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7/30/2019 Valvular Heart Disease2
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Valvular Heart Disease
Cardiology Division, Medical Faculty Diponegoro University
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Rheumatic Fever
Etiologi
Acute rheumatic fever is a systemic disease of
childhood,often recurrent that follows group A
beta hemolytic streptococcal infection
It is a delayed non-suppurative sequelae to
URTI with GABH streptococci.
It is a diffuse inflammatory disease ofconnective tissue,primarily involving heart,blood
vessels,joints, subcut.tissue and CNS
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Epidemiology
Ages 5-15 yrs are most susceptible Rare boys
Common in 3rd world countries Environmental factors-- over crowding,
poor sanitation, poverty,
Incidence more during fall ,winter &early spring
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Pathogenesis
Delayed immune response to infection withgroup.A beta hemolytic streptococci.
After a latent period of 1-3 weeks, antibody
induced immunological damage occur toheart valves,joints, subcutaneous tissue &
basal ganglia of brain
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Pathologic Lesions
Fibrinoid degeneration of connective
tissue,inflammatory edema, inflammatory cell
infiltration & proliferation of specific cells
resulting in formation of Ashcoff nodules,
resulting in-- Pancarditis in the heart
- Arthritis in the joints
- Ashcoff nodules in the subcutaneoustissue
- Basal gangliar lesions resulting in
chorea
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Clinical Features
(Mayor feature)
1. Arthritis
2. Carditis
3. Sydenham Chorea4. Erythema Marginatum
5. Subcutaneous nodules
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Other features (Minor features)
Fever
Arthralgia
Pallor
Anorexia Loss of weight
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Laboratory Findings
High ESR
Anemia, leucocytosis
Elevated C-reactive protien
ASO titre >200 Todd units.
(Peak value attained at 3 weeks,then comes down to
normal by 6 weeks)
Anti-DNAse B test
Throat culture-GABH streptococci
ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion
2D Echocardiography- valve edema, mitral
regurgitation, LA & LV dilatation, pericardial effusion,
decreased contractility
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Diagnosis
Rheumatic fever is mainly a clinical
diagnosis
No single diagnostic sign or specific
laboratory test available for diagnosis
Diagnosis based on MODIFIED J ONES
CRITERIA
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Jones Criteria (Revised) for GuidanDiagnosis of Rheumatic Fev
Major Manifestation MinorManifestations
Supporting Evidenceof Streptococal Infectio
CarditisPolyarthritis
ChoreaErythema Marginatum
Subcutaneous Nodules
Clinical Laboratory
Increased Titer of Anti-Streptococcal Antibodies AS
(anti-streptolysin O),
othersPositive Throat Culturefor Group A Streptococcus
Recent Scarlet Fever
Previous
rheumaticfever orrheumaticheart diseaseArthralgiaFever
Acute phase
reactants:Erythrocytesedimentationrate,C-reactive
protein,
leukocytosisProlonged P-
R interval
*The presence of two major criteria, or of one major and two min
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Treatment
Step I - primary prevention
(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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STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb)
1 200 000 U for patients >27 kg
or
Penicillin V Children: 250 mg 2-3 times daily Oral 10 d(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)
or
Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Recommendations of American Heart Association
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Step II:Anti inflammatory treatmentClinical condition DrugsArthritis only Aspirin 75-100
mg/kg/day, give as 4
divided doses for 6 weeks
(Attain a blood level 20-30 mg/dl)Carditis Prednisolone 2-2.5 mg/kg/day,
give as two divided doses for 2 weeks
Taper over 2 weeks & while tapering add
Aspirin 75 mg/kg/day for 2 weeks
Continue aspirin alone
100 mg/kg/day for another 4 weeks.
