Cvs146 Slide Demam Rematik Dan Penyakit Jantung Rematik

DEMAM REMATIK DEMAM REMATIK dan dan

PENYAKIT JANTUNGPENYAKIT JANTUNGPENYAKIT JANTUNG PENYAKIT JANTUNG REMATIKREMATIKAbdullah Afif SiregarAbdullah Afif Siregar

Departemen Kardiologi dan Kedokteran VaskulerDepartemen Kardiologi dan Kedokteran VaskulerDepartemen Kardiologi dan Kedokteran Vaskuler Departemen Kardiologi dan Kedokteran Vaskuler Fakultas Kedokteran USU Fakultas Kedokteran USU

MedanMedan

Rheumatic feverRheumatic fever is an immunologically mediated is an immunologically mediated inflammatory disease that occurs as a delayedinflammatory disease that occurs as a delayedinflammatory disease, that occurs as a delayed inflammatory disease, that occurs as a delayed sequel to group A streptococcal throat infection, sequel to group A streptococcal throat infection, in genetically susceptible individuals.in genetically susceptible individuals.Rheumatic heart diseaseRheumatic heart disease is the most serious is the most serious complication of rheumatic fevercomplication of rheumatic feverA h i f d h i hA h i f d h i hAcute rheumatic fever and rheumatic heart Acute rheumatic fever and rheumatic heart diseasedisease are thought to result from an are thought to result from an autoimmune responseautoimmune response, but the exact, but the exactautoimmune responseautoimmune response, but the exact , but the exact pathogenesis remains unclear pathogenesis remains unclear

• The rheumatic fever follows 0.3% of cases of group A beta-hemolytic streptococcal pharyngitis in children. A 39% f ti t ith t h ti f• As many as 39% of patients with acute rheumatic fever may develop varying degrees of pancarditis with associated valve insufficiency, heart failure, pericarditis, and even death.

• With chronic rheumatic heart disease, patients develop valve stenosis with varying degrees of regurgitationvalve stenosis with varying degrees of regurgitation, atrial dilation, arrhythmias, and ventricular dysfunction.

• Chronic rheumatic heart disease remains the leading f it l l t i d l l t icause of mitral valve stenosis and valve replacement in

adults and children

Pathophysiology:Pathophysiology: • Rheumatic fever develops in children and adolescents following

pharyngitis with group A beta-hemolytic Streptococcus (ie, Streptococcus pyogenes)Streptococcus pyogenes).

• The organisms attach to the epithelial cells of the upper respiratory tract and produce a battery of enzymes allowing them to damage and invade human tissuesto damage and invade human tissues.

• After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response with 3-5 days of sore throat, f l i h d h d l t d l k t tfever, malaise, headache, and an elevated leukocyte count.

• In 0.3-3% of cases, infection leads to rheumatic fever several weeks after the sore throat has resolved. Only infections of the h i i i i h i fpharynx initiate or reactivate rheumatic fever.

• The organism spreads by direct contact with oral or respiratory secretions, and spread is enhanced by crowded living conditions.

Th th i h i i l d i th d l t f RF i

Etiopathogenesis :

• The pathogenic mechanisms involved in the development of RF remain unclear. But it is evident that an abnormal humoral and cellular immune response occurs.

• Antigenic mimicry between streptococcal antigens mainly M protein• Antigenic mimicry between streptococcal antigens, mainly M-protein epitopes and human tissues, such as heart valves, myosin and tropomyosin, brain proteins, synovial tissue and cartilage has been proposed as the triggering factor leading to autoimmunity in individualsproposed as the triggering factor leading to autoimmunity in individuals with genetic predisposition.

• Several genetic markers of susceptibility have been studied but no consistent association found. Associations with different HLA class II antigens have been observed in several populations.

• Molecular mimicry was first demonstrated by humoral immune response. Streptococcal antibodies cross-react with several human tissues including heart, skin, brain, glomerular basement membrane, striated and smooth muscles.

• The presence of CD4+ T cells at lesions sites in the heart has been demonstrated s ggesting a direct role of these cells in the pathogenesis ofdemonstrated, suggesting a direct role of these cells in the pathogenesis of RHD.

Etiopathogenesis :

• Infiltrating T lymphocytes from heart lesions of severe RHDheart lesions of severe RHD patients and peripheral T lymphocytes were capable of recognising immunodominantrecognising immunodominant myocardium M5 peptides and valve proteins. These results showed the significance of gmolecular mimicry between beta hemolytic streptococci and heart tissue assessing the T-cell

Fi 1 S h ti t ti

repertoire leading to local tissue damage in RHD.

