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RHEUMATIC HEART DISEASE

DR FELCIAN PETER KIBACHA (RESIDENT-PAEDIATRICS AND CHID HEALTH)

INTRODUCTION
AETIOLOGY

AND EPIDEMIOLOGY

CLINICALFEATURES

MAJOR

AND MINOR DIAGNOSTIC CRITERIA TREATMENT

INTRODUCTION

In Africa,and South America,rheumatic fever remains a common disease and is associated with significant morbidity and mortality rates Both in the acute phase of the disease and as a result of chronic,cardiac valvular sequelae,it remains the most common cause of acquired heart disease in children In most European and North American countries,the incidence of rheumatic fever declined to the 50 years preceding 1980 The reduction,both in incidence and severity,commenced before the adventof antibiotics and appears to have been related mainly to improvements in the general health and socio-economic standards of the populations in these
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However,since the mid-1980s,there has been an unexpected resurgence of rheumatic fever in the United states This has occurred in middle- class families with ready access to medical care The exact reasons are still unclear,but there has been the reappearance of rheumatogenic strains of group A haemolytic streptococci(M tpyes1,3,5,6 and in particular 18) in the affected areas

AETIOLOGY

In susceptible individuals(possibly with a specific class II HLA )rheumatic fever follows a pharyngeal infection with one of the Group A beta-haemolytic streptococci Specific rheumatogenic strains of streptococci can now be identified and it is significant that these strains are rarely found today in populations where rheumatic fever has virtually disappeared

EPIDEMIOLOGY

Global prevalence : Ranges from 0.2 (Cuba) to 77.6 per 1000 ( W.Samoa) 1 % of all school children in developing countries show signs of the disease. 12 million cases with 30% having CHF Commonest cause of Heart Disease in age group 5 30 years worldwide.

12 -65% of all hospital admissions related to CVD. in developing countries Mortality varies 2 5% Incidence dropped in many developed countries long before modern treatment due to improve socioeconomic conditions. Pockets are still found in some rich countries.

Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys ? ( No Gender Differences) High Carrier rate ( 1/3) Common in 3rd world countries Environmental over crowding, poor sanitation, poverty,

CLINICAL FEATURES

Diagnostic criteria of rheumatic fever


MAJOR 1.Carditis ECG 2.Polyarthritis 3.Chorea rheumatic fever 4.Erthema Marginatum MINOR 1. Prolonged PR interval on 2. Arthralgia 3.Previous history or evidence of 4. Fever

5.Nodules

5 Acute phase reactants


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Note; use 1 and 2 as either minor or major criteria but not as both

MAJOR CRITERIA

CARDITIS-70% of hospitalized black children presenting with rheumatic fever have evidence of carditis on their first admission(world figures vary from 40 to 75%) The diagnosis of carditis is made if one or more of the following is present: Cardiac murmurs indicate endocarditis,the most common of these is a high pitched,blowing,pancystolic murmur heard at the apex and is due to mitral valve incompetence caused by distortion of the mitral valve cusps and mitral annular dilatation Sometimes this is associated with a short low pitched middiastolic murmur at thre apex,which disappears as the acute process resolves
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Involvement of the aortic valve causes incompetence,which is recognized by a high pitched early diastolic murmur,heard best at the aortic area and down the left sternal edge when the patient is sitting up and leaning forward If severe,a wide pulse pressure and collapsing pulses will also be present

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Polyarthitis. The typical presentation is a flitting arthritis with red, hot, swollen, tender, large joints which become involved sequentially. As a new joint is involved, the arthritis in new in the previously affected joint subsides. It is important to consider rheumatic fever in the differential diagnosis of mono-arthritis in children. Once the acute inflammation has settled, the joints recover completely.

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Chorea.This occurs most often in girls between seven and fourteen years of age. There is a long latent period of weeks or months between the streptococcal infection and the onset of symptoms. In some cases, chorea may develop some weeks after joint symptoms but often it occurs without any recognized symptoms or sign of rheumatic fever. The onset is gradual over one or two weeks during which the child is scolded for being clumsy, spilling drinks or dropping articles
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cont........
Erythema marginatum. this is a transient erthematous rash.

When it fades it leaves irregular thin lines which make circular patterns on te trunk and occasionally the limbs, but never on the face.

It is easily seen on light-skinned patients, but neither easily nor often seen on darkly pigmented skin.

