Acute Rheumatic Fever ppppppppooopop.pdf

A presentation on acute rheumatic fever in pediatric

1. ACUTE RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
VICTOR ZABLON MD

2. Outline
1.Definition
2.Epidemiology and Risk factors
3.Pathophysiology
4.Clinical presentation
5.Work up
6.Management
7.Follow-up

3. Definition
•Acute Rheumatic Fever : Is an immune mediated consequence of GAS
infection of the throat ( throat pharyngitis )
•Its highly infectious and spreads via saliva and nasal droplets.
•It causes long term, irreversible damage to the heart valves termed as RHD
,often as a recurrence.
•It continues to be a major public health concern to many parts of the world.

4. Epidemiology
•Incidence of ARF and RHD has decreased in the United States and other
industrialized countries during the past 80 years toless than 0.05 per 1000
population, with rare regional outbreaks.
•Worldwide based upon conservative estimates there are approximately 470,000
new cases of ARF and 275,000 deaths. In indiathere approx. 50,000 cases every
year.
•ARF, RHD and glomerulonephritis is estimated to affect over 33 million people .
And is the leading cause of cardiovascular death during the first 30 decades.

5. Epidemiology
•ARF is principally a disease of childhood with a median age of 10yrs, Its rare in children < 4year
and after 20 years, most frequently occurs in children from 5 to 15 years,
•It affects both males and females, but females with ARF fare worse than males and have a slightly
higher incidence of chorea
•Decreased incidence of rheumatic fever has been attributed to the introduction of penicillin or a
change in the virulence of the streptococci.
•3-6% of those infected develop acute rheumatic fever, and genetic host susceptibility factors are
thought to play a key role in disease development
•.Incidence is higher in low and middle income countries. RHD is reported > RF with most patients
presenting for the first time with complications of RHD in late adolescent or early adulthood

6. Risk factors
•Vulnerability of ARF starts at about 4 –20 years.
•If exposed to GAS in the throat or skin can develop mild risk ARF.
•After 3 weeks individual risk of developing ARF is partially influenced by –
genetic factors , poverty and social deprivation.
•Poor quality housing and crowding, poor nutrition , poor utilization or access
to health care are associated factors
•Host oral health has been associated with ARF.

7. Pathogenesis/pathophysiology
•The pathogenic mechanisms that lead to the development of acute rheumatic fever
(ARF) remain incompletely understood.
•Clearly streptococcal pharyngeal infection is necessary for the development of ARF.
•Genetic susceptibility may be present, om the other hand, evidence is sparse that
toxins produced by the streptococcus are important.
•Pharyngeal infection with GAS leads to activation of cells of the innate immune system
such as Neutrophiles, macrophages , and dendritic cells proceed to phagocytose the
bacteria, followed by antigen processing and presentation to the T-cell result in
activation of both the humoral and cellular immunity.
•The immunity against GAS is intended to eliminate the bacterium from the bodies.

8. Pathogenesis.
•The immune response becomes cross reactive with human tissue in susceptible
individual ( this is the driving mechanism of ARF.)
•These cross reactive antibodies and T-cell are generated thru the process of
molecular mimicry.
•Antigenic epitopes are shared between the host and the bacteria, this autoimmune
responses are responsible for the clinical presentation seen in ARF.
ØCarditis-cross reactive antibodies , and T-cells
ØArthritis –immune complex deposition
ØChorea-antibodies bind to the basal ganglia and neuronal cells.
ØSkin & subcutenoustissue –due to delayed hypersensitivity

9. Revised Jones Criteria for diagnosis of ARF

12. Investigations used in
diagnosis
•Acute phase reactants-ESR & CRP
•FBC
•Blood culture
•Throat culture
•Streptococalserology;ASOT
•CXR
•Echocardiograph
•ECG
Management:
Establishing the Dx
1.Admit to hospital
2.clinical evaluation and investigation to
confirm ARF.
3.Do acute phase reactants, serology for
streptococcal, blood and thtoatcultures.
ECG, ECHO
4.Eradicate the streptococcal organism
-Oral penicillin V

13. Management continue
•Carditis / Heart failure
•An urgent echocardiogram and
cardiology assessment.
Anti-Failure
•Diuretics
•Fluid restriction
•ACE inhibitors
•Glucocorticoidssevere carditis
•Digoxin if atrial fibrillation present
•Treat anaemiaany intercurrent infections.
Arthritis / Arthralgia
•Radiography of an affected joint may demonstrate a slight effusion
but is usually unremarkable.
•Analysis of the synovial fluid in rheumatic fever with arthritis
generally demonstrates sterile inflammatory fluid
•Rx; Paracetamol, NSAIDS after dx has been made (ibuprofen,
naproxen ) corticosteroids
Chorea
-Penicillin V or IM benzathine
-Haloperidol ( 0.025mg/kg/day -0.15mg/kg/day )
-Carbamazepine-
-Valproic acid (15-20mg/kg/day)
-( if abnormal movement interfere with daily activities
-Cortocosteroidplay role in chorea

14. Discharge protocol
•Discharge once there is clinical improvement
•Reduction in the acute phase reactants.
•Notify to the infectious disease surveillance
•Patient and family education
•Need for secondary prophylaxis
•Out patient follow up.

