Rhd 11 4-2016

1. RHEUMATIC FEVERRHEUMATIC FEVER
&&
RHEUMATIC HEARTRHEUMATIC HEART
DISEASEDISEASE

2. ValvesValves
 The four cardiac valves (tricuspid, pulmonary, mitral,The four cardiac valves (tricuspid, pulmonary, mitral,
and aortic) maintain unidirectional blood flowand aortic) maintain unidirectional blood flow
 The ability of the valves to permit unobstructedThe ability of the valves to permit unobstructed
forward flow depends on the mobility and pliabilityforward flow depends on the mobility and pliability
of their leafletsof their leaflets
 The competency (ability to prevent reverse flow) ofThe competency (ability to prevent reverse flow) of
thethe semilunarsemilunar valves (aortic and pulmonaryvalves (aortic and pulmonary) depends) depends
on the stretching and molding of their three leafletson the stretching and molding of their three leaflets
(cusps)(cusps)

3.  TheThe atrioventricular (AV) valves -(mitral andatrioventricular (AV) valves -(mitral and
tricuspidtricuspid))
 Their free margins are tethered to the ventricularTheir free margins are tethered to the ventricular
wall by many delicate tendinous cordswall by many delicate tendinous cords (chordae(chordae
tendineae)tendineae) attached to papillary muscles that areattached to papillary muscles that are
contiguous with the underlying ventricular wallscontiguous with the underlying ventricular walls
 Valve competency depends on the coordinatedValve competency depends on the coordinated
actions of annulus (the outer edge of the valveactions of annulus (the outer edge of the valve
orifice, where the leaflets attach)orifice, where the leaflets attach)

4. CHRONIC RH. VALVULITISCHRONIC RH. VALVULITIS

5. RHEUMATIC FEVERRHEUMATIC FEVER
It is an acute, immunologically mediated, multisystemIt is an acute, immunologically mediated, multisystem
inflammatory disease that occurs a few weeks following aninflammatory disease that occurs a few weeks following an
episode ofepisode of group A streptococcal pharyngitisgroup A streptococcal pharyngitis
Note: Rheumatic fever does not follow infections byNote: Rheumatic fever does not follow infections by
streptococci at other sitesstreptococci at other sites
Acute rheumatic carditis during the active phase of RFAcute rheumatic carditis during the active phase of RF
▼▼
Chronic rheumatic heart disease (RHD)Chronic rheumatic heart disease (RHD)

6. Consequence of RFConsequence of RF
Chronic valvular deformities, characterizedChronic valvular deformities, characterized
principally by deforming fibrotic valvular diseaseprincipally by deforming fibrotic valvular disease
(Mitral Stenosis)(Mitral Stenosis)
↓↓
Produces permanent dysfunctionProduces permanent dysfunction
↓↓
May be fatal( Decades later )May be fatal( Decades later )

7. TheThe incidence and mortalityincidence and mortality
1.1. Improved socioeconomic conditionsImproved socioeconomic conditions
2.2. Rapid diagnosis & T/t of streptococcal pharyngitisRapid diagnosis & T/t of streptococcal pharyngitis
3.3. UnexplainedUnexplained ↓↓ in the virulence of group Ain the virulence of group A
streptococcistreptococci
RHD - Important public health problem inRHD - Important public health problem in
developing countries & many crowded urban areasdeveloping countries & many crowded urban areas
in the Western worldin the Western world

8. MORPHOLOGYMORPHOLOGY
ACUTE RFACUTE RF
Focal inflammatory lesions are found in various tissues, mostFocal inflammatory lesions are found in various tissues, most
distinctive within the heart-distinctive within the heart- Aschoff bodiesAschoff bodies
ASCHOFF BODIES-ASCHOFF BODIES-
- Characteristic granulomatous lesion of rheumatic- Characteristic granulomatous lesion of rheumatic
myocarditismyocarditis
- Foci of swollen eosinophilic collagen surrounded by- Foci of swollen eosinophilic collagen surrounded by
lymphocytes , Ascoff giant cells, occ. plasma cells & plumplymphocytes , Ascoff giant cells, occ. plasma cells & plump
macrophages calledmacrophages called Anitschkow cellsAnitschkow cells