Figure 1: Schematic representation of the aetiopathogenic events occurring during the development of carditis

DIAGNOSIS :

Sambungan Tabel 4.1

Carditis (40% )

Carditis (40% )

Clinical picture of carditis : • The clinical picture includes high pulse rate, congestive

heart failure arrhytmias and pericardial friction rubsheart failure, arrhytmias and pericardial friction rubs. • On the first attack, valvulitis is suspected in the presence

of a new apical systolic murmur of mitral regurgitation(associated or not with an apical mid-diastolic murmur) and/or a basal diastolic murmur of aortic regurgitation.

• Cardiomegaly is noted on X-Ray and on echocardiogram. g y y g• Myocarditis and/or pericarditis in the absence of valvular

involvement is unlikely due to acute RF

Polyarthritis (75%)Polyarthritis (75%)

• Arthritis is the most common manifestation, present in 60-80% of patientspresent in 60-80% of patients.

• It usually affects the peripheral large joints; small joints and axial skeleton are rarely involved.involved.

• Knees, ankles, elbows and wrists are the most frequently affected. The joints are red, warm and swollen.

• Arthritis is characteristically asymmetrical, migratory, and very painful, although some patients may present mild joint complaints. It usually resolves spontaneously at the most in 2 or 3 weeks.

• Arthritis in ARF has an excellent response to salicylatessalicylates

Sydenham ChoreaSydenham Chorea ::

Sydenham’s choreaSydenham’s chorea is is characterized by characterized by involuntary involuntary movements specially of the facemovements specially of the facemovements, specially of the face movements, specially of the face and limbs, muscle weakness, and limbs, muscle weakness, disturbances of speech and gait. disturbances of speech and gait. ChildrenChildren usually exhibitusually exhibitChildrenChildren usually exhibit usually exhibit concomitant concomitant psychologic psychologic dysfunctiondysfunction, especially , especially obsessi eobsessi e comp lsi e diso decomp lsi e diso deobsessiveobsessive--compulsive disorder, compulsive disorder, increased emotional lability, increased emotional lability, hyperactivity, irritablility and hyperactivity, irritablility and

d b h id b h iageage--regressed behavior. regressed behavior. It is usually a It is usually a delayed delayed manifestationmanifestation, and is often the , and is often the sole manifestation of ARF. sole manifestation of ARF.

Erythema marginatumErythema marginatum ::This is an evanescent, erythematous, nonThis is an evanescent, erythematous, non--pruritic rash with pruritic rash with

l d d dl d d dpale centers and rounded or serpiginous margins. Lesions pale centers and rounded or serpiginous margins. Lesions occur mainly on the trunk and proximal extremities and occur mainly on the trunk and proximal extremities and may be induced by application of heat may be induced by application of heat y y ppy y pp

Diagnosis :Diagnosis :gg

Based on Jones Criteria, 1992 Update : Based on Jones Criteria, 1992 Update : -- 22 Major criteria +Major criteria + 11 Minor criteriaMinor criteria oror22 Major criteria + Major criteria + 11 Minor criteria, Minor criteria, oror

-- 11 Major criteria + Major criteria + 22 minor criteriaminor criteria** ff* * plus supporting evidence of preceding GAS plus supporting evidence of preceding GAS

infection infection

Table : Differential diagnosis of rheumatic fever

Juvenile rheumatoid arthritisJuvenile rheumatoid arthritis

Systemic lupus erythematosusSystemic lupus erythematosus

f i d di if i d di iInfective endocarditis Infective endocarditis

Reactive arthritis Reactive arthritis

Sickle cell diseaseSickle cell disease

Drug reactionsDrug reactions

Other connective tissue diseasesOther connective tissue diseases

SepticaemiaSepticaemiaSepticaemiaSepticaemia

LeukaemiaLeukaemia

Gonoccocal arthritisGonoccocal arthritis

T b l iT b l iTuberculosisTuberculosis

Lyme diseaseLyme disease

Serum sicknessSerum sickness

Medical therapy involves the following 5 areas:

Treatment :Medical therapy involves the following 5 areas:1. Treat group A streptococcal infection regardless of organism

detection. 2 Steroids and salicylates are useful in the control of pain and2. Steroids and salicylates are useful in the control of pain and

inflammation. The nonsteroidal anti-inflammatory drug (NSAID) naproxen has also been studied. It is effective and may be easier to use than aspirinto use than aspirin.