16. Differential Diagnosis

17. Work up

18. Work up

20. Prevention in the context of ARF and RHD
1.Primordial prevention
2.Primary prevention
3.Secondary prevention.

21. Primordial prevention
Social , economic and environmental initiatives undertaken to prevent or limit the
impact of GAS infection in a population
§Improve housing, alleviate over-crowding
§Improve health knowledge and access to quality health care
§Education
§Alleviate poverty

22. Primary prevention
Aim:
•Reducing GAS transmission
Øtreat GAS infections effectively to prevent the development of ARF in individuals
•Early treatment of GAS pharyngitis
•Treatment of ARF-Carditis, Arthritis, Sydehan’sChorea to prevent progression
to RHD

23. Antibiotics used Treatment/primary prevention.
AntibioticDose Duration
Benzathinepenicillin G900 mg (1,200,000 U) ≥20 kg
450 mg (600,000 U) <20 kg
I.Minjection
once
Phenoxymethylpenicillin15 mg/kg up to 500 mg, bdOral For 10
days
Amoxicillin50mg/kg /dayin 2 divided dosesOral for10 days
Erythromycin
For pts with hypersensitivity to
penicillinsor Azithromycinfor 5/7
Child: 12.5 to 20 mg/kg up to 800 mg,
bd
Oral For 10
days

24. Secondary prevention
•Administering regular prophylactic antibiotics to individuals who have
had an episode of ARF to prevent the development of RHD or to
individuals who have established RHD to prevent the progression of
disease

25. 1.Antibiotic prophylaxis
•1stLine-I.M Benzathinepenicillin G 900 mg (1,200,000 U) for ≥20kgor 450 mg
(600,000 U) <20 kg once every 4 weeks
•Phenoxymethylpenicillin(Penicillin V) 250 mg Oral Twice daily
•Erythromycin 250 mg Oral Twice daily for those with allergy to penicillins(
clindamycin, clarithromycin,,cephalexin, azithromycin )
2.Duration of Prophylaxis
-

26. Rheumatic Heart Disease outline
•Definition
•Epidemiology
•Components of RHD care
•Individual lesions
•Conclusion

27. Introduction
•Rheumatic Heart Disease (RHD) is the permanent heart valve damage resulting
from one or more attacks of ARF Carditis.
•Rheumatic heart disease (RHD) remains a major cause of cardiovascular
disease in resource-limited nations,
•It is thought that 40-60% of patients with ARF will go on to developing RHD.
•The commonest valves affected are the mitral and aortic. However all four
valves can be affected

29. Epidemiology
•Rheumatic heart disease (RHD) currently affects about 15.6million people
worldwide, mainly young adults with about 345,000 annual deaths.
•Sub-Saharan Africa-> 1million children with RHD
•Female > Male
•The prevalence of RHD peaks in the third and fourth decades of life.

30. Mitral Insufficiency
•The result from structural changes ; loss of valvular substance & shortening
and thickening of the chordae tendineae
•The left ventricle becomes enlarged due to the high volume load and
inflammatory process
•The left atrium dilates as blood regurgitates into this chamber èIncreased
left atrialpressure èpulmonary congestionèleft-sided heart failureè
raised pulmonary artery pressure èright sided heart failure

31. •Clinical signs
-mild-moderate –asymptomatic for many years
_ Apex beat displaced laterally
Øhigh-pitched holosystolic murmur at the apex radiating to the axilla
ØSigns of chronic heart failure
ØHeaving apical left ventricular impulse
ØApical systolic thrill
ØAccentuated S2 in case of pulmonary hypertension
ØA short mid-diastolic murmur
•Investigations
ØCXR, ECG-bifid P waves, signs of LV hypertrophy
ØCardiac Echo-demonstrate enlargement of the left atrium and ventricle, Doppler-
demonstrates the severity of the mitral regurgitation
ØCardiac Catheterization
ØLeft Ventriculography

