4. Rheumatic heart disease
Acute rheumatic fever(ARF)
Incidence
Children
Young adults
Rare in western Europe & Northen America
Endemic;
parts of Asia
Africa
South America
100/100,000
5. Pathogenesis
Immune-mediated delayed response to infection with
specific strain of group A streptococci that possess
antigen which cross-react with cardiac myosin &
sarcolemmal membrane protein
Ab against the streptococcal Ag mediate inflammation in
endocardium,myocardium,pericardium,joint & skin
Fibrinoid degeneration in the collagen of connective
tissues
Aschoff nodules –only in the heart
6. Clinical features
Streptococcal pharyngitis
Fever,anorexia,lethargy,joint pain
2-3 wks after initial attack of pharyngitis
Arthritis
Rashes
Carditis
Neurological changes
7. Jones criteria for the diagnosis of acute
Rheumatic fever
Major manifestations
Carditis Erythema marginatum
Polyarthritis Subcutaneous nodules
Chorea
Minor manifestations
Fever Raised ESR or CRP
Arthralgia Leucocytosis
Previous rheumatic fever First degree AV block
Plus
Supporting evidence of streptococcal infection;recent
scarlet fever, raised ASO or other streptococcal antibody
titre, positive throat swab culture
11. Arthritis
Early feature
Acute,painful,asymmetric and migratory joint
inflammation of the large joints
Red, tender & swollen b/t a day & upto 4 wks
Characteristically response to aspirin
12. Skin lesions
Erythema marginatum Subcutaneous nodules
<5% 5-7%
Red macules which fade 0.5-2 cm
in the centre Firm & painless
Remain red at the edges Extensor surface of
Trunk & proximities but bone or tendon
not the face 3 wks after onset of
May coalesce or overlap
other menifestations
13. Sydenham’s chorea(st Vitusdance)
Late neurological manifestation
3/12 after episode of ARF
1/3 of cases
More common in females
Emotional lability
Purposeless choreiform movements of the hands,feet or
face
Explosive & halting speech
Spontaneous recovery within a few months
1/4 of pts with Sydenham’s chorea –chronic rheumatic ht
disease
14. Investigations
Evidence of a systemic illness(non-specific)
Raised WBC,ESR,CRP
Evidence of preceding streptococcal infection(specific)
Throat swab culture(pt& family contact)
( + ) in 10-25% of cases
ASO titre >200(adults) ,>300(children)
1/5 of cases & most cases of chorea
15. Investigations
Evidence of carditis
CXR
cardiomegaly,pulmonary congestion
ECG
Features of pericarditis,1st & 2nd Degree ht
block, low QRS voltage
Echo;
Cardiac dilatation,Valve abnormalities,
Pericardial effusion
16. Treatment of acute attack
A single dose of benzyl penicillin 1.2 MU im
Oral phenoxymethyl penicillin 250mg 6hrly for 10 d
Erythromycin or cephlosporin
Limiting cardiac damage and relieving symptoms
17. Bed rest & supportive therapy
Bed rest
Avoid strenuous exercise
Treat cardiac failure
Valve replacement if not respond to medical
treatment
Pacemaker insertion in cases with progressive AV
block
18. Aspirin
60mg/kg /day 6 dividing doses
100mg/kg/d in adults or Maximum 8g/d
Nausea,tinitus,deafness
Vomiting
tachypnoea
Acidosis
Should be continued until ESR has fallen & then
gradually tailed off
19. Corticosteroids
More rapid symptomatic relief > aspirin
Carditis
Severe arthritis
Prednisolone 1-2mg/kg/d in divided doses
Should be continued until the ESR is normal &
then gradually tailed off
20. Secondary prevention
Long term prophylaxis
IM benzyl penicillin 1. 2 million U monthly
Oral phynoxymethyl penicillin 250mg 12hrly
Sulphadiazine or erythromycin in pts allergic to
penicillin
At the age of 21 it should be stopped
21. Secondary prevention
Treatment s/b extended
if an attack has occurred in the last 5 years
Pts live in an area of highly prevalence
Has an occupation with high exposure to
streptococcal infection
In those with residual heart disease s/continue until
10yrs after last episode or 40yrs of age
**Not protect infective endocarditis
22. Chronic Rheumatic Heart Disease
Chronic rheumatic heart disease
In at least half of the those affected by rheumatic
fever with carditis
Two third of cases – women
Only possible to elicit a history of RF or chorea in
about half of all patients with chronic rheumatic
heart disease
Symptomatic during fulminant forms of ARF
Asymptomatic for many years
26. Mitral valve disease
