Rheumatic Fever and Rheumatic Heart Disease

1. Guide: Dr Praveen Kusubi
Student :Dr.Kashibai S.P.
Rheumatic Fever and
Rheumatic Heart Disease

2. RHEUMATIC FEVER
 ARF is an inflammatory disease of the heart
involving all layers.(endocardium, myocardium ,
epicardium)
 It is a result of autoimmune process to infection with
group A Streptococci.
 RHD is a chronic condition resulting from rheumatic
fever that is characterized by scarring and deformity
of the heart valves.

3. Incidence
 ARF is disease involving mainly 5 to 14yrs.
 Initial episodes less common in older adolescents
and young adults and rare in persons aged
>30yrs.
 More common among females than males.
 Decreased incidence because of antibiotics and
improved living conditions.

4. ETIOLOGY
 Causative organism :
Caused by group A beta hemolytic
streptococcus.
Latent period is about 3weeks,from group A
streptococcal infection and appearance of the
clinical features of ARF.

5. PATHOPHYSIOLOGY
Causative
organism –group
A streptococci
Untreated
streptococcal
fever
Rheumatic fever

6. all 1ayers of heart and mitral
valve and other connective
tissue inflammation
Vegetation formation
Valvular regurgitation and
stenosis and joint pain
Heart failure

7. CLINICAL MANIFESTATIONS
 sore throat
 Tender lymph nodes
 Close contact with infected person
 Scarlet fever rash
 Tonsilar exudates in older children.

8. DIAGNOSTIC FEATURES
 Cluster of signs and symptoms
 A group of criteria developed by T.D.Jones
 Following upper airway infection with GAS
silent period for 2-6wks
sudden onset of fever ,pallor,fatigue.

9. JONES MAJOR CRITERIA(in
high risk groups )
POLYARTHITIS or
POLYARTHALGIA or
ASEPTIC
MONOARTHRITIS
CARDITIS
(subclinical evidence
of rheumatic
valvulitis on ECG)
SYNDEMHAM
CHOREA
ERYTHEMA
MARGINATUM
SUBCUTANEOUS
NODU1ES

10. MINOR
IN HIGH RISK IN 1OW RISK
FEVER >=38C >= 38.5C
MONOARTHRALGIA POLYARTHRALGIA
ESR>30mm/Hr or CRP
>30mg/L
ESR>60mm/Hr or
CRP>30mg/L
Prolonged PR interval on
ECG
Prolonged PR interval on
ECG

11. UPDATED 2020 CRITERIA FOR
ARF DIAGNOSIS
Definite initial episode of ARF
 2 Major +evidence of preceding strep A infection or
 1major +2 minors+ evidence of preceding strep A
infection
Definite recurrent episode of ARF in a patient with a
documented history of ARF OR RHD
 2major+ evidence of preceding strep A infection ,or
 1major +2minor + evidence of preceding strep A
infection ,or
 3minor + evidence of preceding strep A infection

12.  Possible ARF :Incomplete features of ARF with normal
ECHO and ECG .
 Probable ARF: Highly suspected ARF with normal
ECHO.
 Definite ARF(no cardia involvement) : ARF with normal
ECG and ECHO throughout ARF Episode .
 Definite ARF ( cardia involvement ): ARF with carditis or
RHD on ECHO or with AV conduction abnormality on
ECG during ARF episode.
 Borderline RHD : Borderline RHD on ECHO without
documented history of ARF.

14.  Mild RHD : Mild RHD on ECHO, AV conduction
abnormality on ECG during ARF episode.
 Moderate RHD : Moderate RHD on ECHO.
 Severe RHD : Severe RHD on ECHO ,or
previous valve repair or prosthetic valvee
replacement.

15. 1.Polyartritis
 Most common feature : in 90% cases
 Painful migratory ,short duration type.
 Usually >5 large joints ,mainly large joints.

16. 2.Carditis
 Early ,most serious manifestation.
 Manifests as pancarditis.
 60-70%cases.
 Myocarditis –apical systolic or basal diastolic murmur
- cardiomegaly or CHF
-ECG– AV conduction defect
 Pericarditis – anterior chest pain ,pericardia friction
rub
 Mc valve -involved is mitral valve,
 Mc lesion- Mitra1 regurgitation- apical pansystolic
murmur.

