Valvular Heart Disease

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
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
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
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 Features
(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
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 Diagnosis of Rheumatic Fever*

Major Manifestation
Carditis Polyarthritis Chorea Erythema Marginatum Subcutaneous Nodules

Minor Manifestations
Clinical Previous rheumatic fever or rheumatic heart disease Arthralgia Fever Laboratory Acute phase reactants: Erythrocyte sedimentation rate, C-reactive protein, leukocytosis Prolonged PR interval

Supporting Evidence of Streptococal Infection

Increased Titer of AntiStreptococcal Antibodies ASO (anti-streptolysin O),

others
Positive Throat Culture for Group A Streptococcus Recent Scarlet Fever

*The presence of two major criteria, or of one major and two minor c a high probability of acute rheumatic fever, if supported by evidence

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 Benzathine penicillin G Dose Mode Duration Once 600 000 U for patients Intramuscular 27 kg (60 lb) 1 200 000 U for patients >27 kg

or
Penicillin V Children: 250 mg 2-3 times daily Oral (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: Estolate 20-40 mg/kg/d 2-4 times daily (maximum 1 g/d) Oral 10 d 10 d

or
Ethylsuccinate 40 mg/kg/d 2-4 times daily (maximum 1 g/d) Oral 10 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
.

3. Step III: Supportive management &

management of complications
Bed rest Treatment of congestive cardiac failure: - digitalis,diuretics Treatment of chorea: - diazepam or haloperidol Rest to joints & supportive splinting

STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)

Agent
Benzathine penicillin G

Dose
1 200 000 U every 4 weeks*

Mode
Intramuscular

or
Penicillin V 250 mg twice daily Oral

or
Sulfadiazine 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
Rheumatic fever with carditis and residual heart disease (persistent valvar disease*)

Duration
At least 10 y since last episode and at least until age 40 y, sometimes lifelong prophylaxis 10 y or well into adulthood, whichever is longer

Rheumatic fever with carditis but no residual heart disease (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
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 Leaflet annular calcification myxomatous degeneration rheumatic deformity infectious perforation myxomatous degeneration spontaneous rupture rheumatic shortening infectious destruction infarction ischemic lengthening

Chordae

Papillary

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

Acute
Diuretics IABP LV filling P, p oedema Vasodilators forward SV

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 severe mitral regurgitation

No symptoms Echocardiography

Symptoms Echocardiography

Left ventricle ejection fraction >0.60 and end-systolic dimension <45 mm

Left ventricle ejection fraction <0.60 or end-systolic dimension >45 mm

Mitral valve reparable

Mitral valve not reparable

No atrial fibrillation Atrial fibrillation or pulmonary or pulmonary hypertension hypertension

Ejection fractionEjection fraction >0.30 <0.30

Clinical and echocardiographic follow up

Mitral-valve surgery (valve repair preferred If technically feasible

Mitral-valve replacement

Medical therapy

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 Mild Moderate Severe Very Severe MVA (cm) >2.0 1.1-2.0 <1.0 <0.8 LAP (mmHg) <10-12 ~10-17 >18 >20-25 CO NL NL

Severity Mild

Symptoms Asymptomatic or mild DOE

Moderate
Severe Very Severe

Mild-mod DOE; orthopnea, PND, hemoptysis

Dyspnea at rest; possible pulmonary edema 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

LV decompensation

Maybe asymptomatic, LVF, angina

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

Mean Peak Ao AVA gradient velocity (cm2) (mmHg) 1.0-2.0 >2.5 <20 2.5-2.9 3.0-4.0 >4.0 >1.7 1.0-1.7 <1.0

Normal Mild

Moderate 20-40 Severe >40

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 - AVR

Approach to symptomatic patient

Ao V max 4.0m/s
3.0-4.0m/s

3.0m/s

Doppler AVA 1.0cm2 1.1-1.6cm2 AI severity 1.7cm2

2-3+ AVR recommended

0-1+ AVR for AS not recommended