Mitral stenosis
causes
Almost always rheumatic in origin
Heavy calcification in elderly
Congenital
27. Pathophysiology
In rheumatic MS
progressive calcification of fusion of cups
fibrosis the valve leaflet & subvalvular
apparatus
Mitralvalve orifice
restricted flow from LA to LV
pulmonary venous congestion
(enlarged LA & LV filling mainly on LA
contraction)
28. Pathophysiology
Increase in heart rate
shortens diastole
Further rise in LA pressure
Demand an increase in cardiac output
Further increase in left atrial pressure
29. Pathophysiology
MV orifice 5cm2
1cm2 or less in severe MS
Remain asymptomatic until MV orifice 2cm2
At first,symptoms occur only on exercise
Severe stenosis ; breathlessness at rest
Reduced lung compliance due to chronic
pulmonary congestion
Low cardiac output ;fatigue
30. Pathophysiology
Progressive dilatation of the LA
Atrial fibrillation
Tachycardia Loss of atrial
contraction
Marked Haemodynamic deterioration
with rapid rise in LA pressure
Pulmonary oedema
31. Pathophysiology
More gradual rise in LA pressure
An increase pulmonary vascular resistance
Pulmonary hypertension
Right ventricular hypertropy & dilation
Tricuspid regurgitation
Rt heart failure
32. Pathophysiology
In sinus rhythm
< 20%
Small LA
Severe pulmonary hypertension
All pts with MS particularly in those with AF
LA thrombosis
systemic thromboembolism
34. Investigations
ECG Doppler
LAH(If not in AF)
Pressure gradient
RVH
across the mitral valve
CXR
Pulmonary arterial
Enlarged LA
Signs of pulmonary venous
pressure
congestion LV function
Echo Cardiac catherization
Thickened immobile cusps
Assessment of
Reduced valve area
coexisting coronary
Reduced rate of diastolic
artery disease
filling of LV
&mitral regurgitation
35. Management
Medical treatment
Pts with minor symptoms
Definitive treatment
Pts remain symptomatic with medical treatment
Balloon valvuloplasty
Mitral valvotomy
Mitral valve replacement
36. Medical treatment
Atrial fibrillation
Anticoagulant
Digoxin
B blockers
Rate limiting calcium antagonist
Heart failure
Diuretics
Prophylaxis of infective endocarditis
Antibiotics
37. Specific management
Mitral balloon valvuloplasty
Treatment of choice
Criteria
significant symptoms
isolated MS
no or trivial MR
mobile non-calcified valve/subvalve apparatus on
echo
LA free of thrombus
38. Specific management
Closed or open mitral valvotomy
No facilities or expertise for balloon valvuloplasty
s/receive prophylactic antibiotics for IE
Follow up 1-2 yrly
Mitral valve replacement
substantial mitral reflux
rigid or calcified
39. Mitral regurgitation
Causes
Rheumatic disease
Mitral valve prolapse
After mitral valvotomy or valvuloplasty
Dilation of LV and mitral valve ring
Damage to valve cusps and cordae
Damage to papillary muscle
Myocardial infarction
40. Pathophysiology
Chronic Mitral regurgitation
Gradual dilation of the LA
with little in pressure gradual LV
diastolic pressure&
LA pressure
No symptoms Breathlessness &
pulmonary oedema
41. Pathophysiology
Acute mitral regurgitation
Rapid rise in LA pressure
Marked symptomatic deterioration
42. Mitral valve prolapse
Floppy mitral valve
Congenital
Degenerative myxoematous changes
A features of connective tissue disorders
43. Pathophysiology (MVP)
Mildest form Regurgitation haemodynamically
significant
Competent valve
during systole Infective
endocarditis
Bulge back to LA
Mid-systolic click click followed by Antibiotics
( no murmur) late systolic murmur
48. Treatment
Mitral valve repair
MVP
More advantage > MV replacement
Prevent irreversible LV damage
Those with CAD-CABG + MV repair by inserting
annuloplasty ring to overcome annular dilation &
to bring the valve leaflets closer together
50. Aortic stenosis
2nd most frequently affected by rheumatic fever
Commonly both mitral & aortic valves are
affected
In elderly structurally normal TV; similar process
of arthrosclerosis in arterial wall
Haemodynamically significant AS develops slowly
Age 30-60 rheumatic fever
50-60 bicuspid AV
70-90 degenerative AS
51. Aortic stenosis(AS)
Causes
Infants,children,adolscents
Congenital AS
Congenital subvalvular AS
Congenital supravalvular AS
Young adults and middle-aged
Calcifications and fibrosis congenital bicuspid aortic valve
