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Valvular Heart Disease
Dr. M A Sungkar,SpPD,SpJP
Cardiology Division, Medical Faculty Diponegoro University
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 of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS
Epidemiology Epidemiology
• Ages 5-15 yrs are most susceptible• Rare <3 yrs• Girls>boys• Common in 3rd world countries
• Environmental factors-- over crowding, poor sanitation, poverty,
• Incidence more during fall ,winter & early spring
Pathogenesis Pathogenesis
• Delayed immune response to infection with group.A beta hemolytic streptococci.
• After a latent period of 1-3 weeks, antibody
induced immunological damage occur to
heart valves,joints, subcutaneous tissue &
basal ganglia of brain
Pathologic Lesions 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 subcutaneous tissue
- Basal gangliar lesions resulting in
chorea
Clinical FeaturesClinical Features (Mayor feature)(Mayor feature)
1. Arthritis
2. Carditis
3. Sydenham Chorea
4. Erythema Marginatum
5. Subcutaneous nodules
Other features (Minor features)
• Fever• Arthralgia• Pallor• Anorexia• Loss of weight
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
Diagnosis Diagnosis
• Rheumatic fever is mainly a clinical
diagnosis
• No single diagnostic sign or specific
laboratory test available for diagnosis
• Diagnosis based on MODIFIED JONES
CRITERIA
Jones Criteria (Revised) for Guidance in theDiagnosis of Rheumatic Fever*
Major Manifestation MinorManifestations
Supporting Evidence of Streptococal Infection
Clinical LaboratoryCarditisPolyarthritis
ChoreaErythema Marginatum
Subcutaneous Nodules
Previousrheumaticfever orrheumaticheart diseaseArthralgiaFever
Acute phasereactants:Erythrocytesedimentationrate, C-reactiveprotein,leukocytosis Prolonged P-R interval
Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O),othersPositive Throat Culture for Group A StreptococcusRecent Scarlet Fever
*The presence of two major criteria, or of one major and two minor criteria,indicates a high probability of acute rheumatic fever, if supported by evidence ofGroup A streptococcal nfection.
Treatment 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)
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
Step II: Anti inflammatory treatment
Clinical condition Drugs
Arthritis 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
.
• 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
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
orPenicillin 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
Duration of Secondary Rheumatic Fever Prophylaxis
Category Duration
Rheumatic 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
Prognosis Prognosis
• Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines
• Good prognosis for older age group & if no carditis during the initial attack
• Bad prognosis for younger children & those with carditis with valvar lesions
Mitral Regurgitation - Aetiology
Primary Annulus annular calcification
Leaflet myxomatous degeneration
rheumatic deformity
infectious perforation
Chordae myxomatous degeneration
spontaneous rupture
rheumatic shortening
infectious destruction
Papillary infarction
ischemic lengthening
Functional
LV dilatation and PM displacement
Mitral Regurgitation - Clinical findings
Acute dyspnoea, orthopnoea
no cardiomegaly, short murmur, S3
Chronic variable symptoms
cardiomegaly, murmur, P2 loud, S3
Quantification
• echocardiography, angiography
• serial studies, LV function
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
Mitral Regurgitation - Treatment
Diuretics LV filling P, p oedema
Vasodilators forward SV
IABP
Acute
Chronic
No known effective therapy
Vasodilators - theoretical risks
Treat complications
Mitral Regurgitation - Surgery
Options
Valve repair
MVR with chordal preservation
MVR with destruction MV apparatus
Outcome
Mortality 80-94% v 40-60% at 5-10years
Valve function