Nodules. subcutaneous, non-tender, small, mobile nodules are found over the extensor surfaces (around the elbows, wrists, knuckles, knees, ankles

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MONIR CRITERIA

Previous history or evidence of previous episode of rheumatic fever


Arthralgia.It is important to exclude vague limb pains due to myalgia This should not be used as a criterion if arthritis is a major manifestation

Fever.Temperature recordings are usually 38C or more in acute rheumatic fever


Prolonged P-R interval on the electrocardiogram to greater than 0.18 seconds This should not be used where carditis is a major criterion
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Acute phase reactants.These are not specific for rheumatic fever

CONT..

leucocytosis. this is usually between 12 and 15*10


Erythrocyte sedimentation rate (ESR). this is raised, but the level does not correlate with severity. it may, however, be normal in chorea C-reactive protein (CRP). this is invariably found in the serum of almost all cases.

The presence of two major criteria, or one major and two minor criteria, together with evidence of a preceding streptococcal infection, makes the diagnosis of rheumatic fever probable
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TREATMENT

Step I - Antibiotics (eradication of streptococci) Step II - anti inflammatory treatment (aspirin, steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks)

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PROGNOSIS

Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions

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PREVENTION

Primordial : Preventing risk factors Primary : Identifying all those suffering from sore throat early and give proper , prompt treatment Detect inapparent infections and identify carriers and give proper treatment. Surveillance of high risk groups, school ages, throat swab , early diagnosis

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Secondary Prevention: for those diagnosed as having RF, give prophylactic penicillin 1.2. million unit every 3 /52 for Five years or till age 18 years ( which ever comes later)For 10 years or age 25 years for those with carditis . For life after surgery Tertiary Prevention :Rehabilitation

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Treatment of rheumatic fever

Prevention

Rheumatic fever is rare where standards of living are good.


A high incidence is associated with poor housing, overcrowding, and lack of primary health care facilities.

Treatment of streptococcal throat infections with penicillin within one week of the onset of symptoms, prevents the development of rheumatic fever, provided the treatment is adequate. The recommended dosage are;
One IM injection of benzathine penicillin G 600000 units (below 30kg body weight) or 1200000 units (over 30kg) or

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cont.......

Oral penicillin V 50mg/kg/d, given three times a day for ten days. it is important that oral penicillin is given before meals and the course completed. In patients sensitive to penicillin, treatment is with erthromycin 125 to 250mg, four times daily for ten days; or cephaloridine IM 30 to 50 mg/kg/d in divided doses, eighthourly; or oral cephalexin, 25 to 100 mg/kg/d in divide doses, eighth-hourly

Ideally all children complaining of a sore throat should have a throat swab cultured and, if group A beta-haemolytic strepptococci are found the patient should be given penicillin treatment or erythromycin, if they are penicillin sensitive) 22

cont...

Rest. Children with painful joints and acute carditis invariably lie still. As they recover they may be allowed to move around in bed, but should not be allowed to walk until joint involvement has subsided, cardiac enlargement decreased, and the 'sleeping' pulse rate diminished. Thereafter they should be allowed progressively more activity; most children on adequate treatment should be back to normal, non strenuous activity within three weeks. If there has been cardiac failure, convalescence may be prolonged and activity should be restricted until evidence of rheumatic activity has been absent for two weeks
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Anti-inflammatory treatment

Salicylates are particularly useful in alleviating the pain of arthritis and the discomfort of fever. The ESR returns to normal more quickly, but salicylates do not have any effect on valvular damage Dosage. sodium salicylate 40 to 60 mg/kg/d, or acetylisalicylic acid (aspirin),80 to 120mg/kg/d Treatment should continue until all signs of activity have subsided and then be gradually withdrawn over a two-week period Recurrence of symptom will require increasing the dosage until control is achieved
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cont...

Symptoms of salicylate toxicity, (tinnitus, dizziness, nausea, and vomiting) are rarely seen at the recommended dosage. If they occur, treatment must be stopped for 48 hours and recommenced at lower dosage.

Gastric irritation with bleeding from the mucosa may occur and therefore, stools should be examined for occult blood regularly while the patient is on treatment. Giving extra milk or using buffered aspirin may overcome this problem
Contra-indications. salicylates may precipitate pulmonary oedema in patients with acute carditis and are, therefore, best avoided in patients with obvious cardiac embarrassment
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cont......