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Bed rest
Treatment of congestive cardiac failure:
- digitalis,diuretics
Treatment of chorea:- diazepam or haloperidol
Rest to joints & supportive splinting
3. Step III: Supportive management &
management of complications
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STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
orSulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and
recommended
Recommendations of American Heart Association
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Duration of Secondary Rheumatic Fever
Prophylaxis
Category DurationRheumatic fever with carditis and At least 10 y since last
residual heart disease episode and at least until
(persistent valvar disease*) age 40 y, sometimes lifelong
prophylaxis
Rheumatic fever with carditis 10 y or well into adulthood,
but no residual heart disease whichever is longer
(no valvar disease*)
Rheumatic fever without carditis 5 y or until age 21 y,whichever is longer
*Clinical or echocardiographic evidence.
Recommendations of American Heart Association
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Prognosis
Rheumatic fever can recur whenever theindividual experience new GABH
streptococcal infection,if not on prophylactic
medicines
Good prognosis for older age group & if nocarditis during the initial attack
Bad prognosis for younger children & those
with carditis with valvar lesions
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Mitral Regurgitation - Aetiology
Primary
Annulus annular calcification
Leaflet myxomatous degeneration
rheumatic deformity
infectious perforation
Chordae myxomatous degenerationspontaneous rupture
rheumatic shortening
infectious destruction
Papillary infarction
ischemic lengthening
Functional
LV dilatation and PM displacement
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Mitral Regurgitation - Clinical findings
Acute dyspnoea, orthopnoea
no cardiomegaly, short murmur, S3
Chronic variable symptoms
cardiomegaly, murmur, P2 loud, S3Quantification
echocardiography, angiography
serial studies, LV function
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Mitral Regurgitation - Outcome in
Chronic MR
Variable course - diagnosis to symptoms 16 years
Symptomatic severe - survival 33% at 5 years
mortality ~5% per year
LV dysfunction most important factor
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Mitral Regurgitation - Treatment
Diuretics LV filling P, p oedema
Vasodilators forward SV
IABP
Acute
Chronic
No known effective therapyVasodilators - theoretical risks
Treat complications
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Mitral Regurgitation - Surgery
OptionsValve repair
MVR with chordal preservation
MVR with destruction MV apparatus
Outcome
Mortality 80-94% v 40-60% at 5-10years
Valve function
Ventricular function
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Mitral Regurgitation - Indications for surgery
No randomised trials!!
1. Symptomatic with normal LV function
prognosis worse once NYHA class II
symptoms
2. Symptomatic with abnormal LV function
If severe LV impairment - poor outlook
EF < 30% ? medical Rx better
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Mitral Regurgitation - Indications for surgery
3. Asymptomatic with abnormal LV function
? Asymptomatic
Detection of LV dysfunction is the key
EF 45mm, LVESV>55ml/m2
4.Asymptomatic with normal LV function
? guaranteed repair
PHT, recent AF
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Mitral Regurgitation - Indications for surgery
Chronic severe
mitral regurgitation
No symptoms Symptoms
EchocardiographyEchocardiography
Left ventricle ejectionfraction >0.60
and end-systolicdimension
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Mitral Regurgitation - Prolapse
2-4% population
females:males 2:1
diagnosis from echocardiography
subcategory according to leaflet abnormality
SBE prophylaxis; normal + MR or abnormal leaflets
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Mitral Stenosis
Causesrheumatic fever
congenital abnormality, calcification, myxoma
Natural historyRF age 12
murmur 1st heard 20 yrs later
symptoms in 4-5th decade
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Mitral Stenosis - Clinical features
Severity MVA (cm) LAP (mmHg) CO
Mild >2.0
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Mitral Stenosis - Examination
Inspection