3. Heart failure may require digitalis. 4. Administer prophylaxis to patients who have developed ARF.

Patients with ARF should receive prophylaxis against futurePatients with ARF should receive prophylaxis against future GABHS infections. Available regimens include benzathine penicillin G 1.2 million U IM every month, penicillin V 200,000 U or 250 mg PO bid or erythromycin 250 mg PO bid Mostor 250 mg PO bid, or erythromycin 250 mg PO bid. Most authorities suggest that prophylaxis be given for 5 years. For those who have rheumatic carditis, some authorities suggest life-l h l ilong prophylaxis.

5. Haloperidol may be helpful in controlling chorea.

Drug NameDrug Name Penicillin G benzathinePenicillin G benzathine(Bicillin LA)(Bicillin LA)

Penicillin G procainePenicillin G procaine(Crysticillin, Wycillin) (Crysticillin, Wycillin)

Penicillin VKPenicillin VK (Beepen(Beepen--VK, VK, BetapenBetapen--VK, Robicillin VK, Veetids) VK, Robicillin VK, Veetids)

DescriptionDescription Interferes with synthesis of Interferes with synthesis of cell wall mucopeptide duringcell wall mucopeptide during

LongLong--acting parenteral penicillin acting parenteral penicillin (IM only) indicated in the(IM only) indicated in the

Inhibits the biosynthesis of the cellInhibits the biosynthesis of the cell--wall mucopeptide and is effectivewall mucopeptide and is effectivecell wall mucopeptide during cell wall mucopeptide during

active multiplication resulting active multiplication resulting in bactericidal activity against in bactericidal activity against susceptible bacteria.susceptible bacteria.Because of its prolonged blood Because of its prolonged blood level, several authors believe level, several authors believe

(IM only) indicated in the (IM only) indicated in the treatment of moderately severe treatment of moderately severe infections caused by penicillin infections caused by penicillin GG--sensitive microorganisms.sensitive microorganisms.Some prefer 10Some prefer 10--d therapy.d therapy.Administer by deep IM injection Administer by deep IM injection

wall mucopeptide and is effective wall mucopeptide and is effective during the stage of active during the stage of active multiplication. Inadequate multiplication. Inadequate concentrations may produce only concentrations may produce only bacteriostatic effects. Penicillin VK bacteriostatic effects. Penicillin VK is the oral alternative for the is the oral alternative for the ,,

this to be the DOC. Others this to be the DOC. Others prefer daily injections.prefer daily injections.

y p jy p jonly into the upper outer only into the upper outer quadrant of the buttock. quadrant of the buttock.

treatment of rheumatic fever. treatment of rheumatic fever.

Adult DoseAdult Dose 2.4 million U IM once 2.4 million U IM once 500 mg PO q6h for 10 d

Pediatric DosePediatric Dose Infants and children <30 Infants and children <30 lb: <12 years: 25-50 mg/kg/d PO lb: 600,000 U IM onceChildren 30-60 lb: 900,000 to 1.2 million U IM once

600,000 U IMChildren 30-60 lb: 900,000 to 1.2 million U IM

divided tid/qid; not to exceed 3 g/d >12 years: Administer as in adults

ContraindicationsContraindications Documented hypersensitivityDocumented hypersensitivity Documented hypersensitivityDocumented hypersensitivity Documented hypersensitivity Documented hypersensitivity

InteractionsInteractions Probenecid can increaseProbenecid can increase Increases risk of bleeding whenIncreases risk of bleeding when Probenecid may increaseProbenecid may increaseInteractionsInteractions Probenecid can increase Probenecid can increase penicillin effectiveness by penicillin effectiveness by decreasing its clearance; decreasing its clearance; coadministration of coadministration of tetracyclines can decrease tetracyclines can decrease penicillin effectivenesspenicillin effectiveness

Increases risk of bleeding when Increases risk of bleeding when administered concurrently with administered concurrently with warfarin; ethacrynic acid, warfarin; ethacrynic acid, aspirin, indomethacin, and aspirin, indomethacin, and furosemide may compete with furosemide may compete with penicillin G for renal tubularpenicillin G for renal tubular

Probenecid may increase Probenecid may increase effectiveness by decreasing effectiveness by decreasing clearance; tetracyclines are clearance; tetracyclines are bacteriostatic, causing a decrease bacteriostatic, causing a decrease in the effectiveness of penicillins in the effectiveness of penicillins

penicillin effectivenesspenicillin effectiveness penicillin G for renal tubular penicillin G for renal tubular secretion increasing penicillin secretion increasing penicillin serum concentrations serum concentrations

when administered concurrentlywhen administered concurrently

PregnancyPregnancy B B -- Usually safe but benefits Usually safe but benefits must outweigh the risks.must outweigh the risks.