32. •Treatment
ØProphylaxis against recurrent RF
ØAfter load reducing agents ( ACEIs, ARBs)
ØTreatment of complications e.gAF
ØSurgical treatment
•Annuloplastyring /band
•Prosthetic mitral valve replacement
•Complications:
ØHeart failure,
Øartrial(e.gAF) & ventrculararrhythmias,
Øinfective endocarditis,

33. Mitral Stenosis
•Results from fibrosis of the mitral valves leaflets contracture of the valve
leaflets, chordae, and papillary muscles over time.
•Usually takes 10 yr or more for the lesion to become fully established. Rare <
10 years
•Increased atrial pressureèenlargement and hypertrophy of the left atrium
èpulmonary venous hypertensionèpulmonaryhypertensionèRight
ventricular hypertrophy and right atrial dilatation èright ventricular dilation,
tricuspid regurgitation, and clinical signs of right-sided heart failure.

34. Clinical features
•Mild-asymptomatic
•Severe
oExercise intolerance
oPND
oDyspnoea
oLess commonly-systemic embolism from L.A e.gstroke, PAD
oFeatures of RHF
oPalpable RV heave in the Lt. parasternal region
oMitral diastolic murmur best heard at apex
oHolosystolicmurmur at tricuspid area in case of TR
Investigations; ECG, CXR, Cardiac Echo & Doppler, Cardiac Catherization

35. Treatment
•Diuretics and B-blockers for HF
•Digoxin+/-diltiazemfor AF
•Surgical valvotomy
•balloon catheter mitral valvuloplasty
Balloon valvuloplastyis indicated for symptomatic, stenotic, pliable, non
calcified valves of patients without atrialarrhythmias or thrombi
•valve replacement is avoided unless absolutely necessary

36. Aortic Insufficiency
•Sclerosis of the aortic valve distortion and retraction of the cusps then
regurgitation of blood > volume overload > dilatation and hypertrophy of
the left ventricle
•Combined mitral and aortic insufficiency is more common than aortic
involvement alone

37. Clinical features
•Palpitations
•Dyspnoea
•Angina
•PND
•Tachycardia
•Chest pain
•Features of CCF e.gedema
•In severe AR, the pulse pressure is widened, and the Korotkoffsounds are heard
almost down to the pressure of zero.
•Hypertension i.eSystolic hypertension
•Wide pulse pressure with bounding peripheral pulses.
•LV apical heave

38. Investigations
•CXR –enlarged LV, dilated ascending Aorta
•CardiaEcho-dilated left ventricle, diastolic mitral valve flutter or oscillation,
irregular or focal thickening of Aortic valve, decreased systolic excursion, a
coaptationdefect,andleaflet prolapse.
•ECG –unspecific ST-T wave changes, Left axis deviation.
•Doppler evaluation demonstrates the degree of aortic insufficiency.
•Magnetic resonance angiography (MRA) for quantitating regurgitant volume &
assessing LV size and systolic function.
•Cardiac catheterization when echocardiographicor imaging data are equivocal

39. Treatment of Aortic Insufficiency
•After load reducers (ACEIs, ARBs)
•Prophylaxis against recurrence of ARF
•Surgical intervention (valve replacement) should be
carried out well in advance of

40. Tricuspid Valve disease
•Isolated is rare
•More common secondary to right ventricular dilatation resulting from
unrepaired left-sided lesions
•Signs; prominent pulsations of the jugular veins, systolic pulsations of the liver,
and a blowing holosystolic murmur at the lower left sternal border that
increases in intensity during inspiration
•Signs of tricuspid insufficiency decrease or disappear when heart failure
produced by the left-sided lesions is successfully treated

41. Pulmonary valve disease
•Rare
•Occurs after pulmonary hypertension
•Late finding after mitral stenosis

42. Components of RHD care require:
•Secondary prevention with penicillin prophylaxis
•Adequate monitoring of anticoagulation therapy in patients with AF and/or
mechanical prosthetic valves with warfarin
•Access to oral healthcare to reduce risks of I.E
•Access to echocardiography
•Access to paediatricianand/or cardiologist, preferably the same specialist,
for regular follow up visits
•Access to cardiothoracic and interventional cardiology

43. Conclusion
•Rheumatic heart disease is a preventable chronic
heart condition
•Primary prevention include;
•Penicillin for suspected strep sore throat
•Secondary prevention includes;
•Penicillin prophylaxis

44. REFERENCES
•Nelsons text book of paediatrics21stedition
•Australian guidelines for management of rheumatic fever
2017.
•Acute rheumatic fever treatment guidelines 2015(
newzealandand Australian)
•Rudolp,stext book of paediatrics23rdedition
•Up to date.