This document discusses rheumatic heart disease, which is a chronic condition that develops due to damage caused by acute rheumatic fever during childhood. Rheumatic fever is an inflammatory disease that occurs after a streptococcal throat infection and can cause long-term heart valve problems and scarring referred to as rheumatic heart disease. It most often affects the mitral valve and is characterized by narrowed or leaky valves. Treatment involves antibiotics to prevent recurrence, as well as medications and potentially surgery to address heart failure and valve abnormalities.

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