B B -- Usually safe but benefits Usually safe but benefits must outweigh the risks.must outweigh the risks.

B B -- Usually safe but benefits must Usually safe but benefits must outweigh the risks outweigh the risks

C i i i i d lC i i i i d lPrecautionsPrecautions Caution in impaired renal Caution in impaired renal functionfunction

Never use IV route to adminis Never use IV route to adminis ter penicillin G procaine; admi ter penicillin G procaine; admi nister >10 d to eliminate orga nister >10 d to eliminate orga nism and prevent complications. nism and prevent complications.

Caution in renal impairment Caution in renal impairment

Drug NameDrug Name Erythromycin (EES, E-Mycin, Ery-Tab, Erythrocin)

DescriptionDescription DOC for patients allergic to penicillin; inhibits RNADOC for patients allergic to penicillin; inhibits RNA--dependent protein dependent protein synthesis, possibly by stimulating the dissociation of peptidyl tsynthesis, possibly by stimulating the dissociation of peptidyl t--RNA RNA from ribosomes, which inhibits bacterial growth.from ribosomes, which inhibits bacterial growth.In children, age, weight, and the severity of infection determine the In children, age, weight, and the severity of infection determine the proper dosage When bid dosing is desired oneproper dosage When bid dosing is desired one--half the daily dosehalf the daily doseproper dosage. When bid dosing is desired, oneproper dosage. When bid dosing is desired, one half the daily dose half the daily dose may be administered q12h. For more severe infections, the dose may may be administered q12h. For more severe infections, the dose may be doubled.be doubled.

Adult DoseAdult Dose 1 g/d PO divided bid for 10 d

Pediatric DosePediatric Dose 30-50 mg/kg/d PO divided bid

ContraindicationsContraindications Documented hypersensitivity; hepatic impairment Documented hypersensitivity; hepatic impairment

InteractionsInteractions Coadministration may increase toxicity of theophylline, digoxin, Coadministration may increase toxicity of theophylline, digoxin, carbamazepine, and cyclosporine; may potentiate anticoagulant carbamazepine, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin, effects of warfarin; coadministration with lovastatin and simvastatin, increases risk of rhabdomyolysis increases risk of rhabdomyolysis

PregnancyPregnancy B B -- Usually safe but benefits must outweigh the risks.Usually safe but benefits must outweigh the risks.

PrecautionsPrecautions Caution in liver disease; estolate formulation may cause cholestatic Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc);jaundice; GI adverse effects are common (give doses pc);jaundice; GI adverse effects are common (give doses pc); jaundice; GI adverse effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur fever occur

Drug Category: Glucocorticoids

Drug NameDrug Name Prednisone (Deltasone Sterapred)Drug NameDrug Name Prednisone (Deltasone, Sterapred)

DescriptionDescription

Patients with carditis require prednisone instead of aspirin. The goal is to decrease myocardial inflammation.Useful in treatment of inflammatory and autoimmune disorders. R i i d ill bilit d i PMN ti itReversing increased capillary permeability and suppressing PMN activity may decrease inflammation.

Adult DoseAdult Dose 60-80 mg/d PO

Pediatric DosePediatric Dose 2 mg/kg/d POPediatric DosePediatric Dose 2 mg/kg/d PO

ContraindicationsContraindications Documented hypersensitivity; viral, fungal, or tubercular skin infections

Coadministration with estrogens may decrease clearance; concurrent use with digoxin, may cause digitalis toxicity secondary to hypokalemia;

InteractionsInteractionsg , y g y y yp ;

phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

PregnancyPregnancy B - Usually safe but benefits must outweigh the risks.PregnancyPregnancy

PrecautionsPrecautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Anti inflammatory.