17.  C1inica1 features of common va1ve 1esions:
SYMPTOMS SIGNS COMP1ICATI
ONS
MITRAL
REGURGITATION
Mild –moderate:
asymptomatic
Dyspnea on exertion
Fatigue
Orthopnea
PND
Mid /pan systolic
murmur at apex
radiating laterally
CHF
Pulmonary
hypertension
MITRAL STENOSIS Exertional dyspnea
Orthopnea,PND,hem
optysis
low pitch, diastolic
murmur at apex
with patient in left
lateral position
Atrial
arrythmia
Pulmonary
hypertension
Systemic
embolism(str
oke,PAD)
AORTIC
REGURGITATION
Mild –moderate:
asymptomatic
Dyspnea on exertion
Fatigue
Orthopnea
PND
Blowing
decrescendo
diastolic murmur at
eft sternal edge
Systolic murmur
due to increased
flow
CHF

18. AORTIC
STENOSIS
Dyspnea
Angina
Presyncope
and syncope all
associated with
exertion.
Ejection
systolic
murmur over
aortic region
,radiating to
neck
Slow rising
pulse
HF with pEF or
rEF
Atria arythmia
TRICUSPID
REGURGITATIO
N
Peripheral
edema
Abdominal
discomfort and
distension
PSM at left
parasternal edge
Raised JVP with
prominent V
waves
Pusati1e 1iver
Right ventricular
heave
Right heart
failure
TRICUSPID
STENOSIS
Fatigue
Anorexia
Abdominal
discomfort
Soft, high pitch
diasto1ic murmur
at left
parasternal edge
Abdominal
ascitis
Hepatomegaly
Anasarca
Hepatomega1y
Hepatic
dysfunction

19. ECHO features of RHD
MITRAL
VALVE
FEATURES
Prolapse of anterior leaflet
Thickening
Restricted posterior leaflet
Chordal thickening
leaf1et calcification
Diastolic doming of anterior leaflet
(dog leg or hockey stick appearance)
AORTIC
VAVLE
FEATURES
Cusp prolapse
Cusp thickening ,restriction, fibrosis ,retraction,
calcification.
Rolled cusp edges
Dilated aortic root
TRICUSPID
VALVE
FEATURES
Leafet thickening ,calcification,restriction,retraction.
Chordal shortening

20. Role of investigations in diagnosis of
RHD
TTE:
 Baseline investigation
 Assessment of valve pathology
 Assessment of cardiac function and chamber
TOE:
 Presurgical planning
 Anatomical assessment for valve repair
 Exclusion of a thrombus and significant MR prior
to percutaneous balloon mitral vavuloplasty.

21.  ECG:
Identify arrythmias that may complicate RHD (e.g.
AF)
Identify structural changes of RHD (e.g. LVH ,P
mitrale)
 Exercise stress test& stress ECHO :
objective assessment when valve severity
discordant from symptoms.
 Coronary angiography& CT angiography :
exclude concomitant CAD prior to surgery.

22.  Cardiac MRI :
Role in assessing etiology of cardiomyopathy and
quantifying chamber size and function.
 ESR ,CRP,ASO Titres :
In cases of newly diagnosed RHD to exclude
possible ARF episode.
 BNP ,pro -NT BNP: Role in assessment of heart
failure

23.  Morphological features of RHD
 Mitral valve : AMVL thickening >3mm
chorda thickening
restricted leaflet motion
excessive leaflet tip motion during
systole
 Aortic valve : Irregular or focal thickening
coaptation defect
restricted leaflet motion
leaflet prolapse.

24. World Heart Federation minimum
ECHO criteria for diagnosis of
pathologic valvular regurgitation
caused by rheumatic carditis
 Pathological MR : seen in 2views
in at least one view ,jet length
>2cm.sq
peak velocity: >3m/s
pansystolic jet in atleast one view.
 Pathological AR : seen in 2views
in at least one view ,jet length
>1cm.sq
peak velocity: >3m/s
pandiastolic jet in at least one view

25. CHOREA
 Major CNS manifestation.
 Characterized by involuntary movements
,especially of the face and limbs,causing
disturbances of speech or gait.
 Demonstrated by milkmaid grip or jack in the
box tongue.
 lasts for 1wk to 2yrs.
 Chorea overlapped with neuropsychiatric
disorder, with tics or ocds –PANDAS.

26. SUBCUTANEOUS NODULES
 Firm, painless, freely mobile over subcutaneous
tissue, about 0.5 to 2cm
 Resembles of rheumatoid arthritis nodules.
 In ARF nodules occurs around elbow near
olecranon process, where as in RA nodules occur
over extensor aspect of elbow.
 Usually seen in children with prolonged active
carditis.