9. ACUTE RHDACUTE RHD

10. Abundant cytoplasm and central round-to-Abundant cytoplasm and central round-to-
ovoid nuclei, slender, wavy ribbon likeovoid nuclei, slender, wavy ribbon like
chromatin in the centre-chromatin in the centre- ("caterpillar("caterpillar
cells")cells")

12. ACUTE RFACUTE RF
 PancarditisPancarditis- Diffuse inflammation and Aschoff bodies may- Diffuse inflammation and Aschoff bodies may
be found in any of the three layers of the heartbe found in any of the three layers of the heart
 Bread-and-butter pericarditis-Bread-and-butter pericarditis- Inflammation accompaniedInflammation accompanied
by a fibrinous or serofibrinous pericardial exudateby a fibrinous or serofibrinous pericardial exudate
 MyocarditisMyocarditis—Scattered Aschoff bodies within the—Scattered Aschoff bodies within the
interstitial connective tissue often perivascularinterstitial connective tissue often perivascular
 Small vegetationsSmall vegetations along the line of closure- Concomitantalong the line of closure- Concomitant
involvement of the endocardium and the left-sided valvesinvolvement of the endocardium and the left-sided valves
 MacCallum plaques-MacCallum plaques- Subendocardial lesions in LASubendocardial lesions in LA

13. CHRONIC RHDCHRONIC RHD
GrossGross:-:-
- Valvular leaflet thickening- Valvular leaflet thickening
- Commissural fusion , shortening thickening & fusion of theCommissural fusion , shortening thickening & fusion of the
tendinous cordstendinous cords
- Fibrous bridging across the valvular commissures andFibrous bridging across the valvular commissures and
calcification createcalcification create "fish mouth" or "buttonhole" stenoses"fish mouth" or "buttonhole" stenoses
MicroscopicallyMicroscopically :-:-
 Diffuse fibrosisDiffuse fibrosis
 NeovascularizationNeovascularization

14. CHRONIC RH. VALVULITISCHRONIC RH. VALVULITIS

15. CHRONIC RH. VALVULITISCHRONIC RH. VALVULITIS

16. Calcific Rh.ASCalcific Rh.AS

17. RHEUMATIC ASRHEUMATIC AS

18. Frequency of valvular involvementFrequency of valvular involvement

19. Mitral valve alone is involved in 65% to 70% of casesMitral valve alone is involved in 65% to 70% of cases
Mitral and aortic in about 25%Mitral and aortic in about 25%
Tricuspid valve –less oftenTricuspid valve –less often
Pulmonary valve rarely involvedPulmonary valve rarely involved
Tight mitral stenosis- Left atrium progressively dilatesTight mitral stenosis- Left atrium progressively dilates
and may harbor mural thrombusand may harbor mural thrombus
Long-standing congestive changes in the lungs mayLong-standing congestive changes in the lungs may
induce pulmonary vascular and parenchymalinduce pulmonary vascular and parenchymal
changes- RVHchanges- RVH

20. PATHOGENESISPATHOGENESIS
- Exact pathogenesis remains uncertain- Exact pathogenesis remains uncertain
- Acute rheumatic fever is a hypersensitivity reactionAcute rheumatic fever is a hypersensitivity reaction
induced by group A streptococciinduced by group A streptococci ––
- Antibodies directed against theAntibodies directed against the M proteinsM proteins of certainof certain
strains of streptococci cross-react with glycoproteinstrains of streptococci cross-react with glycoprotein
antigens in theantigens in the heart, joints & other tissuesheart, joints & other tissues

22. RF results from an immune response againstRF results from an immune response against
the offending bacteriathe offending bacteria
 The onset of symptoms 2 to 3 weeks after infectionThe onset of symptoms 2 to 3 weeks after infection
and the absence of streptococci from the lesionsand the absence of streptococci from the lesions
 Nature of cross-reacting antigens has been difficultNature of cross-reacting antigens has been difficult
to define, autoimmune response against self-to define, autoimmune response against self-
antigensantigens
 Genetic susceptibility influences theGenetic susceptibility influences the
hypersensitivity reactionhypersensitivity reaction