Drug NameDrug Name Aspirin (Ascriptin Bayer Buffered Aspirin Ecotrin)Drug NameDrug Name Aspirin (Ascriptin, Bayer Buffered Aspirin, Ecotrin)

DescriptionDescription Treats mild to moderate pain. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.

Adult DoseAdult Dose 6-8 g/d PO for 2 mo or until ESR has returned to normal

Pediatric DosePediatric Dose 80-100 mg/kg/d PO for 2 mo or until ESR has returned to normal

Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K ContraindicationsContraindications

yp y; g , yp p ,deficiency, bleeding disorders, asthma; because of association with Reye syndrome, do not use in children ( <16 y) with flu

Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and

InteractionsInteractions

decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose lowering effect of sulfonylurea drugs

PregnancyPregnancy C - Safety for use during pregnancy has not been established.


Pregnancy category D if full dose given during third trimester; may cause transient decrease in renal function and aggravate chronic kidney disease; PrecautionsPrecautions avoid use in patients with severe anemia, with history of blood coagulation defects, or taking anticoagulants

Drug Category: Neuroleptic agentsMay help to control the chorea associated with ARF.

Drug NameDrug Name Haloperidol (Haldol)Drug NameDrug Name Haloperidol (Haldol)

DescriptionDescription A dopamine receptor blocker useful in the treatment of irregular spasmodic movements of limbs or facial muscles.

Adult DoseAdult Dose 0.5-2 mg PO bid/tidg /

Pediatric DosePediatric Dose

<3 years: Not established3-12 years: 0.05 mg/kg/d or 0.25-0.5 mg/d bid/tid; increase by 0.25-0.5 mg q5-7dMaintenance dose: 0.05-0.15 mg/kg/d bid/tid; not to exceed 0.15 mg/kg/d>12 years: Administer as in adults

ContraindicationsContraindications Documented hypersensitivity; narrow-angle glaucoma; bone marrow suppression; severe cardiac and liver disease; severe hypotension; subcortical brain damage


May increase tricyclic antidepressant serum-concentrations and hypotensive action of antihypertensive agents; phenobarbital or carbamazepine may decrease effects; coadministration with anticholinergics may increase intraocular pressure; encephalopathy-like syndrome associated with concurrent administration of lithium and haloperidoland haloperidol


Severe neurotoxicity manifesting as rigidity, or inability to walk or talk may occur in

PrecautionsPrecautionspatients with thyrotoxicosis also receiving antipsychotics; if IV/IM, watch for hypotension; caution in CNS depression or cardiac disease; if history of seizures, benefits must outweigh risks; significant increase in body temperature may indicate intolerance to antipsychotics (discontinue if this occurs)

Drug Category: Inotropic agentsSome believe that digoxin may be helpful in congestive heart failure.

Drug NameDrug Name Digoxin (Lanoxin)

DescriptionDescription

Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system.Effects on the myocardium involve a direct action on cardiac muscle that increases myocardial systolic contractions and indirect actions that result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Adult DoseAdult Dose 0 125-0 375 mg PO qdAdult DoseAdult Dose 0.125-0.375 mg PO qd

Pediatric DosePediatric Dose

Digitalizing dose:2-5 years: 30-40 mcg/kg PO5-10 years: 20-35 mcg/kg PO>10 years: 10-15 mcg/kg POM i t d 25 35% f PO l di dMaintenance dose: 25-35% of PO loading dose

ContraindicationsContraindications Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid sinus syndrome

Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine propafenone propantheline quinidine diltiazem aminoglycosides oral amiodarone


cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, anti histamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins,hypo glycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotre g y g , p ( g , y ,xate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid


Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias ;


yp y p p g y p yhypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective; magnesium replacement therapy must be instituted in patients with hypomagnesemia; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis

Table : Secondary prevention of rheumatic fever.

AgentAgent Therapeutic SchemeTherapeutic SchemeBenzathineBenzathine penicillin Gpenicillin G 1,200,000 U every 4 weeks*, IM1,200,000 U every 4 weeks*, IM

orororor

Penicillin VPenicillin V 250mg twice daily, PO250mg twice daily, POoror

SulfadiazineSulfadiazine 500mg once daily for patients < 27kg; 1g once daily500mg once daily for patients < 27kg; 1g once dailySulfadiazineSulfadiazine 500mg once daily for patients < 27kg; 1g once daily 500mg once daily for patients < 27kg; 1g once daily for patients > 27kg, POfor patients > 27kg, PO

For individuals allergic to penicillin and sulfadiazine:For individuals allergic to penicillin and sulfadiazine:

ErythromycinErythromycin 250mg twice daily PO250mg twice daily POErythromycinErythromycin 250mg twice daily, PO250mg twice daily, PO

*In high-risk situations, administration every 3 weeks is recommended.

