Rheumatic Heart

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RHEUMATIC HEART DISEASE

DR FELCIAN PETER KIBACHA

(RESIDENT-PAEDIATRICS AND CHID HEALTH)

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INTRODUCTION AND EPIDEMIOLOGY

AETIOLOGY

CLINICALFEATURES

MAJOR AND MINOR DIAGNOSTIC CRITERIA

TREATMENT

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INTRODUCTION

In Africa,and South America,rheumatic fever remains acommon disease and is associated with significant morbidityand mortality rates

Both in the acute phase of the disease and as a result of

chronic,cardiac valvular sequelae,it remains the mostcommon cause of acquired heart disease in children

In most European and North American countries,theincidence of rheumatic fever declined to the 50 years

preceding 1980

The reduction,both in incidence and severity,commencedbefore the adventof antibiotics and appears to have beenrelated mainly to improvements in the general health andsocio-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 re-

appearance of rheumatogenic strains of group A haemolytic

streptococci(M tpyes1,3,5,6 and in particular 18) in the

affected areas

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AETIOLOGY

In susceptible individuals(possibly with a specific classII HLA )rheumatic fever follows a pharyngeal infectionwith one of the Group A beta-haemolytic streptococci

Specific rheumatogenic strains of streptococci cannow be identified and it is significant that these strainsare rarely found today in populations where rheumaticfever has virtually disappeared

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EPIDEMIOLOGY

Global prevalence : Ranges from 0.2 (Cuba) to77.6 per 1000 ( W.Samoa)

1 % of all school children in developingcountries show signs of the disease.

12 million cases with 30% having CHF

Commonest cause of Heart Disease in agegroup 5 30 years worldwide.

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12 -65% of all hospital admissions related toCVD. in developing countries

Mortality varies 2 5%

Incidence dropped in many developed countrieslong before modern treatment due to improvesocioeconomic conditions. Pockets are stillfound in some rich countries.

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Ages 5-15 yrs are most susceptible

Rare boys ? ( No Gender Differences)

High Carrier rate ( 1/3)

Common in 3rd world countries

Environmental over crowding, poor sanitation, poverty,

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CLINICAL FEATURES

Diagnostic criteria of rheumatic fever

MAJOR MINOR

1.Carditis 1. Prolonged PR interval onECG

2.Polyarthritis 2. Arthralgia

3.Chorea 3.Previous history or evidence ofrheumatic fever

4.Erthema 4. Fever

Marginatum

5.Nodules 5 Acute phase reactants

Note; use 1 and 2 as either minor or major criteria but not as both9


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MAJOR CRITERIA

CARDITIS-70% of hospitalized black children presentingwith rheumatic fever have evidence of carditis on their firstadmission(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 atthe apex and is due to mitral valve incompetence caused bydistortion of the mitral valve cusps and mitral annulardilatation

Sometimes this is associated with a short low pitched mid-diastolic murmur at thre apex,which disappears as the acute

process resolves10


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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 arthritiswith red, hot, swollen, tender, large joints which becomeinvolved sequentially.

As a new joint is involved, the arthritis in new in thepreviously affected joint subsides.

It is important to consider rheumatic fever in the differentialdiagnosis of mono-arthritis in children.

Once the acute inflammation has settled, the joints recovercompletely.

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Chorea.This occurs most often in girls between seven andfourteen 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 circularpatterns on te trunk and occasionally the limbs, but never on

the face.

It is easily seen on light-skinned patients, but neither easilynor often seen on darkly pigmented skin.

Nodules. subcutaneous, non-tender, small, mobile nodules arefound 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 dueto 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 greaterthan 0.18 seconds

This should not be used where carditis is a major criterion 15


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CONT.. Acute phase reactants.These are not specific for

rheumatic fever leucocytosis. this is usually between 12 and 15*10

Erythrocyte sedimentation rate (ESR). this is raised, butthe level does not correlate with severity. it may,

however, be normal in chorea

C-reactive protein (CRP). this is invariably found in theserum of almost all cases.

