k - 7 demam rematik (kardiologi)

DEMAM REMATIK DEMAM REMATIK dan dan

PENYAKIT JANTUNG PENYAKIT JANTUNG REMATIKREMATIK

Abdullah Afif SiregarAbdullah Afif SiregarDepartemen Kardiologi dan Kedokteran Departemen Kardiologi dan Kedokteran

Vaskuler Vaskuler Fakultas Kedokteran USU Fakultas Kedokteran USU

MedanMedan

Rheumatic feverRheumatic fever is an immunologically is an immunologically mediated inflammatory disease, that mediated inflammatory disease, that occurs as a delayed sequel to group A occurs as a delayed sequel to group A streptococcal throat infection, in streptococcal throat infection, in genetically susceptible individuals.genetically susceptible individuals.

Rheumatic heart diseaseRheumatic heart disease is the most is the most serious complication of rheumatic feverserious complication of rheumatic fever

Acute rheumatic fever and rheumatic Acute rheumatic fever and rheumatic heart diseaseheart disease are thought to result from are thought to result from an an autoimmune 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. • 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 regurgitation, atrial dilation, arrhythmias, and ventricular dysfunction. • Chronic rheumatic heart disease remains the leading cause of mitral valve stenosis and valve replacement in adults and children

Pathophysiology: • Rheumatic fever develops in children and adolescents following pharyngitis with group A beta-hemolytic Streptococcus (ie, 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 tissues. • After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response with 3-5 days of sore throat, fever, 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 pharynx initiate or reactivate rheumatic fever. • The organism spreads by direct contact with oral or respiratory secretions, and spread is enhanced by crowded living conditions.

• 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 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 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, suggesting a direct role of these cells in the pathogenesis of RHD.

Etiopathogenesis :

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

• Infiltrating T lymphocytes from heart lesions of severe RHD patients and peripheral T lymphocytes were capable of recognising immunodominant myocardium M5 peptides and valve proteins. These results showed the significance of molecular mimicry between beta hemolytic streptococci and heart tissue assessing the T-cell repertoire leading to local tissue damage in RHD.

Etiopathogenesis :

DIAGNOSIS :

Sambungan Tabel 4.1

Carditis (40% )

Clinical picture of carditis : • The clinical picture includes high pulse rate, congestive heart 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. • Myocarditis and/or pericarditis in the absence of valvular involvement is unlikely due to acute RF

Carditis (40% )

Polyarthritis (75%)Polyarthritis (75%)

• Arthritis is the most common manifestation, present in 60-80% of patients.• It usually affects the peripheral large joints; small joints and axial skeleton are rarely 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 salicylates

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Sydenham Sydenham ChoreaChorea : :

Sydenham’s choreaSydenham’s chorea is is characterized by characterized by involuntary involuntary movements, specially of the movements, specially of the face and limbs, muscle face and limbs, muscle weakness, disturbances of weakness, disturbances of speech and gait. speech and gait.

ChildrenChildren usually exhibit usually exhibit concomitant concomitant psychologic psychologic dysfunctiondysfunction, especially , especially obsessive-compulsive obsessive-compulsive disorder, increased disorder, increased emotional lability, emotional lability, hyperactivity, irritablility and hyperactivity, irritablility and age-regressed behavior. age-regressed behavior.

It is usually a It is usually a delayed delayed manifestationmanifestation, and is often , and is often the sole manifestation of the sole manifestation of ARF. ARF.



Erythema marginatumErythema marginatum : :This is an evanescent, erythematous, non-pruritic This is an evanescent, erythematous, non-pruritic rash with pale centers and rounded or serpiginous rash with pale centers and rounded or serpiginous margins. Lesions occur mainly on the trunk and margins. Lesions occur mainly on the trunk and proximal extremities and may be induced by proximal extremities and may be induced by application of heat application of heat

Diagnosis Diagnosis ::

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

- - 11 Major criteria + Major criteria + 22 minor criteria minor criteria

* * 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 Infective endocarditis Infective endocarditis Reactive arthritis Reactive arthritis Sickle cell diseaseSickle cell disease Drug reactionsDrug reactions Other connective tissue Other connective tissue

diseasesdiseases SepticaemiaSepticaemia LeukaemiaLeukaemia Gonoccocal arthritisGonoccocal arthritis TuberculosisTuberculosis Lyme diseaseLyme disease Serum sicknessSerum sickness

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 and

inflammation. The nonsteroidal anti-inflammatory drug (NSAID) naproxen has also been studied. It is effective and may be easier to 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 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. Most authorities suggest that prophylaxis be given for 5 years. For those who have rheumatic carditis, some authorities suggest life-long prophylaxis.