Table. Guidelines for Bed Rest and Ambulation and Recommended antiinflammatory agents

Arthritis Carditis Carditis Carditisalone minimal moderate severe

Bed Rest 1-2 wk 2-3 wk 4-6 wk 2-4 mo

I d b l ti 1 2 k 2 3 k 4 6 k 2 3Indoor ambulation 1-2 wk 2-3 wk 4-6 wk 2-3 mo

Outdor activity 1-2 wk 2-3 wk 4-6 wk 2-3 mo(school)( )

Full activity 1-2 wk 2-3 wk 4-6 wk 2-3 mo

Prednisone 0 0 2 4 wk 2 6 wkPrednisone 0 0 2-4 wk 2-6 wkAspirin 0 0 2-4 wk 2-6 wk

Minimal Carditis Questionable cardiomegaly ; Moderate carditis definite but mild cardiomegaly, Severe carditis, marked cardiomegaly or CHF

ComplicationsCarditisCarditis Mitral stenosis Congestive heart failure (CHF)Congestive heart failure (CHF)

PrognosisSequelae are limited to the heart and are qdependent upon the severity of the carditis during the acute attack.

.

Rheumatic Heart DiseaseRheumatic Heart Disease

Rheumatic heart disease is the most serious complication Rheumatic heart disease is the most serious complication of rheumatic fever. of rheumatic fever. A t h ti f f llA t h ti f f ll 0 3%0 3% f f Af f AAcute rheumatic fever follows Acute rheumatic fever follows 0.3%0.3% of cases of group A of cases of group A betabeta--hemolytic streptococcal pharyngitis in children. As hemolytic streptococcal pharyngitis in children. As many as many as 39%39% of patients with acute rheumatic fever of patients with acute rheumatic fever may develop varying degrees of pancarditis withmay develop varying degrees of pancarditis withmay develop varying degrees of pancarditis with may develop varying degrees of pancarditis with associated associated valve insufficiency, heart failure, pericarditis, valve insufficiency, heart failure, pericarditis, and even death.and even death.WithWith chronic rheumatic heart diseasechronic rheumatic heart disease patients developpatients developWith With chronic rheumatic heart diseasechronic rheumatic heart disease, patients develop , patients develop valve stenosisvalve stenosis with varying degrees of with varying degrees of regurgitation,regurgitation,atrial dilation, arrhythmiasatrial dilation, arrhythmias, and , and ventricular dysfunctionventricular dysfunction. . Chronic rheumatic heart disease remains the leading Chronic rheumatic heart disease remains the leading ggcause of mitral valve stenosis and valve replacement in cause of mitral valve stenosis and valve replacement in adults adults

Frequency:In the US:Prevalence of rheumatic heart disease in the United States now is less than 0.05 per 1000 populationInternationally:Th i id f h i f d h i h di hThe incidence of rheumatic fever and rheumatic heart disease has not decreased in developing countries. Retrospective studies reveal developing countries to have the highest figures for cardiac involvement and recurrence rates of rheumatic fever Estimations worldwide are that 5 30 million childrenrates of rheumatic fever. Estimations worldwide are that 5-30 million children and young adults have chronic rheumatic heart disease, and 90,000 patients die from this disease each year. There were no data available in Indonesia

Mortality/Morbidity:Rheumatic heart disease is the major cause of morbidity fromRheumatic heart disease is the major cause of morbidity from rheumatic fever and the major cause of mitral insufficiency and stenosis in the Indonesia and the world. Variables that correlate with severity of valve disease include the ynumber of previous attacks of rheumatic fever, the length of time between the onset of disease and start of therapy, and sex. (The disease is more severe in females than in males.) Insufficiency from acute rheumatic valve disease resolves in 60-80% of patients who adhere to antibiotic prophylaxis. Race: The race (when controlled for socioeconomic variables) has not been documented to influence disease incidence. Sex:Rheumatic fever occurs in equal numbers in males and females, but the prognosis is worse for females than for males.

Age:Age: Rheumatic fever is principally a disease of childhood, with a median age of 10 years, although it also occurs in d lt (20% f )

Socio-economic factors :

adults (20% of cases).

