Aute Reumatic Fever in children warf case.PPT

Detail presentation

1. ACUTE RHEUMATIC FEVER
CASE PRESENTATION
BY
Dr.Ram Chand
Post fellow
PEADIATRIC CARDIOLOGY Department
NICVD

2. AGE: 10 YEARS
SEX: FEMALE
WEIGHT: 20 KG
PRESENTING COMPLAINTS:
FEVER 20 DAYS
JOINT PAIN 15 DAYS
CHEST PAIN 3DAYS
BREATHLESSNES 3DAYS

3. PAST HISTORY:
HAD SORE THROAT AND FEVER WHICH WAS
TREATED BY SOME MEDICATIONS FOR FEW DAYS
2 PREVIOUS SAME EPISODES OF JOINT PAIN AND
FEVER.
Investigation at the time of 1
ST
presentation
INVESTIGATION:
MP,WIDAL,MOUNTEX --IVE
ESR 70MM/1ST HR
ASOT >400UNITS
URINE DR ,STOOL DR
CXR :CT RATIO O.48

4. FAMILY HISTORY:
4 SIBLINGS ,HEALTHY
SOCIOECONOMIC:
LOWER MIDDLE CLASS, 7 MEMBERS
LIVE IN 3 ROOMS HOUSE WITH ALL
BASIC FACILITIES

5. CLINICAL FEATURES:
BREATHLESS PALE
HR:150/MIN R/R: 45/MIN
TEMP: 100-102F BP:110/60MMHG
JVP:6CM ABOVE THE STERNAL ANGLE
NO SUBCUTANEOUS NODULES
NO ERYTHEMA MARGINATUM

6. CVS:
APEX BEAT IN 6TH ICS,2CM LATERAL TO MCL
FORCEFULL ,ILL SUSTAINED
S1 SOFT P2 LOUD S3
PSM 3/6 AT APEX RADIATING TO AXILLA
PSM 2/6 AT LLSB ,ON INSPIRATION
PRECORDIAL RUB

7. CHEST:
BILATERAL CREPITATIONS
ABDOMEN:
LIVER 5CM BELOW THE COSTAL MARGIN
JOINTS:
RT.KNEE JOINT SWOLLEN, TENDER

8. CHEST XRAY:
CT RATIO 0.57
ECG:
HR:150/MIN
AXIS90
LVH
UPER LOBE SHUNTING
FLUFFY OPACITIES

9. BLOOD CP:
Hb: 9Gm/dl
TLC: 14000/cmm
ESR: 107mm/in 1st HR
ASOT:. > 400UNITS
THROAT SWAB NEGATIVE

10. ECHOCARDIOGRAPHY REPORT
LVIDD 50MM LVISD 42MM EF 55%
AO 18MM LA 37MM LA/AO 2/1
THICKENED MITRAL VALVE
NORMAL AORTIC VALVE
ENLARGED LV WITH PERSERVED LV
FUNCTION
MODERATE TO SEVER MR
MILD TR
MILD PERICARDIAL EFFUSION
GRADIENT ACROSS TV 45MMHg

11. MODIFIED JONES CRITERIA
(UPDATED 1992)
MAJOR CRITERIA MINOR CRITERIA
CARDITIS
POLYARTHRITIS
CHOREA
ERYTHEMA MARGINATUM
SUBCUTANEOUS NODULES
CLINICAL FINDINGS
ARTHARALGIA
FEVER
LAB FINDINGS
 ESR OR CRP
PROLONGED PR INTERVAL
SUPPORTING EVIDENCE OF ANTECEDENTGROUP A STREPTOCOCCAL INFECTION
POSITIVE THROAT CULTURE /RAPID ANTIGEN TEST
ELEVATED OR RISING ASOT TITER

12. DIAGNOSIS:
RECURRENCE OF ARF WITH CARDITIS
3RD EPISODE
RHD

13. Questions?
•Why this child had ARF
•Why first episode was not diagnosed?
•Why had recurrences?

14. Questions?
•Why this child had ARF
•SORE THROAT WAS NOT TREATED ADEQUATELY
•Failure of primary prophylaxis

15. Questions?
•Why first episode was not diagnosed?
Investigations at that time clearly showed the
diagnosis

16. Investigation at the time of 1
ST
presentation
INVESTIGATION:
MP,WIDAL,MOUNTEX --IVE
ESR 70MM/1ST HR
ASOT >400UNITS
URINE DR ,STOOL DR
CXR : CT RATIO .48

