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Rheumatic feveris an immunologically mediatedinflammatory disease, that occurs as a delayedsequel to group A streptococcal throat infection,in genetically susceptible individuals.

Rheumatic heart diseaseis the most seriouscomplication of rheumatic fever

Acute rheumatic fever and rheumatic heartdiseaseare thought to result from an

autoimmune response, but the exactpathogenesis remains unclear

Introduction

Fuster V, et al. Acute Rheumatic Fever in Hurst the Heart 10th ed. New York: Mc GrawHill. 2001.

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Causes

Rheumatic fever is caused by a group A streptococcal. Itoccurs 2-3 weeks later after pharyngeal infection in a

small percentage of children aged 5-15 years. It is an

antibody-mediated autoimmune response and occurs

where antibodies directed against bacterial cellmembrane antigens cross-react and cause multiorgan

disease.

Fuster V, et al. Acute Rheumatic Fever in Hurst the Heart 10th ed. New York: Mc GrawHill. 2001.

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Pathogenesis

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The pathogenic mechanisms involved in the development of RF

remain unclear. But it is evident that an abnormal humoral and

cellular immune responseoccurs.

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.

Molecular mimicry was first demonstrated by humoral immuneresponse.

Streptococcal antibodies cross-react with several human tissues including

heart, skin, brain, glomerular basement membrane, striated and smooth

muscles.

Etiopathogenesis:

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Jones Criteria (2003 Revision) for Diagnosis of Acute

Rheumatic Fever

Major manifestations

1. Carditis2. Polyarthritis

3. Chorea

4. Erythema marginatum

5. Subcutaneous nodules

Minor manifestations1. Fever

2. Arthralgias

3. Previous rheumatic fever or rheumatic heart disease

4. Increased C- reactive protein concentrations or ESR

5. Prolonged PR interval on ECG Evidence of antecedent group A streptococcal

infection1. Positive throat culture or rapid antigen test positive for

group A streptococcus

2. Increased or increasing streptococcal antibody titer

Report of a WHO Expert Consultation Geneva. Geneva 2004.

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Jones Criteria (2003 Revision) for Diagnosis of

Acute Rheumatic Fever

A firm diagnosis requires

1) 2majormanifestations or1 major and 2 minormanifestations

and2 ) Evidence of a recent streptococcal infection.

Blood test reveal rising antistreptolysin O(ASTO) titres when taken 2 weeks apart. The

throat swabmay be positive.

Report of a WHO Expert Consultation Geneva. Geneva 2004.

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Carditis

Miocarditis: resting tachycardia,muffled or soft first heart sound, galloprhytm, cardiomegaly, or congestiveheart failure.

Endocarditis - valvulitis: changingmurmur (new apical systolic murmur of

mitral regurgitation and/or a basaldiastolic murmur of aortic regurgitation

Pericarditis: chest pain, friction rub,pericardial effusion

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Polyarthritis

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 in2 or 3 weeks.
http://www.netterimages.com/image/2507.htm

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Sydenham Chorea

Sydenhams chorea is

characterized by involuntarymovements, specially of the face

and limbs, muscle weakness,

disturbances of speech and gait.
http://www.netterimages.com/image/1171.htm

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MANAGEMENT OF RHEUMATIC

FEVER/RHEUMATIC HEART DISEASE

PRIMARY PREVENTION

adequate antibiotic therapy of group A Streptococcal

Upper Respiratory Tract infections to prevent an initial

attack of acute rheumatic fever SECONDARY PREVENTION

Continues administration of specific antibiotics to

patients with a previous attack of Rheumatic Fever

1. Report of a WHO Expert Consultation Geneva. Geneva 2004.2. Fuster V, Alexander RW, ORourke RA. Acute Rheumatic Fever in Hurst the Heart 10th ed. 2001. 3. Calleja HB, Guzman SV,eds. Rheumatic Fever And Rheumatic Heart Disease. Manila. 2001

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Managements

Treatment with high-dose benzatine benzylpenicillinis started immediately to eradicate thecausative organism.

Anti-inflammatory agents are given to suppress

the autoimmune response. Salicylatesareeffective. Corticosteroidsare used if there is anycarditis.

Long-term follow-up is required and any patients

who have resulting valve damage needprophylactic antibiotics to prevent infectiveendocarditis.