The value of corticosteroids in the treatment of children with carditis is controversial They may possibly be life-saving in cases of pancarditis Signs of acute rheumatic fever(e.g.fever,raised ESR,and arthritis) may respond rapidly to corticosteroid therapy but there is no effect on long term valvular damage A recent double-blind placebo -controlled trial of prednisolone failed to show any benefit either in the short term clinical response or in the long term follow up of patients with active rheumatic carditis Dosage:Prednisolone,2mg/kg/day in four divided doses
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cont..

Treatment should be continued at full dosage until the patient is symptomatically well and the acute phase reactants have been normal for one week This usually takes two to three weeks Thereafter the dosage should be reduced by 10 percent every second day,untill the daily dose is one third of the initial dose Thereafter the reduction should be by 5% every second day

Regular assessment of the acute phase reactants should show a decline to normal levels
Should these become elevated again during the withdrawal period,the dosage should be increased to the previous level and maintained at that until signs of activity have 27 subsided,before gradual withdrawal is recommenced

Congestive cardiac failure

Slow digitalization with digoxin,0.04 to 0.06 mg/kg total in four equal doses at six-hourly intervals is reccommended
The maintenance dose is 0.01mg/kg/day in ten divided doses

Large doses of digoxin are dangerous in children with myocarditis


Diuretics are indicated if there is pulmonary edema or severe congestive failure hypokalaemia is particularly dangerous in children with myocarditis during digitalization and potassium suppliments must be given if a diuretic which eliminates potassium is used
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cont..

Hydrochlorothiazide,0.5 to 2.0 mg/kg/d in two to three divided doses,is a safe diuretic


Forusemide 1mg/kg/dose iv,is used for pulmonary edema

This can be repeated until improvement occurs.potassium supplimentation is essential when using furosemide
Spinorolactone (aldactone),2 to 3 mg/kg/d divided in two to three doses may be required when oedema is chronic and does not respond to the above mentioned drugs Captopril (a vasodilator) in a dose of 0.5 to 6.0mg/kg/d,in four divided doses may be added to the antfailure therapy,especially if there is significant mitral regurgitation 29 mitral regurgitation

chorea

If involuntary movements are severe,haloperidol 0.025mg to 0.05mg/kg/day can be given orally in divided doses In milder cases phenobarbitone 3 to 5mg/kg/d may be used

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CONCLUSSION

Acute Rheumatic Fever leading to Rheumatic Heart Disease Disease is a problem world wide but a major problem in developing countries Appropriate treatment of group A strep pharyngitis necessary to prevent disease. Preventing recurrences causing chronic heart disease is : simple, universally available, and cost- effective.

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Crowding, poor housing, maternal literacy Primary prevention sore throat, skin sores, no vaccine Secondary prevention prevention of recurrent ARF with penicillin; proven to be cost-effective Tertiary management medical treatment and surgeryRehabilitation

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RHEUMATIC HEART DISEASE

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INTRODUCTION

The symptoms and signs of acute rheumatic fever subside


The joints recover completely and in those who have not had carditis,there is a return to normality

Once rheumatic fever has occurred,however there is always a danger of reccurence


If there had been carditis during the first attack,subsequent attacks will involve the heart again,causing increasing damage to the valves These become progressively more distorted,and incompetent and/or adherent and stenotic
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Mitral valve disease

The mitral valve is the most commonly affected by rheumatic fever, either alone or in combination with the aortic valve and,occassionaly,with the tricuspid valve By far the most common lesion is mitral incompetence The apical pansystolic murmur radiating to the axilla,heard during the acute attack, may disappear within a few months, only to reappear and become harsher and louder as the valves contract The hemodynamic effect of mitral incompetence depends on the amount of blood which regurgitates into the left atrium through the damaged valve during ventricular systole A small amount has litle effect, but large amounts of blood result in distension and increased pressure in the left atrium and pulmonary vein
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Cont..