17. Questions?
•Why had recurrences?
After 1st episode secondary prophylaxis not
started.
Failure to start secondary prophylaxis

18. Why We are concerned about the Prophylaxis??
Primary and Secondary

19. Review
Nature Reviews Cardiology 10, 284-292 (May 2013) |
doi:10.1038/nrcardio.2013.34
Position statement of the World Heart Federation on the prevention
and control of rheumatic heart disease
Bo Remenyi, Jonathan Carapetis, Rosemary Wyber, Kathryn Taubert&
BonganiM. Mayosi
•After recovery from the initial episode of RF, 60–65% of
patients develop valvularheart disease
•About one 1/4 of pts require surgery within 10-15 years of
first presentation.

20. 193,000,000
1,910,356
Population Estimated
Used
Extrapolated
Incidence in Pakistan
According to calculated extrapolations with Incidence (annual) of
ARF: 12 per 1000
the actual prevalence or incidence of Acute rheumatic
fever is much higher region.

21. •So if about 1/3 pts require some
intervention then about 50,000 pts /year in
Pakistan requires surgeries
who can cope with this burden???

22. What Is the Solution
Only Primary Prevention
One Injection of Benzathine
Penicillin can save the lives
of Millions

23. Prevention of Acute Rheumatic Fever:
Primordial prevention
prevent or limit the impact of GAS infection in a population ie
Improve living condition and hygiene
Primary Prevention
Prompt diagnosis and treatment of Acute Streptococcal Pharyngitis.
Secondary Prevention:
Prevention of recurrences
Tertiary prevention:
intervention in individuals with RHD to reduce symptoms and disability, and
prevent premature death

24. Problems with
PRIMARY PROPHYLAXIS:
ABOUT 1/3 EPISODES OF STREPTOCOCCAL
PHARYNGITIS ARE SUBCLINICAL.
EVEN CLINICAL EPISODES ARE NOT
DIAGNOSED PROPERLY
MAY NOT BE TREATED WITH PROPER AND
ADEQUATE ANTIBIOTIC

26. Problems in Diagnosis
•Jones criteria is for the Diagnosis of First Episode
•Not helpful in diagnosis of recurrence of Rheumatic Fever
•Revision and modification has increased the specificity but
reduced the sensitivity –reduces over diagnosis
•Jones Criteria are guidelines. Strict adherence to this criteria
may result in under diagnosis in developing countries
•To address the difficulties in diagnosis of ARF in ptwith
RHD ,WHO has published a guide line in 2004 and then in
2015

27. AHA revised 2015 Jones Criteria:
•recognizes the variability in clinical presentation in populations at
different levels of risk
•suggests that diagnostic criteria may be applied differently, depending
on rate of ARF or rheumatic heart disease (RHD) in the population.
•This can help avoid overdiagnosisin low-incidence populations and
underdiagnosisin high-risk ones.
•includes Doppler echocardiography as a tool to diagnose cardiac
involvement to diagnose Sub clinical Carditis

28. •Subclinical carditis
refers exclusively to the circumstance in which classic auscultatory
findings of valvar dysfunction either are not present or are not recognized
by the diagnosing clinician but echocardiography/Doppler studies reveal
mitral or aortic valvulitis

29. low risk HighRisk
ARF incidence <2 per
100 000 school-aged
children (usually 5–14
years old) per year or
Class IIaChildren not clearly from
a low-risk population are
at moderate to high risk
depending on their
reference population
Class I
an all age prevalence of
RHD of ≤1 per 1000
population per year
Class IIa

30. •Following are added as major criteria in
AHA revised 2015 Jones Criteria
–Sub clinical Carditis
•for moderate to high risk population
–monoarthritis
–Polyarthralgia

31. Summary of the AHA revised 2015 Jones Criteria

32. Importance of Secondary Prophylaxis
Of these, secondary prophylaxis has been proved to be cost-
effective and practical even in the poorest countries. (WHF)
Only 2% of children receiving secondary prophylaxis have
recurrence
While 44% of those who did not receive prophylaxis develop
•recurrences
After the first attack of ARF, the residual heart disease frequently
disappears or regresses clinically prevented by continuous Anti
Streptococcal Prophylaxis.

