RHEUMATIC FEVERIs an inflammatory disease occurring in response to a group A beta-haemolytic streptococcal infection.Affects even the very young beginning from the age of three.Peak incidence occurs between 6-15 years.First attacks are uncommon after adolescence.Slightly more common in girl than boys.

AETIOLOGY The disease is a sequel to group A beta haemolytic streptococcal pharyngitis.Development of Rheumatic fever depends on : 1. Individual susceptibility 2. The promptness or otherwise with which effective treatment is given 3. Rheumatogenicity of the infecting streptococci

PATHOGENESIS/ PATHOLOGYAfter infection of the throat with streptococci. The host has an autoimmune response to epitopes in the bacterium that are immunologically cross- reactive with host tissues in synovium, heart, brain and skin.This causes proliferative and exudative inflammatory reactions in these tissues.Effusions into joints, pericardial or pleural spaces result from exudative reactions.

Proliferative process is typified by the Ascoff nodules most abundant in the left atrial myocardium.Clusters of large multinucleated cells in a mass of fragmented swollen collagen, (Granulomatous collections of leukocytes, myocytes, and interstitial collagen)The cardiac lesion comprise fibrinous pericarditis, myocarditis and endocarditis (pancarditis).Endocarditis is most conspicuous in the left ventricle and mitral valves.Mitral valves becomes roughened and oedematous.

Varying degrees of cardiac enlargement may occur at this acute stage.Mitral regurgitation is common.Later on as healing progresses, the pericarditis resolves completely.Myocardium may become scarred.The valve edges become thickened and rolled up.The papillary muscles become shortened thus preventing full approximation of valve leaflet during ventricular systole.

Commissural adhesions may subsequently lead on to valve stenosis.

CLINICAL FEATURESInvolve a pharyngeal infection followed 2-6 weeks later by a polyarthritis, fever, subcutaneous nodules, erythema marginatum, tarchyardia and signs of heart failure. May present with choreaExact symptoms depend on the organs involved.

DIAGNOSISIs based on Jones criteria which has major and minor manifestations. Need 2 major or one major and 2 minor.Major criteria: pancarditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules.Minor criteria: arthralgia, fever, increased P-R interval, raised ESR, increased C- reactive protein, history of rheumatic fever, raised ASO titres and positive throat culture.

Can make a diagnosis if there is only chorea or pancarditis. INVESTIGATIONSThroat swab culture for streptococciASO titres if exceeds 200i.u.ECG prolonged PR interval. Raised ESR >120mm/hrC- reactive protein >2g/dl.

Differential diagnosisSickle cell diseaseSepticaemia with infective endocarditisJuvenile rheumatoid arthritis.

TREATMENT AND PROGNOSIS Treatment should be aimed at 1. Eradicating any streptococci patient is still harbouring.2.Suppress the inflammatory reactions3. Reduce cardiac workloadThese can be achieved through bed rest, administration of penicillin and salicylate or steroids.

Procaine penicillin 0.3-0.6 mega units for 10 days or Benzathine penicillin 0.6-1.2 mega units single injection.Aspirin 80-100mg/kg per dayOral Prednisolone 1-2mg/kg per day is indicated if patient has signs of severe carditis like extreme tarchycardia, pansystolic murmur and rapidly progressive valve damage or heart failure.Steroids may be given with salicylate until signs have subsided.

Usual anti-failure measures are applied until heart failure is controlled.Sedating drugs such as chlorpromazine, haloperidol and phenobarbital should be given in severe attacks of chorea.About 75% of children subsequently develop chronic rheumatic heart disease.

PREVENTIONPrevention of Rheumatic fever and Rheumatic heart disease are interwoven.Primary prevention by preventing first attack can be achieved through: 1. Prevention of overcrowding 2. Improved nutrition. 3. Health education and provision of essential health facilities.

4. Prompt and effective treatment of streptococcal infection.A 7-10 day course of oral penicillin or procaine penicillin 0.3-0.6 mega units daily I.M. Or a single dose of Benzathine penicillin 0.6-1.2 mega units will achieve the same results.

Secondary prevention:Aim is to prevent a recurrence of acute rheumatic fever.The main thrust is prevention of streptococcal infections by long term antibiotic prophylaxis.E.g. Benzathine penicillin once monthly.Pen V 250mg BD.