It is well known that socioeconomic and environmental factors play an indirect, but important, role in the magnitude

f S f fand severity of RF and RHD. Such factor as a shortage of resources for providing quality health care, inadequate expertise of health-care providers, and a low level of p p ,awareness of the disease in the community can all impact the expression of the disease in populations. Crowding adversely affects rheumatic fever incidenceadversely affects rheumatic fever incidence

Cardiac manifestations of chronic rheumatic heart disease :• Valve deformities, • thromboembolism, • cardiac hemolytic anemia, and • atrial arrhythmias• atrial arrhythmiasare the most common cardiac manifestations of chronic rheumatic heart disease.

Valve deformities• Mitral stenosis – Mitral regurgitasi occurs in 25% of patients

with chronic rheumatic heart disease and in association with mitral insufficiency in another 40%.

• Aortic regurgitasi – Aortic stenosis are typically from chronic g g yp yrheumatic heart disease. The valve commissures and cusps become adherent and fused, and the valve orifice becomes small with a round or triangular shape. g p

• Thromboembolism occurs as a complication of mitral t i It i lik l t h th l ft t istenosis. It is more likely to occur when the left atrium

is dilated, cardiac output is decreased, and the patient is in atrial fibrillation.patient is in atrial fibrillation.

• Cardiac hemolytic anemia is related to disruption of the red blood cells by a deformed valve. Increased d t ti d l t f l t l t ldestruction and replacement of platelets also may occur.

• Atrial arrhythmias typically are related to a chronicallyAtrial arrhythmias typically are related to a chronically enlarged left atrium (from a mitral valve abnormality). Successful cardioversion of atrial fibrillation to sinus rhythm is more likely to be successful if the left atrium is not markedly enlarged, the mitral stenosis is mild, and the patient has been in atrial fibrillation for lessand the patient has been in atrial fibrillation for less than 6 months.

TREATMENTTREATMENT

M di l CM di l CMedical CareMedical Care::1.1. Medical therapy is directed toward Medical therapy is directed toward eliminating the group eliminating the group

A strepto coccal pharyngitisA strepto coccal pharyngitisA strepto coccal pharyngitis A strepto coccal pharyngitis 2.2. Treatment of the Treatment of the acute inflammatory manifestationsacute inflammatory manifestations of of

acute rheumatic fever consists of administering acute rheumatic fever consists of administering li l t d t idli l t d t idsalicylates and steroidssalicylates and steroids. .

3.3. If If moderatemoderate--toto--severe carditissevere carditis is indicated by is indicated by cardiomegaly, congestive heart failure, or thirdcardiomegaly, congestive heart failure, or third--degree degree g y, g ,g y, g , ggheart block, heart block, oral prednisone should be added to oral prednisone should be added to salicylate therapysalicylate therapy. .

44 Preventive and prophylactic therapy is indicated afterPreventive and prophylactic therapy is indicated after4.4. Preventive and prophylactic therapy is indicated after Preventive and prophylactic therapy is indicated after rheumatic fever and rheumatic heart diseaserheumatic fever and rheumatic heart disease to prevent to prevent further damage to valves. further damage to valves.

TREATMENTTREATMENT

Surgical Care:Surgical Care:When heart failure persists or worsens after aggressive When heart failure persists or worsens after aggressive medical therapy for acute rheumatic heart diseasemedical therapy for acute rheumatic heart diseasemedical therapy for acute rheumatic heart disease, medical therapy for acute rheumatic heart disease, surgery to decrease valve insufficiency may be lifesurgery to decrease valve insufficiency may be life--saving. saving. Forty percent of patients with acute rheumatic feverForty percent of patients with acute rheumatic feverForty percent of patients with acute rheumatic fever Forty percent of patients with acute rheumatic fever subsequently develop mitral stenosis as adults.subsequently develop mitral stenosis as adults.In patients with critical stenosis, mitral valvulotomy, In patients with critical stenosis, mitral valvulotomy, percutaneous balloon valvuloplasty, or mitral valve percutaneous balloon valvuloplasty, or mitral valve p p y,p p y,replacementreplacement may be indicated.may be indicated.Due to high rates of recurrent symptoms after Due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve annuloplasty or other repair procedures, valve p y p p ,p y p p ,replacement appears to be the preferred surgical option.replacement appears to be the preferred surgical option.

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