33. Problems with Secondary Prophylaxis
•Main issue is to diagnose the subclinical or mild RHD.
•the majority of patients in registeriesare symptomatic with
advanced disease, resulting from number of silent or
undetected attacks of ARF.
How to identify people with mild RHD so that they
may be offered secondary prophylaxis earlier,

34. In high risk situations three weekly administration is advised

35. Duration of Secondary Prophylaxsis
Category Duration
Rheumatic fever with carditis
and residual heart disease **
10 y or greater since last episode
and
at least until age 40 y; sometimes
lifelong prophylaxis*
Rheumatic fever with carditis
but no residual heart
disease**
10 y or well into adulthood,
whichever is longer.
Rheumatic fever without
carditis
5 y or until age 21 y, whichever is
longer.
*lifelong” prophylaxis refers to patients who are at high risk and likely to come in contact with
populations with a high prevalence of streptococcal infection
**Clinical /ECHO evidence
There is no agreement about the duration of secondary prophylaxis
The appropriate duration is determined by age, time since the last
episode of ARF, and potential harm from recurrent ARF

36. World Heart Federation has recommended
echocardiographic screening for RHD in high-prevalence
region since 2004
To diagnose previously undiagnosed RHD, including
those without a known history of ARF
To start secondary prophylaxis earlier thus potentially
reducing morbidity and mortality.
,

37. Echocardiography/Doppler should be
performed
in all cases of confirmed and suspected ARF
to assess whether carditisis present in the
absence of auscultatoryfindings
(Class I; Level of Evidence B).
serial studies in any patient with diagnosed or
suspected ARF even if documented carditisis
not present on diagnosis
(Class IIa; Level of Evidence C).
findings not consistent with carditisshould
exclude that diagnosis in patients with a heart
murmur otherwise thought to indicate
rheumatic carditis
(Class I; Level of Evidence B).
Recommendation for Echocardiography and Doppler in
AHA revised 2015 Jones Criteria

38. There are still some cases which do not fullfilthese criterias.
Various groups are defined so that secondary prophylaxis can be started
•Probable ARF (Should be given secondary prophylaxis)
•Cases from a population with high rates of ARF and/or RHD not meeting
the criteria for definite ARF will be considered probable ARF if they meet
any of the following criteria
i. A primary episode with no major manifestations, but with
polyarthralgiaor monoarthritis, plus at least two other(non-joint) minor
manifestations plus
.
ii. A recurrent attack of ARF in a patient Withoutestablished RHD
presenting with two minor manifestations plus
iii. The presence of at least two minor manifestations
in a person with no clinical manifestations of carditisand with no known
history of ARF or RHD but with echocardiographic evidence of definite or
probable RHD
If there is evidence of a preceding GAS infection in all cases

39. •“Possible” Rheumatic Fever
•When updated Jones criteria is not fulfilled but the clinician
still have good reason to suspect that ARF is the diagnosis
then physician manage the patient accordingly
Where there is genuine uncertainty,
1.Give secondary prophylaxis for 12 months
2.Re evaluate by careful history and physical
examination and repeat echocardiogram (Class IIa;
Level of Evidence C).
3.Secondary prophylaxis may be discontinued later in a
patient with recurrent symptoms if there is no
evidence of
a.group A streptococcal infection and
b.echocardiographic evidence of valvulitis, (Class
IIa; Level of Evidence C).

40. •In our country RHD develops very early at age 4-
5 years
•Require valve replacement at age as early as 10
years
•Require PTMC as early as 3 years
•Primary prophylaxis is the main action which can
reduce the incidence of RHD
•Secondary prophylaxis limits the disability and
mortality
•Detection of asymptomatic patients with RHD is
crucial for reducing the RHD

41. Thank You

42. TREATMENT:
:•ANTISTREPTOCOCCAL THERAPY:
BENZATHINE PENICILLIN I/M STAT
THEN AFTER 10 DAYS START MONTHLY
PROPHLECTIC INJECTION
OR
ALTERNATIVE REGIMEN

43. •ANTI INFLAMMATORY THERAPY
WITH NO HEART INVOLVEMENT/MILD TO
MODERATE CARDITIS
ASPIRIN 100MG/KG/DAY
REDUCE BY 25%AFTER 1WEEK IF GOOD
RESPOSE AND CONTINUE FOR 6-8 WEEKS
WITH SEVERE CARDITIS(CCF OR PERICARDITIS)
PREDNISOLONE 1-2 MG/KG/DAY FOR 2
WEEKS THEN TAPER
ADD ASPIRIN 80 MG/KG/DAY AND
CONTINUE FOR 8-12 WEEKS