27. ERYTHEMA MARGINATUM
 Occurs over upper arm and trunk.
 Bright pink. nonpruritic , map like macular lesions.
 Less common manifestation.
 Gets prominent with hot shower.

28. Diagnosis
 Investigations recommended in all suspected
cases:
-WBC
-ESR,CRP
-THROAT SWAB
-BLOOD CULTURE
-Antistreptococcal serology : ASO and
antiDNAase B titres.
-ECG
-CXR
-2DECHO

29. TREATMENT
 AIM:-
 To suppress the inflammation of the heart and
joints.
 To eradicate GAS from pharynx.
 To provide symptomatic relief.
 To commence secondary prophylaxis.

30.  Eradication of inciting infection :
 PRIMARY PREVENTION of RF
Benzathine benzylpenicillin G ,1.2mU I.M single
dose.
Phenoxymethyl penicillin ,500mg bd for 10days.
azithromycin 500mg,od for 5days
.
 SECONDARY PREVENTION :
 Benzathine benzylpenicillin , single i.m every 3 to
4wks
 Penicilline V ,oral 250mg bd
 Erthromycin 250mg bd oral.

31. Symptomatic management of arthritis:
 Choice of antinflammatory is NSAIDS and
corticosteroids.
-Aspirin 100mg/kg/day in four divided doses.
-Prednisone 1 to 2mg/kg/day.

32.  Moderate to severe chorea:
 CBZ 3.5 to 10mg/kg/day, PO bd
 Sodium va1proate 7.5 to 10 mg/kg per dose ,PO
bd.
 Very Severe chorea or chorea para1ytica :
 Anticonvulsant
 Prednisone 1 to 2 mg/kg up to 80mg ,PO

33.  Symptomatic management of carditis :
 Frusemide 20 -40 mg ,po or i.v singe dose
f/by 20-40mg ,po or iv tid.
 Spironolactone 12.5mg to 25 mg od .
 Nitrate therapy.
 ACE inhibitors in case of pt with LV dysfunction.
 Digoxin 15ug/kg ora11y ,sd
5ug/kg after 6 hours , then 3-5ug/kg daily .

34. Management of RHD
 Secondary prophylaxis is integral aspect in
management.
 Choice of valve replacement :
 mechanical valve: proven durability , requires
life1ong anticoagulation.
 Bioprosthetic valve : doesn’t require lifelong
anticoaguants, limited durability ,may need valve
in valve procedure.
 Anticoagulants in RHD : non vitamin K
antagonists preferred in patients with RHD and
AF with an elevated CHA2DS2-VA score, except
in severe MS .

35.  In severe MS with AF , warfarin is preferred.
 Patients with mechanical valve replacement
require anticoagulation with warfarin,
clexane,heparin.

36. Diagnosis Duration of prophy1axis
Possible ARF 12 months then reassess
Probable ARF Minimum of 5years after most recent
episode of probable ARF , or until 21yrs
(whichever is longer)
Definite ARF(No cardiac
involvement)
Minimum of 5years after most recent
episode of ARF , or until 21yrs (whichever
is longer)
Definite ARF( cardiac
involvement)
Based on RHD severity
Borderline RHD -
Mild RHD If documented history present-minimum of
10yrs after most recent episode of ARF ,or
until age 21 (whichever is longer)
If no documented history -Minimum of
5years after diagnosis of RHD , or until
21yrs (whichever is onger)

37. Moderate RHD If documented history present-
minimum of 10yrs after most
recent episode of ARF ,or until
age 35 (whichever is longer)
If no documented history -
Minimum of 5years after
diagnosis of RHD , or until Age
35yrs (whichever is longer)
Severe RHD If documented history present-
minimum of 10yrs after most recent
episode of ARF ,or until age 40yrs
(whichever is longer)
If no documented history -Minimum
of 5years after diagnosis of RHD , or
until Age 40yrs (whichever is
longer)

38.  DURATION OF SECONDARY PROPHYLAXIS
FOR RHEUMATIC FEVER
-
Patient without proven
carditis
For 5yr after last attack or
until 18yrs (whichever is
longer)
Patient with carditis (mild mr
or healed carditis)
10yrs after last attack or
21yr (whichever is longer)
More severe valvular
disease
Life long
After valve surgery Life long

39. THANKYOU