Types and diagnosis and treatment of RHD

1. Rheumatc heart disease
Professor mohammed Ahmed
Bamashmos

2. Acute rheumatic fever
• Rheumatic fever is an acute, immunological
mediated , multisystem ( heart , joint , CNS, skin,
subcutaneous tissue) affecting inflammatory
disease that occurs a few weeks following an
episode of group A Beta streptococcal pharyngitis
• Chronic stage of RF involves all the layers of heart
( pancarditis) causing major cardiac sequelae
referred to as rheumatic heart disease ( RHD).

3. Rheumatic Heart Disease
• Epidemiology
a. Occurs at 5 to 15 years of age
b. Develops over 1 to 5 weeks ( average 20 days) after
group A streptococcal (streptococcus pyogenes)
pharyngitis
– Only site for infection leading to Rheumatic fever
c. Risk factors for streptococcal pharayngitis
(1) crowding
(2) poverty
(3) young age
d. Recurrent RF produces chronic valvular disease

6. • Pathogenesis
a. Immunie- mediated disease that follows group A streptococcal
infection – sore thorat /pharyngitis
b. Antibodies develop against group A streptococcal M proteins
(1.) antibobies cross – react with similar protiens in human tissue
( called mimicry)
- type II hypersensitivity reaction
(2) cell- mediated immunity has also been implicated
- type IV hypersensitivity reaction
(3) nephrogenic strains of group A streptococcus lack M- protiens
- never associated with Rheumatic fever
c. Acute rheumatic fever ( carditis, arthritis ) 🡪 Rheumatic heart
disease
Chronic RF : by damage valves ( mitral , aortic) by fibrosis

7. • Histologically-
– Fibrinoid degeneration is seen in the collagen of
connective tissue
– Aschoff nodules are pathognomonic and occur
only in the heart.

8. Clinical findings
• Jones Criteria (1992 Revision) for Diagnosis of
Acute Rheumatic Fever*
• Major manifestations
• 1. Carditis (~ 35%)
• 2. Migratory Polyarthritis (~ 75%)
• 3. Chorea (~10%)
• 4. Erythema marginatum (10%)
• 5. Subcutaneous nodules (~10%)

9. • Minor manifestations
• 1. Fever
• 2. Arthralgias
• 3. Previous rheumatic fever or rheumatic heart
disease
• 4. Increased C-reactive protein (CRP)
concentrations or erythrocyte sedimentation rate
( ESR)
• 5. Prolonged PR interval on electrocardiogram
• 6. Absolute neutrophilic leukocytosis

10. Evidence of antecedent group A streptococcal infection
1. Positive throat culture
2. Rapid antigen test positive for group A streptococcus (ASO
titre)
3. Recent scarlet fever
• *A firm diagnosis requires
1) 2 major manifestations OR 1 major and 2 minor manifestations
and
2) evidence of a recent streptococcal infection.
• However, when chorea or carditis is clearly present, evidence of
an antecedent group A streptococcal infection is not necessary.

11. 1. Migratory polyarthritis
• This is usually an early feature and most common
• acute, painful, asymmetric and migratory
inflammation of the large joints (typically the knees,
ankles, elbows and wrists).
• The joints are involved in quick succession and are
usually red, swollen and tender for between a day and
up to 4 weeks.
• The pain characteristically responds to aspirin; if it
does not, the diagnosis is in doubt.