Ventricular function
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
Mitral Regurgitation - Indications for surgery
3. Asymptomatic with abnormal LV function
• ? Asymptomatic
• Detection of LV dysfunction is the key
EF<60%, LVESD > 45mm, LVESV>55ml/m2
4. Asymptomatic with normal LV function
• ? guaranteed repair
• PHT, recent AF
Mitral Regurgitation - Indications for surgery
Chronic severemitral regurgitation
No symptoms Symptoms
EchocardiographyEchocardiography
Left ventricle ejectionfraction >0.60
and end-systolicdimension <45 mm
Left ventricle ejectionfraction <0.60
or end-systolicdimension >45 mm
Mitral valvereparable
Mitral valvenot reparable
Atrial fibrillationor pulmonaryhypertension
No atrial fibrillationor pulmonaryhypertension
Clinical andechocardiographic
follow up
Mitral-valve surgery(valve repair preferredIf technically feasible
Mitral-valve
replacement
Medical
therapy
Ejection fraction<0.30
Ejection fraction>0.30
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
Mitral Stenosis
Causes rheumatic fever congenital abnormality, calcification, myxoma
Natural history RF age 12 murmur 1st heard 20 yrs later symptoms in 4-5th decade
Mitral Stenosis - Clinical features
Severity MVA (cm²) LAP (mmHg) CO
Mild >2.0 <10-12 NL
Moderate 1.1-2.0 ~10-17 NL
Severe <1.0 >18
Very Severe <0.8 >20-25
Severity Symptoms
Mild Asymptomatic or mild DOE
Moderate Mild-mod DOE; orthopnea, PND, hemoptysis
Severe Dyspnea at rest; possible pulmonary edema
Very Severe Severe PHT; RV failure, marked dyspnea at rest; severe fatigue; cyanosis
Mitral Stenosis - Examination
Inspection Malar flush Peripheral 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
rumble
Auscultation
Increased intensity of the first heart sound Opening snap Low-pitched diastolic rumbling murmur
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
Mitral Stenosis - Treatment
Balloon Mitral Valvuloplasty
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
Mitral Stenosis - Treatment
Mitral Valve Replacement
Open mitral valvotomy
Mitral valve replacement
Aortic Regurgitation - Aetiology
Root
Annuloaoroectasia
Marfans
Dissection
Syphillis
Ankylosing spondylitis
Leaflet
Endocarditis
Bicuspid valve
Rheumatic heart disease
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
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
Chronic Aortic Regurgitation - Clinical features
Maybe asymptomatic, LVF, angina
LV decompensation
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
• LVEF<55%, LVESD>55mm, LVESV 60ml/m2
2. Symptomatic
• NYHA class II
Aortic Stenosis - Aetiology
Congenital 1st-3rd decade
Valve degeneration and calcification
Rheumatic - 4th decade
Bicuspid valve; 1%, males>females, 5-6th decades
Tricuspid valve - 7-8th decades, 1-2% incidence
Aortic Stenosis - Pathophysiology
LV pressure overload LV hypertrophy diastolic LV dysfunction
Systolic function usually preserved except late in disease
Systolic function improves with AVR
Outcome is dependent on symptoms
Aortic Stenosis - Clinical features
Symptoms
None
SOBOE, dizziness
HF, syncope, angina
Examination
Pulse - amplitude, delay
Sustained apex
S2- soft and single paradoxical splitting
ESM - loud late peak soft
Aortic Stenosis - Severity
Echocardiography
Meangradient(mmHg)
Peak Aovelocity
AVA(cm2)
Normal 1.0-2.0 >2.5
Mild <20 2.5-2.9 >1.7
Moderate 20-40 3.0-4.0 1.0-1.7
Severe >40 >4.0 <1.0
Aortic Stenosis - Outcome
Symptomtic
2-year survival < 50%
Asymptomatic
Generally good prognosis
Peak velocity >4.0m/s 2yr event-free survival
21%
Progression of> 0.3m/s per year - worse
Aortic Stenosis - Treatment Medical
None!!!
Diuretics v LVF
ACEI contraindicated
Balloon aortic valvuloplasty
Average MVA improvement 0.8cm2 1.0cm2
Restenosis <6/12 in 50%
No improvement in mortality
Procedural mortality 5%
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
Aortic Stenosis - AVRApproach 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