Rheumatic AS
Middle-aged to elderly
Senile degenerative aortic stenosis
Calcifications of bicuspid aortic valve
Rheumatic AS
53. Pathophysiology
Fixed outflow obstruction
limit the increase in CO required by exercise
Effort related hypotension
Syncope
LV can no longer overcome outflow obstruction
Pulmonary oedema
56. Management
Asymptomatic
Under review
Symptomatic –prompt surgery
Moderately severe/ severe stenosis yearly
doppler echo
Pts remain symptomatic with medical treatment
Elderly –relatively benign prognosis-medical
treatment
57. Management
AV replacement
Severe stenosis with symptoms
Asymptomatic - careful exercise test;symptoms on
moderate exertion
Valloon valvuloplasty
congenital AS
no long term value in elderly pts with calcified AS
Anticoagulants
AF
Coexisting mitral valve disease
Valve replacement with mechnical prosthesis
58. Aortic stenosis in old patients
Most common form
Syncope,angina,heart failure
Low pulse pressure
Surgery –successful in those aged 80 without co-morbid
condition
higher operative mortality
Prognosis without surgery is poor if pt has symptoms
Valve replacement –bioprosthetic valve
61. Clinical features
Signs Murmur
Pulse Early diastolic murmur
Large or collapsing pulse Systolic murmur(stroke
Low diastolic pressure& volume)
pulse pressure ,Bounding Austin flint murmur(soft
peripheral pulses mid-diastolic murmur
Capillary pulsations in nail Other signs
beds Displaced,heaving apex
Femoral bruit(pistol shot)- beat
duroziez’s sign pre-systolic impulse
Head nodding with pulse
4th heart sound
De Musset ‘s sign
pulmonary venous
congestion
62. Investigations
ECG Doppler
Initially normal detects reflux
Later LVH
T wave inversion Cardiac catheterization
Dilated LV
CXR Aortic regurgitation
Cardiac dilation Dilated aortic root
Features of left heart Presence of coexisting CAD
failure
Echo
Dilated LV
Hyperdynamic LV
Fluttering anterior mitral
leaflet
63. Management
Treat the underlying conditions
Aortic valve replacement ±
aortic root replacement & CABG
symptomatic
Chronic AR without symptoms
s/report if symptoms are developed
Annually f/up with echocardiogram
AVR
if evidence of increasing ventricular size
If systolic dimension ≥55mmLV dilation
Control BP
Nefidipine/ACEI
65. Tricuspid stenosis
Rheumatic in origin
<5%
Always association with mitral & aortic valve
disease
Isolated TV stenosis very rare
TS & TR features of carcinoid syndrome
66. Clinical features and investigations
Symptoms of associated mitral & aortic valve disease
Symptoms of right heart failure
Raised JVP with a prominent a wave
A slow y descent due to loss of normal rapid RV filling
A mid-diastolic murmur at LLSE or RLSE
High pitch > murmur of MS
Increased by inspiration
Hepatomegaly
Presystolic pulsation (large a wave)
Peripheral oedema
Echo & Doppler ;similar appearance of mitral stenosis
68. Clinical features
Non-specific symptoms
Tiredness
Venous congestion
A large systolic phase in JVP
A cv wave replace normal x descent
PSM at LSE
Systolic pulsation of the liver
69. Investigations
Echocardiogram
Dilation of the RV
Thickened valve
Vegetations in endocarditis
Ebstein’s anomaly TV displaced towards the RV
apex
with consequent enlargement of the RA
associated with TR
70. Management
Correct RV overload
Normal pulmonary artery tolerate tricuspid
reflux well
valve damage dut to IE not always needs valve
replacement
repair of the valve with annuloplasty to bring the
leaflets together in patients undergoing MVR
those with rheumatic damage m/require
Tricuspid valve replacement
75. Management
Mild to moderate isolated pulmonary stenosis
Not usually progress
Not required treatment
Low risk for IE
Severe Pulmonary stenosis
( resting gradient >50mmHg with normal CO)
Percutaneous pulmonary balloon valvuloplasty
Not available;surgical valvotomy
Long term results very good
Post operative pulmonary regurgitation is common
Benign
76. Pulmonary regurgitation
Rarely an isolated phenomenon
Usually associated with pulmonary artery dilatation due to
pulmonary hypertension
EDM at LSE in MS( Graham steel murmur)
Pulmonary hypertension
2 to other disease of left heart
primary pulmonary vascular disease
Eisenmenger’s syndrome
Trivial PR frequent doppler finding in normal individuals