A brief overview on rheumatic heart disease

1. RHEUMATIC HEART
DISEASE
Dr Wanjau G.K
gwanjau331@gmail.com

2. Rheumatic fever
• Is a noncontagious acute fever marked by
inflammation and pain in the joints.It chiefly
affecting young people and is caused by
streptococcal infection
• Abnormally regulated immune response that
results in inflammation of target organs
• It affects:heart,joints,cns,subcutaneous tissues
and skin
.

3. epidemiology
• Magnitude enormous in developing countries
• 15.6 million people worldwide have the disease
• 470 000 new cases greater than 60% of which will
develop rheumatic heart disease
• 230000 deaths results annually from its
complication
• All these in developing countries

4. epidemiology
• Incidence rate is as high as 50 cases per 100 000 children
• RF in the 21st century is appears to be a disease of
crowding and poverty
• Other factors include:
• a) no clear genetic predisposition
• b)age- common in aged 5-15,rare in infants and preschool-
aged children.common in yong adults,but incidence falls
after adolescence and rare after 35 years
• c) sex-no clear cut sexual predilection but although
certain clinical manifestations eg mital stenosis and
sydenham chorea more common in female who have gone
through puberty

5. pathogenesis
• Group A beta haemolytic streptococcal infection may
lead to rheumatic fever
• Attack rate is 0.3-3 after the pharyngitis
• Other conditions caused by the microorganism include:
• Tonsillopharyngitis
• Acute otitis media
• Pneumonia
• Skin and soft tissue infection(scarlet fever)
• Bacteremia and
• Also affect lymphatic system
Rheumatic fever only follow pharyngeal infection
• The strains causing the condition
• M type 3,5,6,14 18,19 and 24

6. pathogenesis
• Hypothesis developed in the first five decade to
explain the pathogenesis of rheumatic fever by
streptococcus:
1. direct infection by group A streptococci
2. Effects of streptococcal toxin(streptolysin
O) to the myocardium,valves,synovium and
brain
3. Molecular mimicry in association with an
abnormal imune response----the most
feasible and well described.

7. PATHOGENESIS
• Antibody-mediated disease that follows a group A
streptococcal infection of the pharynx.
• Host develops antibodies against group A
streptococcal M proteins.
• Antibodies that are produced cross-react with
similar proteins in human tissue (called
molecular mimicry).
• Type II antibody-mediated hypersensitivity
reaction

8. Clinical manifestation
• The symptoms may occur alone or in various
combination
1.SORE THROAT
35-60% recall having sore throat several weeks
preceeding the rheumatic fever.
Penicillin or antibiotic reduces ARF by 80%

9. ARF-CLINICAL FEATURES
2.POLYARTHRITIS
75% of cases of first attack.likelihood increases with the age
of the patient (92% in adults)
Symmetrical and involve large joints-knees,ankle,elbows and
wrists.Tenosynovitis common in adults and may be severe
enough to suggest disseminated gonococcal disease
The joint involvement is migratory or additive
Monoarthritis can occur if NSAID is used early-not recognised
in JONES criteria
Subside aftrer within 4 weeks,otherwise……think of a
different diagnosis
Swelling,redness,warmth,tenderness

10. ARF-CLINICAL FEATURES
3.CARDITIS
30-60% cases of first attack.More common in younger
children.
Does not occur in adults
Severe-CCF
Shortness of breath,exertional
dyspnoea,cough,paroxysmal nocturnal dyspnoea or ay
be asymptomatic------auscultate
Is the only significant cause of death in ARF
Usually pancarditis

11. ARF-CLINICAL FEATURES
4.Syndenham chorea
• Upto 25% of cases in children but very rare in
adults.more common in girls
• Molecular mimicry with Autoantibodies reacting with
brain gangliosides
• 1-6months after strept infection
• Resolves 2-3 years without permanent damage
• Emotional lability,personality change,muscular
weakness,uncordinated,involuntary purposless
movements.

