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Diseases of the heart valves
Causes of valve disease

Valve regurgitation        Valve stenosis
 Congenital                Congenital

 Acute rheumatic           rheumatic carditis
  carditis                  Senile degeneration
 Chronic rheumatic
  carditis
 Infective endocarditis

 Syphilitic aortitis

 Valve ring dilatation
Rheumatic Heart Disease

Acute rheumatic fever
Chronic rheumatic heart disease
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
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
Clinical features

   Streptococcal pharyngitis
   Fever,anorexia,lethargy,joint pain
   2-3 wks after initial attack of pharyngitis
   Arthritis
   Rashes
   Carditis
   Neurological changes
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
Investigations

 Positive blood culture
 Raised antistreptolysin O(ASO)

without evidence of recent streptococcal infection
 Isolated chorea

 pancarditis
Carditis

   Pancarditis
   Declines with increasing age
   (90% at 3yrs-30% in adolescent)
   Breathlessness
   Palpitation
   Chest pain
   Tachycardia
   Cardiac enlargement
   New or changed cardiac murmur
Carditis

   Soft MDM(Carey coombs murmur)
   AR 90%
   TV & PV rarely involved
   Pericarditis
   Cardiac failure
   ECG –conduction defect,ST-T changes
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
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
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
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
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
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
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
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
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
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
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
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
Chronic rheumatic heart disease
   Mitral valve – more than 90 %
   Aortic valve
   Tricuspid valve
   Pulmonary valve
   Isolated mitral stenosis-25%
   Mixed mitral stenosis & regurgitation
Pathology

   Progressive fibrosis
   Predominantly involved heart valves
   Involvement of pericardium & myocardium
    m/contribute to heart failure & conduction
    disorder
   Fusion of the mitral valve commissures &
    shortening of the cordae tendinae –mitral
    stenosis+/- mitral regurgitation
   Similar changes in other valves
Mitral Valve Disease
Mitral valve disease

Mitral stenosis

causes
 Almost always rheumatic in origin

 Heavy calcification in elderly

 Congenital
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)
Pathophysiology

        Increase in heart rate

          shortens diastole

     Further rise in LA pressure

Demand an increase in cardiac output

Further increase in left atrial pressure
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
Pathophysiology

  Progressive dilatation of the LA

         Atrial fibrillation

Tachycardia                    Loss of atrial
              contraction

Marked Haemodynamic deterioration
   with rapid rise in LA pressure

         Pulmonary oedema
Pathophysiology

     More gradual rise in LA pressure

An increase pulmonary vascular resistance

         Pulmonary hypertension

  Right ventricular hypertropy & dilation

          Tricuspid regurgitation

              Rt heart failure
Pathophysiology

   In sinus rhythm
   < 20%
   Small LA
   Severe pulmonary hypertension

All pts with MS particularly in those with AF
   LA thrombosis
   systemic thromboembolism
Clinical features

Symptoms                    Signs
 Breathlessness             AF
 Fatigue                    Mitral facies
 Oedema                     Auscultation;
 Ascites                          loud 1st heart sound
 Palpitation                      opening snap
 Haemoptysis                      Mid-diastolic murmur
 Cough                      Signs of raised pulmonary
 Chest pain                       capillary pressure
 Symptoms of                      crepitations,pulmonary
  thromboembolic                   oedema,effusions
  complications              Signs of pulmonary
                               hypertension
                                   RV heave,loud P2
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
Management

Medical treatment
 Pts with minor symptoms



Definitive treatment
 Pts remain symptomatic with medical treatment
 Balloon valvuloplasty
 Mitral valvotomy
 Mitral valve replacement
Medical treatment

Atrial fibrillation
 Anticoagulant

 Digoxin

 B blockers

 Rate limiting calcium antagonist

Heart failure
 Diuretics

Prophylaxis of infective endocarditis
 Antibiotics
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
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
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
Pathophysiology

              Chronic Mitral regurgitation

               Gradual dilation of the LA



with little in pressure                     gradual  LV
diastolic                                        pressure&
                           LA pressure



  No symptoms                         Breathlessness &
                                     pulmonary oedema
Pathophysiology

   Acute mitral regurgitation

    Rapid rise in LA pressure

Marked symptomatic deterioration
Mitral valve prolapse

   Floppy mitral valve
   Congenital
   Degenerative myxoematous changes
   A features of connective tissue disorders
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
Clinical features

Symptoms                      Signs
   Breathlessness               AF/flutter/cardiomegaly
   Fatigue
                                 Auscultation;
                                       apical pansystolic
   Palpitation
                                  murmur
   Oedema                             ±thrill
   Ascites                            soft S1,apical S3
                                 Signs of raised pulmonary
                                       venous congestion
                                       (crepitations,pulmonary
                                       oedema,effusions)
                                 Signs of pulmonary
                                  hypertension & RHF
                                       RV heave,loud P2
Investigations