In severe mitral incompetence there is displacement of the apex which is thrusting in character, and the pansystolic murmur will be loud and may be heard not only in the axilla but at the back as well If a third heart sound is heard before the diastolic murmur, it indicates critical overloading of the left ventricle Such patients are symptomatic, with poor effort tolerance,orthopnoea,and paroxysmal nocturnal dyspnoea,and require treatment with digoxin to prevent cardiac failure In these cases a chest x-ray shows enlargement of both the left atrium and left ventricle Only severe cases show ECG changes of left-atrial and leftventricular hypertrophy
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Mitral stenosis

Usually takes some years to develop as the cusps of the damaged valve fuse together When rheumatic fever occurs at a young age in impoverished third world children, the progression of the valvular disease to incompetence and/or stenosis appears to be more rapid The obstruction of flow into the left ventricle causes enlargement and increased pressure in the left atrium and pulmonary veins, which leads to pulmonary arteriolar hypertension Children with mitral stenosis have poor effort tolerance,dyspnoea,and coughing but hemoptisis is rare in childhood

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Aortic valve disease

Involvement of the aortic valve may occur alone following rheumatic fever, but it is more often associated with mitral valve disease Pure aortic stenosis is usually due to a congenital defect Incompetence invariably occurs if the aortic valve is damaged by rheumatic fever and this may be associated with some degree of stenosis if the distorted cusps fuse together The progression of aortic damage is much slower than mitral valve disease and rarely causes severe disability or symptoms during childhood, unless there is gross incompetence

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Cont..

An ejection systolic murmur, which may be soft or loud, with a thrill if there is significant stenosis,is often heard at the aortic area as well
In severe aortic incompetence, a mid-diastolic murmur is frequently heard at the apex Austin flint murmur)-this is caused by the regurgitant flow striking the anterior mitral leaflet open in diastole which then shudders, thus setting turbulence This may lead to a mistaken diagnosis of co-existent mitral stenosis

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Tricuspid valve

Occasionally the tricuspid valve may be damaged by rheumatic fever This usually causes incompetence, with or without some degree of stenosis Usually the mitral valve, but occasionally the aortic valve, is also involved More often ,tricuspid valve incompetence occurs as a functional complication of severe pulmonary hypertension associated with mitral valve disease, particularly tight mitral stenosis

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Management of Rheumatic Heart Disease

Prevention of recurrent attacks. Once rheumatic fever has occurred, the most important aspect of treatment is to prevent reccurences,particularly if there has been any evidence of carditis,as each subsequent attack causes increasingly severe damage to the heart valves
This is achieved by preventing streptococcal infection by continuous prophylactic treatment with penicillin or, in those few patients who are allergic to penicillin by sulphonamides or erythromycin therapy

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Cont..

While it is rare for a patient to develop a recurrence of rheumatic fever after ten years, adolescents and young adults in close communities, such as boarding-schools, army camps, or mine compound, are at risk of developing streptococcal pharyingits with rheumatogenic strains and recurrence of rheumatic fever Prophylactic treatment should,therefore,be continued into adulthood in patients who are in these situations When prophylactic treatment is uninterrupted, incompetent lesions have been found to improve in some patients

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Cont..

The most effective prophylactic regime is achieved with an injection of long-acting benzathine peniccilin,600,000 units IM for those under 30kg,and 1.2 million units for bigger children and adults,administred every three weeks Oral pennicillin V 250mg twice daily may be used but is less successful in achieving complete protection. patients forget to take their pills before meals and absorption can be disturbed by enteral illness
Oral sulphonamides 0.5mg to 1mg twice daily, or erythromycin 250mg daily may be used as alternative ,but patient compliance must be ensured
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Cont..

Surgical correction of valve defects. Patients who are incapacitated by damaged valves should be referred for detailed echocardiographic and catheterization studies so that surgical intervention or mitral balloon valvuloplasty can be planned Tight pliable mitral stenosis can be relieved either by balloon valvuloplasty(at catheterization ) or by surgical commissurotomy with or without cardiopulmonary bypass support Mitral stenosis,with significant subvalvular thickening or incompetent valves can be repaired or replaced by prosthetic,homograft,or heterograft valves at open heart operations Occasionally, a child with established mitral incompetence and acute carditis,who fails to respond to anti-failure therapy, will require surgical correction of the mechanical defect well in short term 44

Cont..

Unfortunately ,no child who undergoes a surgical procedure for valves damaged by rheumatic fever ceases to be a patient and most require repeated operations
In Africa ,acute rheumatic fever still has a mortality of 2 to 3 percent and there is a considerable morbidity The lifespan in those who have established heart disease has been prolonged by surgical intervention,but it is still limited and the quality of life is poor ,with affected individuals being unable to undertake occupations requiring physical exertion

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Cont..

Only through well-organized health programmes aimed at improved living standards(especially housing),early treatment of streptococcal infections,and persistent prophylaxis of affected individuals,can a reduction in this common and eminently preventable cause of serious cardiac disease be achieved

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THANK YOU

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