5. Haloperidol may be helpful in controlling chorea.

Treatment :

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-VK, (Beepen-VK, Betapen-VK, Robicillin VK, Betapen-VK, Robicillin VK, Veetids) Veetids)

DescriptionDescription Interferes with synthesis of Interferes with synthesis of cell wall mucopeptide cell wall mucopeptide during active multiplication during active multiplication resulting in bactericidal resulting in bactericidal activity against susceptible activity against susceptible bacteria.bacteria.Because of its prolonged Because of its prolonged blood level, several blood level, several authors believe this to be authors believe this to be the DOC. Others prefer the DOC. Others prefer daily injections.daily injections.

Long-acting parenteral Long-acting parenteral penicillin (IM only) indicated penicillin (IM only) indicated in the treatment of in the treatment of moderately severe moderately severe infections caused by infections caused by penicillin G-sensitive penicillin G-sensitive microorganisms.microorganisms.Some prefer 10-d therapy.Some prefer 10-d therapy.Administer by deep IM Administer by deep IM injection only into the upper injection only into the upper outer quadrant of the outer quadrant of the buttock. buttock.

Inhibits the biosynthesis of the Inhibits the biosynthesis of the cell-wall mucopeptide and is cell-wall mucopeptide and is effective during the stage of effective during the stage of active multiplication. active multiplication. Inadequate concentrations Inadequate concentrations may produce only may produce only bacteriostatic effects. Penicillin bacteriostatic effects. Penicillin VK is the oral alternative for VK is the oral alternative for the treatment of rheumatic the treatment of rheumatic fever. fever.

Adult DoseAdult Dose 2.4 million U IM once2.4 million U IM once 2.4 million U IM once 2.4 million U IM once 500 mg PO q6h for 10 d 500 mg PO q6h for 10 d

Pediatric DosePediatric Dose Infants and children <30 Infants and children <30 lb: 600,000 U IM oncelb: 600,000 U IM onceChildren 30-60 lb: 900,000 Children 30-60 lb: 900,000 to 1.2 million U IM onceto 1.2 million U IM once

Infants and children <30 lb: Infants and children <30 lb: 600,000 U IM600,000 U IMChildren 30-60 lb: 900,000 Children 30-60 lb: 900,000 to 1.2 million U IM to 1.2 million U IM

<12 years: 25-50 mg/kg/d PO <12 years: 25-50 mg/kg/d PO divided tid/qid; not to exceed 3 divided tid/qid; not to exceed 3 g/d >12 years: Administer as g/d >12 years: Administer as in adultsin adults

ContraindicatioContraindicationsns

Documented Documented hypersensitivityhypersensitivity

Documented Documented hypersensitivityhypersensitivity

Documented hypersensitivity Documented hypersensitivity

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

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

administered concurrentlyadministered concurrently

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

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

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

PrecautionsPrecautions Caution in impaired renal Caution in impaired renal functionfunction

Never use IV route to Never use IV route to adminis ter penicillin G adminis ter penicillin G procaine; admi nister >10 d procaine; admi nister >10 d to eliminate orga nism and to eliminate orga nism and prevent complications. 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 RNA-DOC for patients allergic to penicillin; inhibits RNA-dependent protein synthesis, possibly by stimulating dependent protein synthesis, possibly by stimulating the dissociation of peptidyl t-RNA from ribosomes, which the dissociation of peptidyl t-RNA from ribosomes, which inhibits bacterial growth.inhibits bacterial growth.In children, age, weight, and the severity of infection In children, age, weight, and the severity of infection determine the proper dosage. When bid dosing is determine the proper dosage. When bid dosing is desired, one-half the daily dose may be administered desired, one-half the daily dose may be administered q12h. For more severe infections, the dose may be q12h. For more severe infections, the dose may be doubled.doubled.

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

Pediatric Pediatric DoseDose

30-50 mg/kg/d PO divided bid30-50 mg/kg/d PO divided bid

ContraindicaContraindicationstions

Documented hypersensitivity; hepatic impairment Documented hypersensitivity; hepatic impairment

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

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

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

Drug Category: Glucocorticoids

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. Reversing 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 PO


Documented hypersensitivity; viral, fungal, or tubercular skin infections

InteractionsInteractions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin, may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

PregnancyPregnancyB - Usually safe but benefits must outweigh the risks.

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)

DescriptionDescriptionTreats 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

ContraindicationsContraindications

Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K 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 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; 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)

DescriptionDescriptionA 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/tid

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


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 haloperidol

PregnancyPregnancyC - Safety for use during pregnancy has not been established.