Malar flushPeripheral cyanosis (severe MS)
Jugular venous distension (right ventricular failure)
Palpation
Parasternal right ventricular impulse
Palpable pulmonary arterial impulse
Palpable S1, P2, and occasionally, the diastolic
rumbleAuscultation
Increased intensity of the first heart sound
Opening snap
Low-pitched diastolic rumbling murmur
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Mitral Stenosis - Treatment
Medical
Diuretic - pulmonary congestion
Prevent embolism - cause of 19% deaths,
with LA size and age
anticoagulate all with PAF/AF, SR in older age
Control atrial fibrillation
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Mitral Stenosis - Treatment
Balloon Mitral Valvuloplasty
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Mitral Stenosis - Treatment
Balloon Mitral Valvuloplasty
100% MVA, final area ~2cm2
Failure rate 1-15%
Mortality 0-3%Severe MR 2-10%
Restenosis ~40% at 7years
Contraindications - thrombus, MR, Ca++, other
disease
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Mitral Stenosis - Treatment
Mitral Valve Replacement
Open mitral valvotomy
Mitral valve replacement
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Aortic Regurgitation - Aetiology
Root
Annuloaoroectasia
Marfans
Dissection
Syphillis
Ankylosing spondylitis
Leaflet
Endocarditis
Bicuspid valve
Rheumatic heart disease
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Acute Aortic Regurgitation - Clinical features
No time for LV to enlarge
total SV, fwd SV, LVEDP
Quiet S1 (presystolic MV closure),
short murmur
Treatment
Medical therapy ineffective
AVR if symptoms/signs LVF
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Chronic Aortic Regurgitation - Clinical features
total SV, maintained fwd SV, RV runoff in diastole
systolic BP, diastolic BP Volume and pressure overload
Examination - hyperdynamic circulation, wide pulse pressure,
dilated LV, EDM duration important
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Chronic Aortic Regurgitation - Clinical features
Maybe asymptomatic, LVF, angina
LV decompensation
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Chronic Aortic Regurgitation - Treatment
Medical - afterload
Nifedipine 20mg bd delayed surgery by 2-3 yrs
Duplicated with small ACEI trials
Surgery - AVR prior to irreversible LV dysfunction
1. Asymptomatic
LVEF55mm, LVESV 60ml/m2
2. Symptomatic
NYHA class II
A ti St i A ti l
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Aortic Stenosis - Aetiology
Congenital 1st-3rd decade
Valve degeneration and calcificationRheumatic - 4th decade
Bicuspid valve; 1%, males>females, 5-6th decades
Tricuspid valve - 7-8th decades, 1-2% incidence
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Aortic Stenosis - Pathophysiology
LV pressure overload LV hypertrophy diastolicLV dysfunction
Systolic function usually preserved except late in
diseaseSystolic function improves with AVR
Outcome is dependent on symptoms
A ti St i Cli i l f t
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Aortic Stenosis - Clinical features
Symptoms
None
SOBOE, dizziness
HF, syncope, anginaExamination
Pulse - amplitude, delay
Sustained apex
S2- soft and single paradoxical splitting
ESM - loud
late peak
soft
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Aortic Stenosis - Severity
Echocardiography
Meangradient(mmHg)
Peak Aovelocity
AVA(cm2)
Normal 1.0-2.0 >2.5
Mild 1.7
Moderate 20-40 3.0-4.0 1.0-1.7
Severe >40 >4.0
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Aortic Stenosis - Outcome
Symptomtic
2-year survival < 50%
Asymptomatic
Generally good prognosisPeak velocity >4.0m/s 2yr event-free survival
21%
Progression of> 0.3m/s per year - worse
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Aortic Stenosis - TreatmentMedical
None!!!
Diuretics v LVF
ACEI contraindicated
Balloon aortic valvuloplasty
Average MVA improvement 0.8cm2 1.0cm2
Restenosis
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Aortic Stenosis - AVR
Indicated only if symptomatic
Mortality 0.6-5%
Survival 67-85% at 5 yrs, 70% at 10yrs
2yr survival 4x greater than medical treatment
LV dysfunction
?impairment from pressure overload or other cause
DSE may be helpful
A ti St i AVR
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Aortic Stenosis - AVR
Approach to symptomatic patient
Ao V max
4.0m/s 3.0m/s3.0-4.0m/s
Doppler AVA
1.1-1.6cm2 1.7cm21.0cm2
2-3+ 0-1+
AVR recommended AVR for AS not recommended
AI severity