ECG                           Echo
 LAH(If not in AF)            Thickened immobile cusps
 LVH                          Reduced valve area

CXR                            Reduced rate of diastolic
 Enlarged LA                   filling of LV
 Enlarged LV                 Doppler
 Signs of pulmonary venous    Detects & quantifies

  congestion                    regurgitation
 Pulmonary oedema            Cardiac catheterization
                               Dilated LA,LV,MR
                               Pulmonary hypertension
                               Assessment of coexisting
                                coronary artery disease
Treatment

Medical treatment
 Moderate severity

Definitive treatment
 Pts remain symptomatic with medical treatment

 Progressive radiological cardiac enlargement or
  echo cardiac evidence of deteriorating LV function
 Mitral valve replacement/repair
Treatment

Atrial fibrillation
 Anticoagulant

 Digoxin

Heart failure
 Diuretics

 Vasodilators e.g ACEI

Prophylaxis of infective endocarditis
 Antibiotics
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
Aortic valve disease disease
           Aortic valve
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
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
Pathophysiology

Steadily increase pressure gradient across the AV

          LV increasingly hypertrophied

         Inadequate coronary blood flow

                     Angina
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
Clinical features

Symptoms                   Signs
Mild to moderate-           Slow rising carotid pulse
   asymptomatic             Narrow pulse pressure
 Exertional dyspnoea
                            Thrusting apex beat(LV
 Angina
                              overload)
 Exertional syncope
                            Harsh ejection systolic
 Sudden death
                              murmur
 Episodes of acute
                            Soft S2
   pulmonary oedema
                            Signs of pulmonary venous
                              congestion(crepitations,pul
                              monary oedema)
Investigations

ECG                              Doppler
                                  Severity of stenosis
 LVH
 LBBB                              Detection of
CXR                                  associated aortic
 Normal                             regurgitation
 Enlarged LV                    Cardiac catheterization
 Dilated ascending aorta(PA )    Assessment of coexisting

 Calcified valve(lateral)         coronary artery disease
                                  Pressure gradient b/t LV &
Echo
                                   aorta
 calcified valve with
                                 CT/MRI
  restricted opening
                                  Degree of valve
 Hypertrophied LV
                                   calcification & stenosis
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
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
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
Aortic regurgitation

Aetiology
 Congenital
 Bicuspid or disproportionate cusps


Acquired
 Rheumatic disease
 Infective endocarditis
 Trauma
 Aortic dilatation(marfan’s
  syndrome,aneurysm,dissectionsyphillis,ankylosing
  spondylitis
Clinical features

Symptoms

Mild- moderate AR
 Often asymptomatic

 Awareness of heart beat



Severe AR
 Breathlessness

 Angina
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
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
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
Tricuspid valve disease




Tricuspid valve Disease
Tricuspid stenosis

   Rheumatic in origin
   <5%
   Always association with mitral & aortic valve
    disease
   Isolated TV stenosis very rare
   TS & TR features of carcinoid syndrome
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
Tricuspid regurgitation

Causes
Primary
 Rheumatic heart disease

 Endocarditis

 Ebstein’s congenital anomaly

Secondary
Rv dilatation( chronic LHF
RV infarction
Pulmonary hypertension( corpulmonale)
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
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
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
Pulmonary Valve disease




       Pulmonary valve Disease
Pulmonary stenosis

Causes
 Carcinoid syndrome

 Usually congenital

 Isolated or associated with other abnormalities
  e.g TOF
Clinical features

   ESM at left upper sternum
   Radiation to left shoulder
   Thrill
   Preceded ejection click
   Wide split S2
   Loud harsh murmur
   inaudible P2
    RV heave
   Prominent a wave in JVP
Investigations

ECG
 RVH

CXR
 Post-stenotic dilation in the pulmonary artery

Doppler echo
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
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
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Vulvular heart diseases

