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