DEMAM REMATIK AKUT

PENYAKIT JANTUNG REMATIK

dr. Lilia Dewiyanti, SpA, MSiMed.

Fakultas Kedokteran Universitas Muhammadiyah Semarang

Etiologi
1. Immunologic Streptococcus beta hemolytic group A 2. Predispositing Factor: - Family history - Socio-economic status - Age 5 15 years ( peak 8 years)

Patogenesis
Inflamatory lesion; heart,brain,joint,skin Ashoff bodies ( in atrial myocardium): characteristic ? Central necrosis surrounded by lymphocytes, plasma cell, and large mononuclear and giant multinucleate cell

Manifestasi Klinik
History Streptococcal pharyngitis, 1 5 weeks (ave 3) before onset. Chorea 2 6 mos. Pallor, easy fatigability, epistaxis, abdominal pain Positive family history.
Rheumatic fever

Strep throat
Strep skin

Acute glomerulonephritis

Histologic section of typical RF

Jones criteria (1992)

o Major criteria : - arthritis - carditis - erythema marginatum - subcutaneous nodules - Sydenhams chorea.
o Minor criteria : - arthalgia - fever - elevated acute phase reactan ( CRP, ESR) - ECG : PR interval > : not specific

plus

Supporting evident of antecedent Strep group A infec. - culture (+) or rapid strept antigent test - elevated or rising ASTO

1. Arthritis * Affects 70 % of cases * Large joints : knee, ankle, elbow, wrist * Often > 1 joints, simultaneously or in succession, migratory. * Swelling, heat, redness, severe paint, tenderness, motion < * Dramatic response to salicylate

Migratory arthritis

2. Carditis
- 50 % of cases - Diagnosis requires presence of : 1. Tachycardia 2. Heart murmur of valvulitis ( MR and AR ) 3. Pericarditis( friction rub, pericard effusion,chest pain, ECG changes) 4. Cardiomegaly on chest X ray 5. Congestif heart failure ( severe carditis)

Involve of the heart; pericarditis

Involve of the heart; myocardium and endocardium

3. Erythema marginatum
- < 10 % of cases - Non pruritic erythematous rashes, never on faces - Most prominent on trunk and inner proximal portion - Disappear on exposure to cold, seldom detected on AC hospital

Erythema marginatum

4. Subcutaneous nodules
- 2 10% of cases, esp in recurrences - Hard, painless, non pruritic,freely moveable, swelling 0,2 2 cm - Ussually symmetric on extensor surfaces of joint, scalp, along spine, last for weeks

Subcutaneous nodules

5. Sydenhams chorea.
- 15% of cases, more often in prepubertal girl - Begin with emotional lability and personality changes. - Spontaneous, purposeless movement followed by motor weakness, slurred speech - Dysfunction of basal ganglia and cortical neural components

Diagnosis of rheumatic fever Based on 2 major criteria or 1 minor criteria + ASTO

Exception to the Jones criteria

1. Chorea may occur as the only manifestation of RF 2. Indolent carditis may be the only manifestation 3. Occasionally patients with RF recurrences may not fulfill the Jones criteria

Differential diagnosis of RF
Juvenile rheumatoid arthritis Collagen vascular disease Virus associated acute arthritis Hematologic disorder: sicklemia and leukemia

Note
RF is a clinical syndrom for which no spesific diagnostic test exist No symptom, sign or lab test result is pathogno monic, although several combinations of them are diagnostic Only carditis can cause permanent cardiac damage. Sign of mild carditis disappear rapidly in weeks but severe carditis may last for 2 6 month. Chorea and arthritis usually subside without permanent damage

Management of RF
Benzathin penicillin G 0,6 1,2 M units i.m for eradication and prophylaxis. Allergic to penicillin : erythromisin 40 mg/kg/day in two to four doses for 10 days. Bed rest Acetosal for mild cases Prednison for severe cases Anti inflammatory agents not needed for isolated chorea Treatment of congestive heart failure

Recommended anti-inflammatory agents

Arthritis alone Mild carditis Moderate carditis Severe carditis

Prednison Aspirin

0 1-2 wk

0 3-4 wk#

0 6-8 wk

2-6 wk* 2-4mo

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

Bed rest and indoor ambulation

Arthritis alone Mild carditis Moderate carditis severe carditis

Bed rest Indoor amb

1-2 wk 1-2 wk

3-4 wk 3-4 wk 2-4 wk >6-10 wk

4-6 wk 4-6 wk 1-3 mo >3-6 mo

as long as CHF 2-3 mo 2-3 mo variable

Outdoor activ 2 wk (school) Full activity >4-6 wk

Mild card: Questionable cardiomegaly, moderate: definite but mild, Severe: marked cardiomegaly or CHF