The presence of two major criteria, or one major andtwo minor criteria, together with evidence of a precedingstreptococcal infection, makes the diagnosis of rheumaticfever probable

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TREATMENT

Step I - Antibiotics (eradication of streptococci) Step II - anti inflammatory treatment (aspirin, steroids)

Step III- supportive management & management ofcomplications

Step IV- secondary prevention (prevention of recurrentattacks)

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PROGNOSIS

Rheumatic fever can recur whenever theindividual experience new GABH streptococcalinfection, 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 , prompttreatment

Detect inapparent infections and identifycarriers and give proper treatment. Surveillanceof high risk groups, school ages, throat swab ,early diagnosis

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Secondary Prevention: for those diagnosed ashaving RF, give prophylactic penicillin 1.2.million unit every 3 /52 for Five years or till age18 years ( which ever comes later)-

For 10 years or age 25 years for those withcarditis .

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 penicillinwithin one week of the onset of symptoms, prevents thedevelopment 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 tendays. 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; orcephaloridine IM 30 to 50 mg/kg/d in divided doses, eight-hourly; or oral cephalexin, 25 to 100 mg/kg/d in dividedoses, eighth-hourly

Ideally all children complaining of a sore throat should havea throat swab cultured and, if group A beta-haemolyticstrepptococci are found the patient should be given

penicillin treatment or erythromycin, if they are penicillinsensitive) 22


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cont... Rest. Children with painful joints and acute carditis

invariably lie still.

As they recover they may be allowed to move around inbed, but should not be allowed to walk until jointinvolvement has subsided, cardiac enlargement decreased,

and the 'sleeping' pulse rate diminished.

Thereafter they should be allowed progressively moreactivity; most children on adequate treatment should be backto normal, non strenuous activity within three weeks.

If there has been cardiac failure, convalescence may beprolonged and activity should be restricted until evidence ofrheumatic 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 donot have any effect on valvular damage

Dosage. sodium salicylate 40 to 60 mg/kg/d, oracetylisalicylic acid (aspirin),80 to 120mg/kg/d

Treatment should continue until all signs of activity havesubsided 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 andrecommenced at lower dosage.

Gastric irritation with bleeding from the mucosa may occurand therefore, stools should be examined for occult bloodregularly 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,andarthritis) may respond rapidly to corticosteroid therapy but

there is no effect on long term valvular damage

A recent double-blind placebo -controlled trial ofprednisolone failed to show any benefit either in the shortterm 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 reactantshave 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 initialdose

Thereafter the reduction should be by 5% every second day

Regular assessment of the acute phase reactants shouldshow a decline to normal levels

Should these become elevated again during the withdrawalperiod,the dosage should be increased to the previous leveland maintained at that until signs of activity have

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Congestive cardiac failure

Slow digitalization with digoxin,0.04 to 0.06 mg/kg total infour 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 withmyocarditis

Diuretics are indicated if there is pulmonary edema orsevere congestive failure

hypokalaemia is particularly dangerous in children withmyocarditis during digitalization and potassium supplimentsmust be given if a diuretic which eliminates potassium isused

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cont..

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

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

This can be repeated until improvement occurs.potassiumsupplimentation is essential when using furosemide

Spinorolactone (aldactone),2 to 3 mg/kg/d divided in two tothree doses may be required when oedema is chronic anddoes not respond to the above mentioned drugs

Captopril (a vasodilator) in a dose of 0.5 to 6.0mg/kg/d,infour divided doses may be added to the antfailuretherapy,especially if there is significant mitral regurgitationmitral regurgitation 29


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chorea

If involuntary movements are severe,haloperidol0.025mg to 0.05mg/kg/day can be given orallyin divided doses

In milder cases phenobarbitone 3 to 5mg/kg/dmay be used

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CONCLUSSION

Acute Rheumatic Fever leading to Rheumatic HeartDisease

Disease is a problem world wide but a major problem indeveloping 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, novaccine

Secondary prevention

prevention of recurrent ARFwith penicillin; proven to be cost-effective

Tertiary management medical treatment and surgery-Rehabilitation

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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 nothad carditis,there is a return to normality

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

If there had been carditis during the first attack,subsequentattacks will involve the heart again,causing increasingdamage to the valves

These become progressively more distorted,and incompetentand/or adherent and stenotic

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Mitral valve disease

The mitral valve is the most commonly affected byrheumatic fever, either alone or in combination with theaortic 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 thevalves contract

The hemodynamic effect of mitral incompetence depends onthe amount of blood which regurgitates into the left atriumthrough the damaged valve during ventricular systole

A small amount has litle effect, but large amounts of bloodresult in distension and increased pressure in the left atriumand pulmonary vein

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Cont..

