ACUTE RHEUMATIC FEVERBurhanuddin IskandarPediatric CardiologyPediatric Department,Medical Faculty, Hasanuddin University/ WS Hospital Makassar

ETIOLOGY1.Immunologic Streptococcus Beta hemolytic group A

2. Predisposing factors - Family history - Socio economic status - Age 5 -15 years ( peak 8 years)

PATHOLOGY Inflammatory lesion : heart, brain, joints, skin

Aschoff bodies (in atrial myocardium) : characteristic ? Central necrosis surrounded by lymphocy tes, plasma cells, and large mononuclear and giant multinucleate cell

Aschoof Body : the cells are large, multinucleotide

CLINICAL MANIFESTATIONS HistoryStreptococcal pharyngitis, 1-5 wks (ave 3 wks) before onset; chorea 2-6 mosPallor, easy fatigability, epistaxis, abdominal pain

Positive family history

2. Carditis 50 % of cases, usu within first 3 wksDiagnosis requires presence of 1 of 4:- organic heart murmur- pericarditis (friction rub, pericard effusion, chest pain, ECG changes) - cardiomegaly on chest X ray- congestive heart failure

Jones criteria (updated 1992)Mayor criteria

1. Arthritis* Affects 70 % of cases* Large joints : knee, ankle, elbow, wrist* Often > 1 joints, simultaneously or in succession, migratory* Swelling, heat, redness, severe pain, tenderness, motion : not specific

Evidence of antecedent Group A Streptococcal infectionPositive throat culture or rapid streptococcal antigen tests for group A :less reliable (recent and chronic infect)Streptococcal antibody tests : most reliableASTO : 80% Anti-DNA se BAnti hyaluronidase

Diagnosis of rheumatic fever Based on

2 major criteria or + ASTO 1 major + 2 minor

ExeptionsChorea may occur as the only manifestations of RFIndolent carditis may be the only manifestationOccasionally patients with RF recurrencesmay not fulfill the Jones criteria

Differential diagnosis of RFJuvenile rheumatoid arthritisCollagen vascular diseasesVirus associated acute arthritis

Note

* Rheumatic fever is a clinical syndrome for which no specific diagnostic test exist !* No symptom, sign or lab test result is pathognomonic, although several combinations of them are diagnostic* Only carditis can cause permanent cardiac damage. Signs of mild carditis disappear rapidly in weeks but severe carditis may last for 2-6 months. Chorea and arthritis usually subside without permanent damage.

Management of RFBenzathin penicillin G 0.6 1.2 M units IM for eradication and prophylaxisBed restAcetosal for mild casesPrednison for severe casesAntiinflammatory agents not needed for isolated chorea

Recommended anti-inflammatory agents_______________________________________________________________________________________ Arthritis Mild Moderate Severe alone carditis carditis carditis__________________________________________________ Prednisone 0 0 0 2-6 wk*

Aspirin 1-2 wk 3-4 wk# 6-8 wk 2-4 mo___________________________________________________

* Prednisone should be tapered and aspirin started during the final week# Aspirin may be reduced to 60 mg/kg/dayDosagesPrednisone : 2mg/kg/day, in 4 divided dosesAspirin : 100 mg/kg/day, in 4-6 divided doses

Bed rest and indoor ambulation____________________________________ Arthritis Mild Moderate Severe Alone Carditis Carditis Carditis__________________________________________________________

Bed rest 1-2 wk 3-4 wk 4-6 wk as long as HF +Indoor ambulation 1-2 wk 3-4 wk 4-6 wk 2-3 mo_________________________________________________________

ESR: important for duration of restriction of activities.Full activity : ESR normal, except significant cardiac involvement _

Mild carditis : questionable cardiomegalyModerate carditis : definite but mild cardiomegalySevere carditis : marked cardiomegaly or HF (heart failure)

PreventionIdeally prophylaxis is indefiniteBenzathin Penicillin (600,000-1,200,000 U) every 28 days, min till age 21-25 ysSulfadiazine 0.5 g 1x daily (BW < 27 kg),1 g 1X (BW >27 kg)Penicillin V 2 x 250 mg /dayErythromycin 2 X 250 mg /day

DEMAM REMATIK

KARDITIS (+)

SEMBUH

KARDITIS (-)

PENYAKIT JANTUNG

REMATIK

REAKTIVASI

SEMBUH

REAKTIVASI

3 6 bulan

Bising masih ada

Bising -

Thank YouNO PAIN NO GAIN

RHEUMATIC HEART DISEASEAffects Mitral valve 75 %Aortic valve 25 %Tricuspid valve rarePulmonary valve never

Stenosis and regurgitation usually occur together

Mitral stenosisPrevalenceMost common valvular involvement in adultRequires 5-10 years from the initial attack

Pathology- Thickening of the leaflets and fusion of the commisureCalcification results overtimeDilated and hypertrophied LA and right sided heartPulmonary venous hypertension pulmonary congestion and edema and fibrosis of the alveolar walls, hypertrophy of the pulmonary arterioles, loss of lung compliance

Stenotic Mitral ValveCommisures are fused and valve thickened

Clinical manifestationsMild MS : asymptomaticMore severe : dyspnea with/out exertion : orthopnea, nocturnal dyspnea or palpitation

Physical ExaminationsIncreased RV impulse along the LSBWeak peripheral pulse with narrow pulse pressurePulmonary hypertension : loud S1 at apex and narrow split S2, accentuated P2Mid diastolic/presystolic murmur

ECG : RAD, LAH, RVH (due to PH)

CXR :Enlarged LA and RV, MPA segment prominentPulmonary venous congestion

Treatment of MSProphylactic antibioticRestriction of activity depends on severitySymptomatic patients (dyspnea on exertion, pulmonary edema, paroxysmal dyspnea) : baloon or surgery

MITRAL REGURGITATIONMost common in RHDPathology Mitral valve leaflets are shortened because of fibrosis. When degree of MR increases, dilatation of LA and LV results, mitral ring becomes dilated

Mitral Valve involvement

Echocardiography

Clinical manifestations * Asymptomatic during childhood * Rare : fatigue, palpitation

Physical examinationHeaving, hyperdynamic apical impulse in severe MRS1 normal or diminished. S2 may split (shortening of LV ejection, early aortic closure)Pansystolic murmur at apex left axilla

ECGNormal in mild casesLVH or LV dominance, with or without LAH

CXRLA and LV enlargedPulmonary congestion pattern in CHF

TreatmentProphylactic antibioticNo restriction of activity in mild casesSurgical : intractable CHF, progressive cardiomegaly, pulmonary hypertension

AORTIC REGURGITATIONLess common than MR. Mostly associated with mitral valve disease.

Pathology* Semilunar cusps are deformed and shortened.* Valve ring is dilated* Commisures usually are fused

Aortic Valvulitis

Clinical Manifestations

Mild regurgitation : asymptomaticMore severe : reduced exercise tolerance test

Physical Examination

Precordium may be hyperdynamic. Diastolic thrill at 3 LICSS1 decreased, S2 may be normal or singleHigh pitched diastolic cresendo murmur at 3 LICS or 4 LICS Systolic murmur at 2 RICS due to relative AS Severe AS : middiastolic murmur at apex

ECGNormal in mild casesSevere : LVH, LAHCXRCardiomegaly (LVH)Dilated ascending aorta

TreatmentProphylactic antibioticsMild cases : no restriction in activitySurgical : in anginal pain or dyspnea on exertion, significant cardiomegaly

Thank YouNO PAIN NO GAIN

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