Affects small joint
3. No response to salicylates
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4. Often renal involvement
5. No carditis
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TO RX PENICILLIN PROPHYLAXIS FOR 1 YEAR
VS JIA
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1. MP rash incl face2. Back inv
3. Small joints inv
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4. LN
5. LFT deranged
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Signifies ARANon-erosive
Can involve lower limbs
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Subcutaneous nodules
Extensor surface
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Elbow forearm
Knee joints knee
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Severe carditis/ active carditisPainless
Freely mobile
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Not attached to tendon
Good response to salicylate
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DDOsler's node
Janeway lesion
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Rheumatoid nodules/JIA
Painful
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MacularPalm soles
-Larger
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Pulp of fingers
Smal er
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blanching-Painful
-Attached to tendons
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Erythema marginatum
In crops
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PainlessAxilla/ thighs+
Never on face
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Annular
Evanescent
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ItchyRare to find in indians
Carditis+
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No response to salicylates
DD
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Scarlet feverScalding
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Sydenhams ChoreaLate manifestation
Never with arthritis
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Carditis+
More in females
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Rare in postpubertal boysResolves in 6m in 75% cases
Jerky speech
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Pronator sign
Jack in the box
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Worms in the tongueMilkmaids grip
Spoon-like configuration
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Pendular knee jerk
OCD
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Poor school performanceThings fall from hands
No sensory or motor inv
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Sydenhams Chorea/ DD
PANDAS
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Early after sore throatOCD
Tics
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Epilepsy
TO RX PENICILLIN
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TX IVIG/PLEXWILSONS
Liver inv
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No carditis
Hereditary
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HUNTINGTONSAnticipation
Psychiatric prob
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Genetic/ Imaging
Antibodies
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ASO > 240 TU in adults, >330 in children
ASO rises after 1 week peaks after 3 weeks
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Anti DNAase B >120 TU in adults, >240 in childrenAnti DNAase B rises after 2 weeks peaks after 6 weeks
Sensitivity
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ASO only 65%
Anti DNAase B 85%
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Together 95%ESR>30, >50 in CHF (ESR falsely high in 50% pts of CHF)
CRP>3
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Throat swab can not differentiate b/w active inf/ carrier
Multiple samples required
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Yield 10%Rapid antigen test also can not differentiate b/w active inf/ carrier
ECG features of active carditis
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Heart blocks
PR prolongation despite
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DDtachy
Dengue
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Relative brady
Diphtheria
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VPCsSmall voltage
Progression of RHD
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? Bland & Jones >20 yrs? In india 5-10 yrs
? CMC Vellore 3months
? Depends on:
- Host factors (no penicillin prohpx)
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- Environmental factors (overcrowding, malnutrition)- Agent factors (Virulent strain, eg. Outbreak in Utah 1987)
RHD
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Manjunath et el:Mitral 60%
1/3 MS
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1/3 MR
1/3 MS+MR
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Mitral + aortic 25%Aortic only 10%
Tricuspid only 10% (TR>>>TS)
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Pulm valve only not reported from India
MVD 1/3
Complications of RHD
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? PVH
? PAH
? LV dysfunction
? CHF
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? AF? Embolic stroke
? IE
Sudden worsening of symptoms
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? Carditis/ ARA
? AF
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? LV dysfunc? Preg (carditis gravidarum)
? Vol overload
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? Bact inf
? Thyrotoxicosis
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? IE? Thrombus
Recurrences
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SB RoyBland & Jones
1. Musical murmer
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2. Rub
1/5 in 5 yrs
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3. Cardiomegaly1/10 in 5-10 yrs
4. CHF
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1/20 in 10-15 yrs
5. Sleeping tachycardia
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1/40 in 15-20 yrsAlso
Sanyal
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1. SC nodules
Carditis in 1st attack 30%
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2. Prolonged PR despite tachy3. Heart blocks
Vaishnab
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4. VPCs w/o digoxin
Carditis in all attacks 90%
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5. Pericardial effusionRHD in Young
? <5 yrs: 5% (Chockalingum)
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? <12 yrs: 10% (Vaishnab) ? Pediatric MS
? <20 yrs: 20% (SB Roy) ? Juvenile MS
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? <40 YRS: 40%Juvenile MS (SB Roy)
- Predominant MS
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- Low ca
- Less AF
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- Severe PAH- Smal aorta
- Cuspal: symp> signs
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- Good result to BMV
ARF: Management
? Bed rest 4-6 weeks
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? 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)
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? 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
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NO PROPHYLAXIS FOR
ASYMP CARRIERS/ CONTACTS
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Rebound/ Recurrence?? On treatment:
Initial recovery. But later worsening = relpase
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? Treatment completedSymptoms reappeared after completion of tx
<6wks = rebound
>8wks = recurrence
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Secondary prophylaxis
Secondary prophylaxis
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Penicil in
? Recurrences
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? Why 3wks?- w/o pen: 10%
Incubation period: 9 days
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- With oral pen: 3% Achieves t1/2: 19 days
- With IM pen: 0.5% Dose: 4 weekly
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For developing countries: 3 wkly? Complications
(Pen level drops after 20 days, Taiwan)
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- al ergy: 3%
- Anaphylaxis: 0.5%
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- Death: 0.05%Infective endocarditis prophylaxis
Q1: Commonest cutaneous manifestation in ARF?
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1. SC Nodules2. Eryhtme Marginatum
3. Oslers Node
4. Janeway Lesion
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Q2: what is the most common cause of Jaccouds arthropathy in India?1. SLE
2. ARF
3. RA
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4. TBQ3: MS/MR patient had recurrence at 45 yrs. 2' prophyx how long?
1. None
2. 1 yrs
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3. 5 yrs4. 10 yrs
Q4: McCal um patch commonest in?
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? Ventr side of LV? Atrial side of LA
? Ventr side of AML
? Atrial side of PML
Q4: In RHD least involved mitral scal ops is?
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1. A2
2. A3
3. P2
4. P3
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