If allergic to penicillin give Erythromycin 250 BD daily.The prophylaxis can be stopped 5 years after the last attack of Rheumatic fever or when patient reach the age of 18 years.

END OF PART ONE.

RHEUMATIC HEART DISEASE ( RHD)Sequel of Rheumatic fever.No complications on all other parts of the body affected.Mitral valve is involved in practically every child who has RHD. Other valves affected are the aortic and tricuspid.. Pulmonary valve involvement is very rare .

Majority of patients have mitral valve regurgitation.Others have both mitral valve regurgitation and stenosis.Pure mitral stenosis is not very common. But it has been reported in a number of tropical countries. It is believed to result from repeated attacks of acute rheumatic fever and associated intense host reaction.

CLINICAL FEATURESMajority are brought with features of cardiac failure.A few because of excessive precordial activity.The signs are those of underlying valve defect plus heart failure if such a complication exist.

INVESTIGATIONSCxRECGECHO

TREATMENTMedical treatment involves taking anti-failure measures.Treat infective endocarditis and use prophylaxis before certain surgical proceduresDefinitive treatment is surgery to replace the damaged valves

CardiomyopathiesThis is a disease of the myocardium. There is damage to heart muscle. There are 3 types:1 dilated cardiomyopathy2. Hypertrophic 3. Restrictive or obliterative

Dilated cardiomyopathy (DCM)Involves dysfunction of the cardiomyocytes resulting in dilatation and impaired function of left or right ventricle. Causes:Most cases are idiopathic.Genetic diseases e.g. mitochondrial abnormalities, carnitine defiency, muscular dystrophy, Pompes disease.Nutrition problems .e.g. selenium, thiamine and calcium defiencies, Iron overload, severe aneamiaInfections: Post viral myocarditis ( coxsackie, echovirus), sepsis, diphtheria, rheumatic fever, HIV

Toxins : mainly drugs like Doxorubicin, Cyclophosphamide, adriamycin, AZT

Clinical Features Heart failure, arrhythmias, embolismInvestigations: CXR shows cardiomegaly ECG shows LVH, non specific T wave abnormalities Echo cardiogram shows atrial and ventricular dilatation poorly contracting. Reduced ejection fraction, mitral and tricuspid regurgitation

Metabolic screenCardiac biopsyTreatment: cardiac failure and anticoagulants( aspirin or warfarin)Cardiac transplant

Hypertrophic cardiomyopathyCharacterized by hypertrophy of the interventricular septum and left ventricular .Left ventricular outflow tract obstruction may occur.Causes: Neurofibromatosis, Autosomal dominant in some cases. Premature infants receiving steroid for lung disease( transient)

Clinical features: Syncope, angina, arrhythmias, sudden death, family history of sudden death.

Investigation:CXR, ECG, Echocardiogram

Treatment:Bita blockers ( propranolol)Amiodarone Cardiac pacingSurgical resection of the septum if significant outflow obstruction

Restrictive cardiomyopathyThere is a rigid noncompliant ventricleStarts with cell death - fibrosis- rigid, noncompliant heart.Caused by scleroderma, hemochromatosis, Gauchers and sarcoidosis.Treatment: No medical treatment Heart transplant

Congestive heart failureCardiac output insufficient to meet systemic demand.Can be right, left or both sided failureCauses:Valve disease, anemia, endocarditis, cardiomyopathy, thyrotoxicosis, hypertension, rheumatic fever, congenital heart diseases.

Signs & symptoms are multiorganLeft sided failure leads to pulmonary oedema, dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspneaMitral regurgitation worsens the reduction in cardiac output.Renal hypoperfusion- activation of renin-angiotensin axis- increased aldosterone- increased sodium retention- increased water retention( increased in total body fluid load)

In right sided failure, lack of forward venous flow leads to liver engorgement and lower extremity oedemaIn children they may present with only respiratory distress, tarchycardia and hepatomegaly

Treatment:Reduce physical activityProp up in bedO2

Digoxin Laxis Slow kACE inhibitors, spironolactone, Bita blockers have been shown to improve survival.When giving ACE and spironolactone do not give slow K.