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Valve lesions and their abbreviations

Valve involved Lesion Abbreviation

Mitral valve Mitral stenosis

Mitral regurgitation

Floppy(prolapsing) mitral valve

MS

MR

MVP

Aortic valve Aortic stenosis

Aortic regurgitation

AS

AR

Tricuspid valve Tricuspid regurgitation

Tricuspid stenosis

TR

TS

Pulmonary

valve

Pulmonary stenosis

Pulmonary regurgitation

PS

PR

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MITRAL STENOSIS

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Definition

an obstruction to LV inflow at the level of mitral valveas a result of a structural abnormality of the mitral valve

apparatus, preventing proper opening during diastolicfilling of the left ventricle

Etiology

Rheumatic carditis ( 60 % )

Congenital malformation ( rare, children )

Prevalance

- Female : male = 2 : 1

- 40% of RHD

ACC/AHA Guidelines for Management ofPatients with valvular heart disease,1998
http://rds.yahoo.com/S=96062857/K=%22mitral+stenosis%22/v=2/SID=w/TID=I998_68/l=II/R=4/SS=i/OID=e51279590db2c284/SIG=1klu0oba7/EXP=1133285867/*-http:/images.search.yahoo.com/search/images/view?back=http://images.search.yahoo.com/search/images?p=%22mitral+stenosis%22&toggle=1&ei=UTF-8&imgsz=all&fr=FP-tab-img-t&b=1&h=378&w=504&imgcurl=home.cc.umanitoba.ca/~soninr/Heart%20Diagrams/Mitral/Mitral%20stenosis.JPG&imgurl=home.cc.umanitoba.ca/~soninr/Heart%20Diagrams/Mitral/Mitral%20stenosis.JPG&size=55.1kB&name=Mitral%20stenosis.JPG&rcurl=http://home.cc.umanitoba.ca/~soninr/Mitral.html&rurl=http://home.cc.umanitoba.ca/~soninr/Mitral.html&p=%22mitral+stenosis%22&type=jpeg&no=4&tt=132&ei=UTF-8

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Pathophysiology

Shahbudin H, Rahimtoola MB,et al; 2002

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Symptoms

- Some are asymptomatic

- Chief complaint : dyspnoe- DOE, OP, PND, and even acute pulmonary oedema

- Pulmonary hypertension with secondary right-sided heart failure

( hepatomegali, ascites, elevated jugular pressure, lower limb

oedema )

- Atrial arrhythmia : AF- Hemoptysis (rare, end stage)Alpert, JS, The AHA Clinical cardiac Consult,2001

Signs

- Loud S1

- Opening snap

- Diastolic rumble, near apex- Variably present ( loud P2, murmur of MR, murmur of TR )

Crawford, MH; Current Diagnosis and treatment in Cardiology, 2003

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Severity of MS


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Management

Medical treatment


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Medical treatment

- Antibiotics prophylaxis ( reccurent RF, IE )- Restrict activities (modsevere MS)

- AF :

- control ventricular rate (Digoxin)

- Anticoagulant : Heparin and Warfarin IV, when INR

is 2 to 3 discontinue heparin

- Diuretics ( elevated pulmonary venous pressure,

pulmonary congestion )

- ACE-I ( LV systolic dysfunction )

- Beta blockers after the patients are stablized

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Invasive Treatment

Shahbudin H, Rahimtoola MB,et al;2002

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Surgical Treatment

Shahbudin H, Rahimtoola MB,et al;2002

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MITRAL REGURGITATION

The mitral valve may become incompetent for four

reasons :

Abnormal mitral valve annulus

Abnormal mitral valve leafletsAbnormal chordae tendineae

Abnormal papillary muscle function

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Pathophysiology

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Etiology and mechanism of MR:

Etiology

Mechanism Non- ischemic Ischemic

Organic Rheumatic, prolapse, Ruptured

flail leaflet, endo- papillarycarditis, etc muscle

Functional Cardiomyopathy Post-MI func-tional MR

Heart 2002;87:79-85

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Clinical Presentation

Heart 2003 ; 89 : 459 464

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On examination

Atrial fibrillationan irregularly irregular pulse is

common, especially in patients who have chronic MRand a dilated left atrium.

Jugular venous pressure may be elevatedif thepatients has developed pulmonary hypertension andright heart failure, or fluid retention

The apex is displaced downward and laterally as the leftventricle dilates

The murmur of MR is pansystolic and best heard at theapex

Signs of congestive cardiac failure

P2may be loud and there may be a right ventricularheave

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Investigations

Electrocardiography

Chest radiography

Echocardiography

Cardiac catheterization

Management

Medical managementThis may consist of diuretics and ACE inhibitors

to treat the congestive cardiac failure.