ESR: important for duration of restriction of activities Full activity: ESR normal, excep significant cardiac involvement

Prevention

Ideally prophylaxis is indefinite Benzathin penicillin every 28 days, min till age 21 25 ys Sulfadiazine 0,5 g 1x daily ( bw < 27 kg) 1 g 1x daily (bw > 27 kg) Peniccilin V 2 x 250 mg/day Erythromycin 2 x 250 mg/day

RHEUMATIC HEART DISEASE

Affecs; - Mitral valve 75% - Aortic valve 25% - Tricuspid valve rare - Pulmonary valve never
Stenosis and regurgitation usually occur together.

MITRAL STENOSIS
prevalent most common valvular involvement in adult requires 5 10 ys from the initial attack if RF is prevalent, MS occurs under age 15 ys

Pathology
Thickening of the leaflets and fusion of the commisure Calsification result overtime LA and right-sides heart chambers become dilated and hipertrophied Pulmonary venous hypertension, pulmonary congestion and edema and fibrosis of the alveolar walls, hypertrophy of the pulmonary alveolar, loss of lung compliance

Aortic and mitral valve

Clinical manifestations
Mild MS : asymptomatic More severe: dyspnea with/out exertion, orthopneu, nocturnal dyspnea and palpitation.

Physical Examinations:
Increased RV impulse along LSB Weak peripheral pulse with narrow pulse pressure Pulmonary hypertension: loud S1 at apex and narrow split S2, accentuated P2 Mild diatolic/presystolic murmur

Cardiac finding of MS

ECG :- RAD, LAH, RVH ( due to PH ) - atrial fibrilation is rare in children

CXR : - enlarge LA and RV, - MPA segment prominent - pulmonary venous congestion

Echocardiography : accurate noninvasive tool to detection of MS M-mode; diminished E to F slope, thickened mitral leaflets, large LA dimention Two D : doming of thick mitral, a small mitral orifice, dilated LA, MPA, RV and RA Doppler: estimate of pressure gradient;Mitral valve and pulmonary valve.

Treatment of MS
prophylactic antibiotic restriction of activity depends on severity symptomatic patient ( dyspnea on exertion, pulmonary edema, paroxysmal dyspnea): balloon or surgery

Typical appearances of advanced MS on mediastinal organ and lung

MITRAL REGURGITATION
Most common in RHD Pathology: - mitral valve leaflets are shortened because of fibrosis. - when degree of MR increases, dilatation of LA and LV result, mitral ring becomes dilated.

Clinical manifestation: asymptomatic during chilhood rare; fatigue, palpitation

Physical examination heaving, hyperdinamic apical impuls in severe MR S1 normal or diminished. S2 may split ( shortening of LV ejection, early aortic closure ) S3 commonly is present and loud Pansystolic murmur at the apex, with transmision to the left axilla

ECG: - normal in mild cases -LVH or LV dominance, with or without LAH -Atrial fibrilation is rare in children CXR: -LA and LV enlarged -Pulmonary congestion pattern in CHF Echocardiography: -two D : dilated LA and LV -color-flow mapping; regurgitant jet into the LA -doppler: asses the severity of the regurgitation

Treatment: Prophylactic antibiotic No restriction of activity in mild cases Surgical: intractable CHF, progressive cardiomegaly, pulmonary hypertension If atrial fibrilation; digoxin Afterload-reducing agent; maintaining the forward stroke volume.

AORTIC REGURGITATION
Less common than MR. Mostly associated with mitral valve disease. Pathology semilunar cusps are deformed and shortened valve ring is dilated commisures usually are fused

Clinical manifestation

mild regurgitation: asymptomatic more severe; reduce exercise tolerance test

Physical examination

precordium may be hyperdinamic, distolic thrill at 3 LICS S1 decreased, S2 may be normal or single high 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

ECG : - normal in mild cases - severe ; LVH, LAH

CXR : - cardiomegaly (LVH) - dilated ascending aorta

Echocardiography : the LV dimension is increased color-flow and doppler to estimate the severe of the regurgitation.

Treatment:
prophylactic antibiotics mild case : no restriction in activity surgical : in anginal pain or dyspnea on exertion, significant cardiomegaly

Thank You