23. DiagnosisDiagnosis
Jones criteriaJones criteria
Evidence of a preceding group A streptococcalEvidence of a preceding group A streptococcal
infection with the presence ofinfection with the presence of
- 2 major manifestations- 2 major manifestations
- 1 major & 2 minor manifestations- 1 major & 2 minor manifestations

24. CLINICAL FEATURESCLINICAL FEATURES
Major manifestationsMajor manifestations
(1) Migratory polyarthritis of the large joints(1) Migratory polyarthritis of the large joints
(2) Carditis(2) Carditis
(3) Subcutaneous nodules(3) Subcutaneous nodules
(4) Erythema marginatum(4) Erythema marginatum
(5) Sydenham chorea(5) Sydenham chorea

25. Minor manifestationsMinor manifestations
- Previous HistoryPrevious History
- FeverFever
- ArthralgiaArthralgia
- Elevated blood levels of acute phase reactantsElevated blood levels of acute phase reactants
(ESR, CRP, leukocytosis & ECG findings)(ESR, CRP, leukocytosis & ECG findings)

26. Lab testsLab tests
 Pharyngeal cultures for streptococci are negative byPharyngeal cultures for streptococci are negative by
the time the illness beginsthe time the illness begins
 Antibodies to one or more streptococcal enzymesAntibodies to one or more streptococcal enzymes
can be detected in the sera of most patients, suchcan be detected in the sera of most patients, such
as :-as :-
antistreptolysin Oantistreptolysin O
anti-DNAse Banti-DNAse B
antihyaluronidaseantihyaluronidase

27. CLINICAL COURSECLINICAL COURSE
Acute RF appears most often in children ( 5 – 15) but 20% ofAcute RF appears most often in children ( 5 – 15) but 20% of
first attacks occur in middle to later lifefirst attacks occur in middle to later life
The long-term prognosis is highly variableThe long-term prognosis is highly variable
In some cases, there is a relentless cycle of valvularIn some cases, there is a relentless cycle of valvular
deformity yielding hemodynamic abnormalitydeformity yielding hemodynamic abnormality
causing deforming fibrosiscausing deforming fibrosis
1% of patients die from fulminant RF1% of patients die from fulminant RF
Cause of death is heart failure due to myocarditisCause of death is heart failure due to myocarditis

28. The predominant clinical manifestations are :-The predominant clinical manifestations are :-
 ArthritisArthritis -migratory polyarthritis accompanied-migratory polyarthritis accompanied
by feverby fever
 acute carditisacute carditis - pericardial friction rubs, weak- pericardial friction rubs, weak
heart sounds, tachycardia & arrhythmias,heart sounds, tachycardia & arrhythmias,
myocarditis- functional mitral valvemyocarditis- functional mitral valve
insufficiency - heart failureinsufficiency - heart failure

29. Myocardial and pericardial components ofMyocardial and pericardial components of
rheumatic pancarditis resolverheumatic pancarditis resolve
Acute valvulitis often results in long termAcute valvulitis often results in long term
structural and functional alterationsstructural and functional alterations

30. Complications of chronic RHDComplications of chronic RHD
 Bacterial endocarditisBacterial endocarditis
 Mural thrombiMural thrombi
 CCFCCF
 AdhesionsAdhesions

31. Treatment of RHDTreatment of RHD
Symptomatic T/t- Salicylates & steroidsSymptomatic T/t- Salicylates & steroids
Surgical repair of diseased valves by incising theSurgical repair of diseased valves by incising the
fused mitral valve commissures & replacementfused mitral valve commissures & replacement
with prosthetic deviceswith prosthetic devices

32. StenosisStenosis- Failure of valve to open completely thereby- Failure of valve to open completely thereby
impeding forward flowimpeding forward flow
Insufficiency-Insufficiency- Failure of a valve to close completelyFailure of a valve to close completely
thereby allowing reverse flowthereby allowing reverse flow
Functional regurgitation-Functional regurgitation- Valve incompetent owing toValve incompetent owing to
either dilatation of ventricle or dilatation of aortic/either dilatation of ventricle or dilatation of aortic/
pul arterypul artery
Murmur-Murmur- Abnormalities of flow producing abnormalAbnormalities of flow producing abnormal
heart soundsheart sounds