  • 1. Diseases of the heart valves
  • 2. Causes of valve disease Valve regurgitation Valve stenosis  Congenital  Congenital  Acute rheumatic  rheumatic carditis carditis  Senile degeneration  Chronic rheumatic carditis  Infective endocarditis  Syphilitic aortitis  Valve ring dilatation
  • 3. Rheumatic Heart Disease Acute rheumatic fever Chronic rheumatic heart disease
  • 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
  • 8. Investigations  Positive blood culture  Raised antistreptolysin O(ASO) without evidence of recent streptococcal infection  Isolated chorea  pancarditis
  • 9. Carditis  Pancarditis  Declines with increasing age  (90% at 3yrs-30% in adolescent)  Breathlessness  Palpitation  Chest pain  Tachycardia  Cardiac enlargement  New or changed cardiac murmur
  • 10. Carditis  Soft MDM(Carey coombs murmur)  AR 90%  TV & PV rarely involved  Pericarditis  Cardiac failure  ECG –conduction defect,ST-T changes
  • 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
  • 23. Chronic rheumatic heart disease  Mitral valve – more than 90 %  Aortic valve  Tricuspid valve  Pulmonary valve  Isolated mitral stenosis-25%  Mixed mitral stenosis & regurgitation
  • 24. Pathology  Progressive fibrosis  Predominantly involved heart valves  Involvement of pericardium & myocardium m/contribute to heart failure & conduction disorder  Fusion of the mitral valve commissures & shortening of the cordae tendinae –mitral stenosis+/- mitral regurgitation  Similar changes in other valves
  • 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
  • 33. Clinical features Symptoms Signs  Breathlessness  AF  Fatigue  Mitral facies  Oedema  Auscultation;  Ascites loud 1st heart sound  Palpitation opening snap  Haemoptysis Mid-diastolic murmur  Cough  Signs of raised pulmonary  Chest pain capillary pressure  Symptoms of crepitations,pulmonary thromboembolic oedema,effusions complications  Signs of pulmonary hypertension RV heave,loud P2
  • 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
  • 44. Clinical features Symptoms Signs  Breathlessness  AF/flutter/cardiomegaly  Fatigue  Auscultation; apical pansystolic  Palpitation murmur  Oedema ±thrill  Ascites soft S1,apical S3  Signs of raised pulmonary venous congestion (crepitations,pulmonary oedema,effusions)  Signs of pulmonary hypertension & RHF RV heave,loud P2
  • 45. Investigations ECG Echo  LAH(If not in AF)  Thickened immobile cusps  LVH  Reduced valve area CXR  Reduced rate of diastolic  Enlarged LA filling of LV  Enlarged LV Doppler  Signs of pulmonary venous  Detects & quantifies congestion regurgitation  Pulmonary oedema Cardiac catheterization  Dilated LA,LV,MR  Pulmonary hypertension  Assessment of coexisting coronary artery disease
  • 46. Treatment Medical treatment  Moderate severity Definitive treatment  Pts remain symptomatic with medical treatment  Progressive radiological cardiac enlargement or echo cardiac evidence of deteriorating LV function  Mitral valve replacement/repair
  • 47. Treatment Atrial fibrillation  Anticoagulant  Digoxin Heart failure  Diuretics  Vasodilators e.g ACEI Prophylaxis of infective endocarditis  Antibiotics
  • 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
  • 49. Aortic valve disease disease Aortic valve
  • 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
  • 52. Pathophysiology Steadily increase pressure gradient across the AV LV increasingly hypertrophied Inadequate coronary blood flow Angina
  • 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
  • 54. Clinical features Symptoms Signs Mild to moderate-  Slow rising carotid pulse asymptomatic  Narrow pulse pressure  Exertional dyspnoea  Thrusting apex beat(LV  Angina overload)  Exertional syncope  Harsh ejection systolic  Sudden death murmur  Episodes of acute  Soft S2 pulmonary oedema  Signs of pulmonary venous congestion(crepitations,pul monary oedema)
  • 55. Investigations ECG Doppler  Severity of stenosis  LVH  LBBB  Detection of CXR associated aortic  Normal regurgitation  Enlarged LV Cardiac catheterization  Dilated ascending aorta(PA )  Assessment of coexisting  Calcified valve(lateral) coronary artery disease  Pressure gradient b/t LV & Echo aorta  calcified valve with CT/MRI restricted opening  Degree of valve  Hypertrophied LV calcification & stenosis
  • 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
  • 59. Aortic regurgitation Aetiology  Congenital  Bicuspid or disproportionate cusps Acquired  Rheumatic disease  Infective endocarditis  Trauma  Aortic dilatation(marfan’s syndrome,aneurysm,dissectionsyphillis,ankylosing spondylitis
  • 60. Clinical features Symptoms Mild- moderate AR  Often asymptomatic  Awareness of heart beat Severe AR  Breathlessness  Angina
  • 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
  • 67. Tricuspid regurgitation Causes Primary  Rheumatic heart disease  Endocarditis  Ebstein’s congenital anomaly Secondary Rv dilatation( chronic LHF RV infarction Pulmonary hypertension( corpulmonale)
  • 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
  • 71. Pulmonary Valve disease Pulmonary valve Disease
  • 72. Pulmonary stenosis Causes  Carcinoid syndrome  Usually congenital  Isolated or associated with other abnormalities e.g TOF
  • 73. Clinical features  ESM at left upper sternum  Radiation to left shoulder  Thrill  Preceded ejection click  Wide split S2  Loud harsh murmur  inaudible P2   RV heave  Prominent a wave in JVP
  • 74. Investigations ECG  RVH CXR  Post-stenotic dilation in the pulmonary artery Doppler echo
  • 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