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Surgical management

Patients are considered for surgery if the MR is severe at

echocardiography and cardiac catheterization. It is important to actbefore irreversible left ventricular damage has occurred.

Mitral valve repairThis may take the form of mitral annuloplasty, repair of a rupturedchorda or reapir of a mitral valve leaflet. These procedures are

performed on patients who have mobile non-calcified and non-thickened valves.

Mitral valve replacementThis is performed if mitral valve repair is not possible. Both repair

and replacement of the mitral valve require a median sternotomyincision and cardiopulmonary bypass.

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AORTIC STENOSIS

Type of AS Cause

Valvular AS Congenital most common,males>females (deformed valve can

be uni-, bi-, or tricuspid)

Senile calcification

Rheumatic feverSevere atherosclerosis

Subvalvular AS Fibrimuscular ring

HOCM

Supravalvular AS Associated with hypercalcaemia inWilliams sydrome, a syndrome

associated with elfin facies, mental

retardation, strabismus,

hypervitaminosis D and

hypercalcaemia; the inheritance is

autosomal dominant

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Pathophysiology

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Pathophysiology

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Clinical features

Dyspnoeamay lead to orthopnoea and paroxysmal

nocturnal dyspnoea as the left ventricle fails.

Anginadue to the increased myocardial work and

reduced blood supply (the coronary arteries may be

normal).

Dizziness and syncopeespecially on exertion.

Sudden death.

Systemic emboli.

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On examination

A slow rising, small volume pulsebest felt at

the carotid pulse.

A low blood pressure.

Heaving apex beatrarely displacedEjection systolic murmur at the aortic area

radiating to the carotids accompanied by a

palpable thrill

Signs of left ventricular failure

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Clinical featuresOn examination

A collapsing high-volume pulse (waterhammer pulse)-due to theincreased stroke volume and the rapid run-off of blood back into theleft ventricle after systole.

A wide pulse pressureon measuring blood pressure.

Early diastolic murmurbest heard at the left lower sternal edge withthe patients sitting forward and in full expiration-it is a soft-pitched

early diastolic murmur, which is sometimes difficult to hear, so besure to listen for it properly with a diaphragm.

Other signs include de Mussets sign(head bobbing with each beat),Quinckes sign(visible capillary pulsation in the nailbed), pistol shotfemoral pulses(an audible murmur over the femoral arteries-a to-and-fro sound).

May be signs of left ventricular or congestive failure.

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Investigations

Electrocardiography

Chest radiography

Echocardiography

Cardiac catheterization

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Management

Medical management

The use of diuretics and ACE inhibitors is valuable to

treat cardiac failure in these patients. It is, however,

important to make the diagnosis and surgically treatthis condition before the left ventricle dilates and fails.

Surgical management

Aortic valve replacement is considered if the patients is

symptomatic or if there are signs of progressive leftventricular dilatation. The aortic root may also need to

be replaced if it is grossly dilated.

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TRICUSPID REGURGITATION

Causes

Most cases of tricuspid regurgitation (TR) aredue to dilatation of the tricuspid annulus

resulting from dilatation of the right ventricle.This may be due to any cause of right ventricularfailure or pulmonary hypertension.

Occasionally, the tricuspid valve is affected

by infective endocarditis (usually in intravenousdrug abusers). Rarer causes include congenital

malformations and the carcinoid syndrome.

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O i f th l l i

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Overview of other valve lesions

Valve lesion Cause Clinical features Management

Tricuspidstenosis

Rheumatic fever; rare Venous congestionJVP

raised, large a waves, ascites,

hepatomegaly, peripheral

oedema, soft deastolic

murmur at left lower sternal

edge

Treat pulmonary

hypertension, valve

replacement

Pulmonary

stenosis

Congenital

malformation-

Noonans syndrome,

maternal rubella

syndrome, carcinoid

syndrome

If mild asymptomatic, if

severe- RVF and cyanosis,

ejection systolic murmur in the

pulmonary area (second left

ICS), wide splittting of second

heart sound

Pulmonary valvuloplasty or

Pulmonary valvereplacement

Pulmonary

regurgitation

(PR)

Dilatation of the valve

ring secondary to

pulmonary

hypertension, infective

endocarditis

RVF in severe cases, low-

pitched diastolic murmur in

pulmonary area, Graham

Steel murmur- in severe PR

the murmur is high pitched

due to the forceful parasternal

edge(i.e. similar to that in

aortic regurgitation but whith

signs of severe pulmonary

hypertension and RVF)

Treat underlying disease

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TERIMA KASIH

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