12. 2.Carditis :
• All 3 layers can be involved ( Pancarditis)
• Cardiomegaly, congestive heart failure Acute
pericarditis, pericardial effusion
• Apical pansystolic murmur (mitral regurgitation)
• Apical mid-diastolic murmur (Carey Coombs)
• Basal diastolic (aortic regurgitation)

13. 3. Erythema marginatum occurs in less than 5%
of patients.
The lesions start as red macules (blotches)
which fade in the center but remain red at
the edges and occur mainly on the trunk and
proximal extremities
• Often associated with chronic carditis

14. 4. Subcutaneous nodules occur in 5-7% of
patients. They are small (0.5-2.0 cm), firm and
painless, and are best felt over extensor
surfaces of bone or tendons.
• Always associated with severe carditis

15. 5. Sydenham's chorea (St Vitus dance)
• Involuntary choreiform movements of the
hands, feet or face. Speech may be explosive
and halting. Spontaneous recovery usually
occurs within a few months.
• Clinical signs- pronator sign, jack in the box
sign , milking sign of hands

17. INVESTIGATIONS IN ACUTE RHEUMATIC FEVER
• Evidence of a systemic illness (non-specific)
– Leucocytosis, raised ESR, raised CRP
• Evidence of preceding streptococcal infection
(specific)
– Throat swab culture: group A ß-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

19. Treatment of the acute attack
• Bed rest and supportive therapy
– The duration of bed rest should be guided by symptoms
and markers of inflammation (e.g. temperature, leukocyte
count and ESR and should be continued until these have
settled.
• Treatment of congestive cardiac failure:
digitalis, diuretics, ACE inhibitor
• Treatment of chorea:
diazepam or haloperidol
• Rest to joints & supportive splinting

20. • Pain relief
• Aspirin
– A reasonable starting dose is 60 mg/kg body weight per
day, divided into six doses.
– In adults, 100 mg/kg per day may be needed up to the
limits of tolerance or a maximum of 8 g per day.
– 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.
– Prednisolone, 1.0-2.0 mg/kg per day in divided doses,
should be continued until the ESR is normal then tailed off.

21. • Antibiotic
– A single dose of Benzathine penicillin 1.2 million U
i.m.(after skin sensitivity) or oral
phenoxymethylpenicillin 250 mg for children and
500mg for 6-8hourly for 10 days
– If the patient is penicillin-allergic; erythromycin

22. PREVENTION IN RF
• Preventing Strep Throat - Vaccine ?
• Treating Strep Throat infection
• Preventing Rheumatic recurrence by
chemoprophylaxis
• Treating RHD

23. SECONDARY PROPHYLAXIS
• Continuous chemoprophylaxis to prevent
recurrence in a patient who had an initial
attack of RF
• STRATEGY
– Chemoprophylaxis
– Treat breakthrough Infection

24. Chemoprophylaxis
• BPG 1.2 million U IM 3 weekly
• Penicillin V 250 mg BD PO daily
• Erythromycin 250 mg BD PO daily
• Sulfadiazine 500 mg OD PO daily
1000 mg

25. • Secondary rheumatic fever prophylaxis
• Bezathine penicillin 1.2 million unit i.m. –
every 3-4 weeks

26. CHRONIC RHEUMATIC HEART DISEASE

27. • Rheumatic heart disease (RHD) is the long-term
consequence of acute rheumatic fever during
childhood.
• As the initial damage (inflammation) subsides,
scar tissue forms leaving the valves either too
narrow (stenotic) or too "leaky" (insufficient); also
atrial dilation, arrhythmias, and ventricular
dysfunction
• Rheumatic heart disease refers to this permanent
scarring of the heart valves

28. • Two-thirds of cases occur in women
• The mitral valve is affected in more than 90% of
cases; the aortic valve is the next most frequently
affected, followed by the tricuspid and then the
pulmonary valve
• Isolated mitral stenosis accounts for about 25% of
all cases
• develops 2-10 years after an episode of acute
rheumatic fever, and recurrent episodes may
cause progressive damage to the valves.

29. • Treatment:
• Monthly treatment with benzathine penicilline
IM to prevent recurrences
• Afterload reduction (ie, using ACE inhibitor
captopril) for heart failure
• For symptomatic congestive heart failure
uncontrolled by medication, surgery may involve
repairing a damaged valves without replacing it
• Permanent pacemaker may be required for AF