12. ARF-CLINICAL FEATURES
5.Erythema marginatum
• Aprox.10%
• Very rare in adults
• Non pruritic,painless,serpiginous erythematous erruptions on the
trunk and proximal extremities,may go unnoticed-clothing
• Only noted in fair skinned patients
• May persist weeks to months
• Evanescent,moving over the skin in a serpiginous pattern
• Like smoke rings,
• Advance at the margins while clearing at the centre
• May be macular and may develop and dissapear in minutes,appearing
to change shape while being examined

13. ARF-CLINICAL FEATURES
6.Subcutaneous nodules-
rarely noticed by the patient.(painless)
Found primarily over the bony surfaces or prominences
and in tendon sheath
Common sites include elbows,knees,ankles,over the
Achilles gtendon,the back of the scalp and spinous
process of the vertebrae
DD:Nodules of rheumatoid arthritis
Asociated with severe carditis
OTHERS
Fever,abdominal pain,arthralgias,malaise,and epistaxis

14. DIAGNOSIS
JONES CRITERIA FOR THE DIAGNOSIS OF
RHEUMATIC FEVER
Major manifestations
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules

15. Minor manifestations
• Fever
• Arthralgia
• Previous rheumatic fever
• Raised ESR or CRP
• Leucocytosis
• First-degree AV block
PLUS Supporting evidence of preceding streptococcal
infection: recent scarlet fever, raised antistreptolysin
0 or other streptococcal antibody titre, positive
throat culture

16. DIFFERENTIAL DIAGNOSIS
• Gonococcal arthritis
• Juvenile idiopathic arthritis
• Lyme disease
• Mixed connective tissue disease
• Sle
• RA
• Septic arthritis
• Sickle cell anaemia

17. Rheumatic heart
disease.

18. RHEUMATIC HEART DISEASE
Is a chronic heart condition due to rheumatic
fever, caused due to cardiac inflammation and
scarring triggered by an autoimmune reaction to
infection with group A streptococcus

19. Carditis-pan
• Most common cause of death in acute RF
• Aschoff bodies are present in myocardial tissue
• It most commonly involves the MV, followed by
the AV, followed by the TV.
• Sterile, verrucous vegetations develop along the
line of closure of the valve
• Valve regurgitation->stenosis

20. Pattern of valvular injury
• Mitral valve affected most-begins as a small
veruccae composed of fibrin and blood clot
• Right sided valvular manifestations rare
• Structural changes of the valve
• Loss of some valvular substance
• Shortening and thickening of chord tendinae

21. pathogenesis
• Mitral insufficiency coupled with
endocarditis,pericarditis and myocarditis-hearf
failure
• Enlargement of LV,dilatation of LV due to
regurgitation,pulmonary congestion-symptoms of
left sided heart failure.
• Chronic mitral insufficiency-pulmonary HTN-
enlarged RV and RA-symptoms of right sided
heart failure

22. S&S
• Physical signs depends on severity
• Mild-no signs of heart failure
-precordium quiet
-high pitched holosystolic murmur radiating to the
axilla

23. S&S
In severe:signs of chronic heart failure
• Fatugue
• Effort intolerance
• Anorrexia
• Abdominal pain
• Dyspnoea
• Cough
• Enlarged heart
• Elevation of systemic venous preassure
• ortho[pnoea
• Basilar rales
• Oedema
• Gallop rhythm
• Holosystolic murmur of mitral or tricuspid valve regurgitation may be heard
• Pneumonitis with or without atelectasis

24. Investigations
• Evidence of a systemic illness (non-specific); Leucocytosis,
raised ESR, raised CRP
• Evidence of preceding streptococcal infection (specific)
Throat swab culture: group A β-haemolytic streptococci (also
from family members and contacts)
• Antistreptolysin 0 antibodies (ASO titres): rising titres, or
levels of > 200 U (adults) or > 300 U (children)
• Evidence of carditis
• Chest X-ray: cardiomegaly; pulmonary congestion
• ECG: first- and rarely second-degree heart block; features of
pericarditis; T-wave inversion; reduction in QRS voltages
• Echocardiography: cardiac dilatation and valve abnormalities

25. Treatment of the acute attack
• Penicillin……if allergic erythromycin and cephalosporin.
• Bed rest is important as it lessens joint pain and reduces
cardiac workload.
• Aspirin This will usually relieve the symptoms of arthritis
rapidly and a prompt response (within 24 hours) helps to
confirm the diagnosis.Mild toxic effects include nausea,
tinnitus and deafness; more serious ones are vomiting,
tachypnoea and acidosis. Aspirin should be continued until
the ESR has fallen and then gradually tailed off.
• Corticosteroids These produce more rapid symptomatic
relief than aspirin, and are indicated in cases with carditis
or severe arthritis

26. CHRONIC RHEUMATIC HEART
DISEASE
• Progressive fibrosis.
• The heart valves are predominantly affected but
involvement of the pericardium and myocardium
may contribute to heart failure and conduction
disorders.
• Fusion of the mitral valve commissures and
shortening of the chordae tendineae may lead to
mitral stenosis with or without regurgitation.