In severe mitral incompetence there is displacement of theapex which is thrusting in character, and the pansystolicmurmur 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, itindicates critical overloading of the left ventricle

Such patients are symptomatic, with poor efforttolerance,orthopnoea,and paroxysmal nocturnaldyspnoea,and require treatment with digoxin to preventcardiac failure

In these cases a chest x-ray shows enlargement of both theleft atrium and left ventricle

Only severe cases show ECG changes of left-atrial and left-ventricular 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 thevalvular disease to incompetence and/or stenosis appears to

be more rapid

The obstruction of flow into the left ventricle causesenlargement and increased pressure in the left atrium andpulmonary veins, which leads to pulmonary arteriolarhypertension

Children with mitral stenosis have poor efforttolerance,dyspnoea,and coughing but hemoptisis is rare in

childhood

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Aortic valve disease

Involvement of the aortic valve may occur alone followingrheumatic fever, but it is more often associated with mitralvalve 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 withsome degree of stenosis if the distorted cusps fuse together

The progression of aortic damage is much slower thanmitral valve disease and rarely causes severe disability orsymptoms during childhood, unless there is grossincompetence

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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 atthe aortic area as well

In severe aortic incompetence, a mid-diastolic murmur is

frequently heard at the apex Austin flint murmur)-this iscaused by the regurgitant flow striking the anterior mitralleaflet open in diastole which then shudders, thus settingturbulence

This may lead to a mistaken diagnosis of co-existent mitralstenosis

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Tricuspid valve

Occasionally the tricuspid valve may be damaged byrheumatic fever

This usually causes incompetence, with or without somedegree of stenosis

Usually the mitral valve, but occasionally the aortic valve, isalso involved

More often ,tricuspid valve incompetence occurs as afunctional complication of severe pulmonary hypertensionassociated with mitral valve disease, particularly tight mitral

stenosis

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Management of Rheumatic Heart


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Management of Rheumatic HeartDisease

Prevention of recurrent attacks. Once rheumatic fever hasoccurred, the most important aspect of treatment is to

prevent reccurences,particularly if there has been anyevidence of carditis,as each subsequent attack causesincreasingly severe damage to the heart valves

This is achieved by preventing streptococcal infection bycontinuous prophylactic treatment with penicillin or, inthose few patients who are allergic to penicillin bysulphonamides or erythromycin therapy

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Cont..

While it is rare for a patient to develop a recurrence ofrheumatic fever after ten years, adolescents and youngadults in close communities, such as boarding-schools, armycamps, or mine compound, are at risk of developingstreptococcal 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, incompetentlesions have been found to improve in some patients

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Cont..

The most effective prophylactic regime is achieved with aninjection of long-acting benzathine peniccilin,600,000 unitsIM for those under 30kg,and 1.2 million units for biggerchildren and adults,administred every three weeks

Oral pennicillin V 250mg twice daily may be used but isless successful in achieving complete protection. patientsforget to take their pills before meals and absorption can bedisturbed by enteral illness

Oral sulphonamides 0.5mg to 1mg twice daily, orerythromycin 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 areincapacitated by damaged valves should be referred fordetailed echocardiographic and catheterization studies sothat surgical intervention or mitral balloon valvuloplasty can

be planned

Tight pliable mitral stenosis can be relieved either by

balloon valvuloplasty(at catheterization ) or by surgicalcommissurotomy with or without cardiopulmonary bypasssupport

Mitral stenosis,with significant subvalvular thickening orincompetent 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-failuretherapy, will require surgical correction of the mechanicaldefect well in short term

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Cont..

Unfortunately ,no child who undergoes a surgical procedurefor valves damaged by rheumatic fever ceases to be a patientand 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 limitedand the quality of life is poor ,with affected individuals

being unable to undertake occupations requiring physicalexertion

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Cont..

Only through well-organized health programmesaimed at improved living standards(especiallyhousing),early treatment of streptococcalinfections,and persistent prophylaxis of affectedindividuals,can a reduction in this common and

eminently preventable cause of serious cardiacdisease be achieved

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THANK YOU

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Rheumatic Heart

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