44. Guide lines forBed Rest and Ambulation in patientswithAcute
rheumatic Fever
•LIMITATION OF ACTIVITY

45. General:
Bed rest
Anti failure
Specific:
Eradication of any residual
Streptococci
Anti inflammatory
treatment.
Treatment of Acute
Rheumatic Fever

46. Guidelines for Bed Rest and Ambulation
in patients with AcuteRheumatic
Fever
:
Bed
Rest
Clinical
State
Arthrit
is Only
Mild
Carditi
s
Modera
te
Carditi
s
Severe
Carditis
Bed
rest
1 to 2
weeks
2 to 4
weeks
2 to 3
months
3 to
6months
Limited
activity
2 to 6
weeks
2
months
3
months
6 to 12
months
Full
activity
After 6
weeks
After 3
months
After 6
months
Individual
ize

47. Anti Streptococcal Therapy:
BenzathinePenicillin
I/M Stat
or
Oral or IV Penicillin for
10 Days
Treatment.
contd:

48. Anti Inflammatory Treatment OF Acute Rheumatic
Fever
With No Heart
Involvement
Mild To Moderate
Carditis
With Severe
Carditis(CCF Or
Pericarditis))
Aspirin 80mg/kg/dayAspirin 80mg/kg/dayPrednisolone 1-2
mg/kg/day for 2
weeksthen taper
Reduce by 25% after
1week if good
response
Reduce by 25% after
1week if good
response
Add aspirin 80
mg/kg/day
continue for 6-8
weeks
continue for 6-8
weeks
continue
for 8-12 weeks
Treatment.
contd:

49. Criteria( AHA –Jones) for diagnosis of ARF exist
No such widely accepted criteria exist for RHD.
World Heart Federation has developed criteria for
echocardiographic identification of RHD in patients who do
not have a history of ARF
These guidelines are not for the diagnosis of carditis in the
setting of ARF or for the diagnosis of RHD in patients with a
history of ARF

50. REASON FOR RECURENCES :
•INITIAL EPISODES WERE NOT DIAGNOSED
(FEVER, JT.PAIN WITH SWELLING.ESR70, ASOT.400)
•SECONDARY PROPHYLAXIS WAS NOT ADVISED
•SORE THROAT WAS NOT TREATED ADEQUATELY
•Failure of primary prophylaxis

51. TREATMENT:
GENERAL;
BED REST O2 INHALATION
ANTIFAILURE;
INJ. FRUSEMIDE 40MG IV STAT THEN 20 MG TDS
DIGOXIN
CAPTOPRIL 25MGTDS

52. ANTI INFLAMMATORY :
PREDNISOLONE 1MG/KG/DAY
TAPERED AFTER 15 DAYS
SALICYLATES 80MG/KG/DAY
ADDED
ESR after 10days 70MM/IN FIRST HOUR
ESR after 20 days40MM/IN FIRST
HOUR
RESPONSE:

53. EXCEPTIONS TO JONES CRITERIA
•CHOREA
•PREVIOUS HISTORY OF ARF/RHD
•INDOLENT CARDITIS

54. So then what we need to do
Prevention
Prevention
Prevention

55. DIAGNOSTIC GUIDLINES
•INITIAL LOW ASOT
•DOES NOT EXCLUDE, ANTIBODY TITERS OF
ANOTHER ANTIGENS CAN BE DONE
IN 95%OF CASES HIGH TITERS CAN BE OBTAINED
BY USING THREE DIFFERENT TESTS
OR RISING TITER OF ASOT IN SECOND SAMPLE
Keep this slide

56. Definationsused for Acute
Rheumatic fever and RHD
•Primary episode:
–Any episode in a patient with no known prior history of ARF or
RHD, and who on presentation has no clear evidence of pre-
existing RHD, will be considered a.
•Recurrent episode
•Any episode in a patient with a known prior history of ARF or
RHD, or who on presentation has clear evidence of pre-existing
RHD,
•To qualify as a recurrence in a patient who has had a recent
episode of definite or probable ARF, the
•symptoms and/or signs of recurrence must begin at least 60 days
following the onset of the previous episode AND after the signs
of active inflammation in the previous episode have resolved