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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.
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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.
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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-88/13/2019 RF & RHD-Ali
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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
Shahbudin H, Rahimtoola MB,et al; 2002
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Management
Medical treatment
Shahbudin H, Rahimtoola MB,et al; 2002
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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