Severe neurotoxicity manifesting as rigidity, or inability to walk or talk may occur in patients 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 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 POMaintenance dose: 25-35% of PO loading dose


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, 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 xate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid

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


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

Benzathine Benzathine penicillin Gpenicillin G

1,200,000 U every 4 weeks*, IM 1,200,000 U every 4 weeks*, IM oror

Penicillin VPenicillin V 250mg twice daily, PO 250mg twice daily, PO oror

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

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

ErythromycinErythromycin 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 CarditisArthritis Carditis Carditis Carditis alonealone minimal moderate severe minimal moderate severe

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

Indoor ambulationIndoor ambulation 1-2 wk 2-3 wk 4-6 wk 2-3 mo 1-2 wk 2-3 wk 4-6 wk 2-3 mo

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

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

PrednisonePrednisone 0 0 2-4 wk 2-6 wk 0 0 2-4 wk 2-6 wkAspirinAspirin 0 0 2-4 wk 2-6 wk 0 0 2-4 wk 2-6 wk

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

ComplicationsCarditis Mitral stenosis Congestive heart failure (CHF)

PrognosisSequelae are limited to the heart and are

dependent upon the severity of the carditis during the acute attack..

Rheumatic Heart Rheumatic Heart

DiseaseDisease Rheumatic heart disease is the most serious Rheumatic heart disease is the most serious

complication of rheumatic fever. complication of rheumatic fever. Acute rheumatic fever follows Acute rheumatic fever follows 0.3%0.3% of cases of of cases of

group A beta-hemolytic streptococcal pharyngitis group A beta-hemolytic streptococcal pharyngitis in children. As many as in children. As many as 39%39% of patients with of patients with acute rheumatic fever may develop varying acute rheumatic fever may develop varying degrees of pancarditis with associated degrees of pancarditis with associated valve valve insufficiency, heart failure, pericarditis, and even insufficiency, heart failure, pericarditis, and even death.death.

With With chronic rheumatic heart diseasechronic rheumatic heart disease, patients , patients develop 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 . Chronic rheumatic heart disease remains the leading cause of mitral valve disease remains the leading cause of mitral valve stenosis and valve replacement in adults stenosis and valve replacement in adults

Frequency: In the US: Prevalence of rheumatic heart disease in the United States now is less than 0.05 per 1000 populationInternationally: The 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 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 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 number 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.

Socio-economic factors :

It is well known that socioeconomic and environmental factors play an indirect, but important, role in the magnitude and 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 awareness of the disease in the community can all impact the expression of the disease in populations. Crowding adversely affects rheumatic fever incidence

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

Cardiac manifestations of chronic rheumatic heart disease : • Valve deformities, • thromboembolism, • cardiac hemolytic anemia, and • atrial arrhythmias are 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 rheumatic heart disease. The valve commissures and cusps become adherent and fused, and the valve orifice becomes small with a round or triangular shape.

• Thromboembolism occurs as a complication of mitral stenosis. It is more likely to occur when the left atrium is dilated, cardiac output is decreased, and the patient is in atrial fibrillation. • Cardiac hemolytic anemia is related to disruption of the red blood cells by a deformed valve. Increased

destruction and replacement of platelets also may occur.•Atrial 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 less than 6 months.

TREATMENTREATMEN

TT Medical CareMedical Care:: 1.1. Medical therapy is directed toward Medical therapy is directed toward eliminating eliminating

the group A strepto coccal pharyngitis the group A strepto coccal pharyngitis 2.2. Treatment of the Treatment of the acute inflammatory acute inflammatory

manifestationsmanifestations of acute rheumatic fever consists of acute rheumatic fever consists of administering of administering salicylates and steroidssalicylates and steroids. .

3.3. If If moderate-to-severe carditismoderate-to-severe carditis is indicated by is indicated by cardiomegaly, congestive heart failure, or third-cardiomegaly, congestive heart failure, or third-degree heart block, degree heart block, oral prednisone should be oral prednisone should be added to salicylate therapyadded to salicylate therapy. .

4.4. Preventive and prophylactic therapy is indicated Preventive and prophylactic therapy is indicated after rheumatic fever and rheumatic heart after rheumatic fever and rheumatic heart diseasedisease to prevent further damage to valves. to prevent further damage to valves.

Surgical Care:Surgical Care: When heart failure persists or worsens after When heart failure persists or worsens after

aggressive medical therapy for acute rheumatic aggressive medical therapy for acute rheumatic heart disease, surgery to decrease valve heart disease, surgery to decrease valve insufficiency may be life-saving. insufficiency may be life-saving.

Forty percent of patients with acute rheumatic Forty percent of patients with acute rheumatic fever subsequently develop mitral stenosis as fever subsequently develop mitral stenosis as adults.adults.

In patients with critical stenosis, mitral In patients with critical stenosis, mitral valvulotomy, valvulotomy, percutaneous balloon valvuloplasty, percutaneous balloon valvuloplasty, or mitral valve replacementor mitral valve replacement 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 replacement appears to be the preferred surgical replacement appears to be the preferred surgical option.option.

TREATMENTREATMEN

TT

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