8/8/2019 PJR Anak

1/54

DEMAM REMATIK AKUT

PENYAKIT JANTUNG REMATIK

dr. Lilia Dewiyanti, SpA, MSiMed.

Fakultas Kedokteran Universitas Muhammadiyah Semarang

8/8/2019 PJR Anak

2/54

1. ImmunologicStreptococcus beta hemolytic group A

2. Predispositing Factor:- Family history- Socio-economic status- Age 5 15 years ( peak 8 years)

Etiologi

8/8/2019 PJR Anak

3/54

8/8/2019 PJR Anak

4/54

Inflamatory lesion; heart,brain,joint,skin

Ashoff bodies ( in atrial myocardium):characteristic ?Central necrosis surrounded by lymphocytes,

plasma cell, and large mononuclear and giantmultinucleate cell

Patogenesis

8/8/2019 PJR Anak

5/54

HistoryStreptococcal pharyngitis, 1 5 weeks (ave 3) beforeonset.

Chorea 2 6 mos.Pallor, easy fatigability, epistaxis, abdominal painPositive family history.

Strep throat

Rheumatic fever

Acute glomerulonephritis

Strep skin

Manifestasi Klinik

8/8/2019 PJR Anak

6/54

8/8/2019 PJR Anak

7/54

Histologic section of typical RF

8/8/2019 PJR Anak

8/54

8/8/2019 PJR Anak

9/54

Jones criteria (1992)

o Major criteria :

- arthritis

- carditis- erythema marginatum- subcutaneous nodules- Sydenhams chorea.

o Minor criteria :

- arthalgia- fever

- elevated acute phasereactan ( CRP, ESR)

- ECG : PR interval > :not specific

plusSupporting evident of antecedent Strep group A infec.

- culture (+) or rapid strept antigent test- elevated or rising ASTO

8/8/2019 PJR Anak

10/54

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 4-6 wk >6-10 wk >3-6 mo variable

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

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

8/8/2019 PJR Anak

31/54

Prevention

Ideally prophylaxis is indefinite Benzathin penicillin every 28 days, min tillage 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

8/8/2019 PJR Anak

32/54

RH

EUMATICH

EART DISEASEAffecs; - Mitral valve 75%

- Aortic valve 25%- Tricuspid valve rare

- Pulmonary valve never

Stenosis and regurgitation usually occurtogether.

8/8/2019 PJR Anak

33/54

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

8/8/2019 PJR Anak

34/54

Pathology Thickening of the leaflets and fusion of the

commisure Calsification result overtime

LA and right-sides heart chambers become dilatedand hipertrophied

Pulmonary venous hypertension, pulmonary congestionand edema and fibrosis of the alveolar walls,

hypertrophy of the pulmonary alveolar, loss of lungcompliance

8/8/2019 PJR Anak

35/54

Aortic and mitral valve

8/8/2019 PJR Anak

36/54

8/8/2019 PJR Anak

37/54

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 narrowsplit S2, accentuated P2

Mild diatolic/presystolic murmur

8/8/2019 PJR Anak

38/54

Cardiac finding ofMS

8/8/2019 PJR Anak

39/54

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;Mitralvalve and pulmonary valve.

8/8/2019 PJR Anak

40/54

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

or surgery

8/8/2019 PJR Anak

41/54

Typical appearances of advanced MS on mediastinal

organ and lung

8/8/2019 PJR Anak

42/54

8/8/2019 PJR Anak

43/54

MITRAL REGURGITATION

Most common in RHD

Pathology:- mitral valve leaflets are shortened because offibrosis.

- when degree of MR increases, dilatation of LAand LV result, mitral ring becomes dilated.

8/8/2019 PJR Anak

44/54

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, earlyaortic closure )

S3 commonly is present and loud Pansystolic murmur at the apex, with transmisionto the left axilla

8/8/2019 PJR Anak

45/54

8/8/2019 PJR Anak

46/54

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

8/8/2019 PJR Anak

47/54

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.

8/8/2019 PJR Anak

48/54

AORTIC REGURGITATION

Less common than MR. Mostly associated withmitral valve disease.

Pathology semilunar cusps are deformed and shortened valve ring is dilated commisures usually are fused

8/8/2019 PJR Anak

49/54

Clinical manifestation

mild regurgitation: asymptomatic more severe; reduce exercise tolerance test

Physical examination

precordium may be hyperdinamic, distolic thrillat 3 LICS

S1 decreased, S2 may be normal or single high pitched diastolic cresendo murmur at 3 LICSor 4 LICS

systolic murmur at 2 RICS due to relative AS severe AS : middiastolic murmur at apex

8/8/2019 PJR Anak

50/54

8/8/2019 PJR Anak

51/54

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 severeof the regurgitation.

8/8/2019 PJR Anak

52/54

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

exertion, significant cardiomegaly

8/8/2019 PJR Anak

53/54

Thank You

8/8/2019 PJR Anak

54/54

Thank You