2. Disease Burden
• Rheumatic heart disease(RHD) is the most significant
sequelae of rheumatic fever(RF)
• RHD - 60% of all cardiac diseases
• 0.3-3% of the population – inherent susceptibility to RF
• World wide, 2.4 million children between 5-14 yrs –RHD
(2005)—79% less developed countries(LDC)
• New RF-- >336,000/year—95% (LDC)
• 60% of RF → RHD
• Acute RF – peak 5yrs-14yrs
• RHD- peak 3rd &4th decade
• Recurrences – Even in the 5th decade
3. Disease Burden
Reported prevalence of rheumatic heart disease in schoolchildren
WHO Region (country, city) Year Rate(per1000population)
Northern India 1992–1993 1.9–4.8
India 1984–1995 1.0–5.4
Nepal (Kathmandu) 1997 1.2
Sri Lanka 1998 6
Estimated deaths due to RHD 5.5/100,000 (2000)
(492,000/year)
4. Pathogenesis
• Associated with pharyngitis caused by Group A
beta haemolytic streptococcus
• Delayed autoimmune response
• Precise pathogenic mechanism unkown
• Possibly involves B and T lymphocyte activation by
streptococcal antigens and super antigens
• Susceptible host
• Environment- poor living conditions, over crowding,
poor access to health care facilities
5. Group A beta haemolytic
streptococcus (Strept. pyogens)
7. Clinical Features
• Symptoms of ARF start 2-3 weeks after pharyngitis
Polyarthritis >75%
Large joints. Good prognosis.
Inverse relationship with Carditis ;Severe (A) 10%,
Arthralgia 33%, No (A) 50%
Carditis ~ 60%
Chorea ~ 10%
Late occurrence, 1 - 7 months after infection.
Common in children and young females. Lasts for
a few weeks to upto 2 years
8. Clinical Features…….
• Erythema Marginatum ~ 10%
Occurs early in the disease. May appear for months.
Bright pink macule/papule. Spreads outwards.
“Smokey rings under the skin”. Blanch on pressure.
Coexist with nodules. Associated with carditis.
• Subcutaneous Nodules 20%
Associated with severe carditis. Firm non tender mobile.
Over bony prominances. 0.5-2cm. Occur in crops. 1-
2weeks following carditis. Last for 1-2weeks.
9. Clinical Features…….
• Arthralgia
Large joints. Mild to severe pain.
• Fever
Almost all patients at onset.38-40 deg. C
• Abdominal pain and epistaxis
May precede major manifestations by hours/days. Abd.
Pain may mimic acute appendicitis but ESR higher in
RF
15. 2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart
disease (based on the revised Jones criteria)
Diagnostic categories
• Primary episode of RF
• Recurrent attack of RF in a patient without
established rheumatic heart disease
• Recurrent attack of RF in a patient with
established rheumatic heart disease
• Rheumatic chorea.
• Insidious onset rheumatic carditis
• Chronic valve lesions of RHD (patients
presenting for the first time with pure
mitral stenosis or mixed mitral valve
disease and/or aortic valve disease)
Criteria
• Two major or one major and two minor
manifestations plus evidence of a preceding group A
streptococcalinfection
•Two major or one major and two minor
manifestations plus evidence of a preceding group A
streptococcal infection.
• Two minor manifestations plus evidence of a
preceding group A streptococcal infection
• Other major manifestations or evidence of group A
streptococcal infection not required.
•Do not require any other criteria to be diagnosed as
having rheumatic heart disease.
17. Evidence supportive of Streptococcal
infection in the last 45 days
• Antistreptolysin O Titre (ASOT)
Appears after 18 days of infection. Peaks in 3 to 4 months.An
elevated or a rising titre is diagnostic.
• Throat Swab for culture/ rapid antigen test
May be positive in carriers as well (colonisation)
• Other Strept. Antibodies
• Recent scarlet fever
18. Treatment of acute illness
• Hospital Admission – Facilitates diagnostic tests and monitoring for
carditis
• Bed/chair rest. 4-6 weeks for carditis
• Antimicrobial therapy (WHO 2009)
Primary Prophylaxis of RHD / Treatment of Strept. pharyngitis
IM Benzathine penicillin 1.2mu for adults and children over 20kg
Secondary Prevention of RHD/ Prophylaxis against pharyngitis
IM Benzathine penicillin as above 3-4 weekly
(This is the only proven cost effective measure to prevent RHD) (WHO 2005)
Alternatives – Phenoxymethyl penicillin, erythromycin
19. Duration of secondary prophylaxis
(WHO 2009)
• Severe valvular disease/ undergone valvular
surgery – Life long
• Proven carditis – 10 years after the last attack
or upto 26 of age years (whichever is longer)
• Without carditis – 5 years or 18 years of age
(whichever is longer)
• 4 weekly interval as routine (2-3 weekly only
for those who continue to get recurrent RF in
spite of strict adherence to 4 weekly regime)
20. • Suppression of Inflammation
Aspirin is the mainstay. (No clinical trials were conducted to compare
with NSAIDS).
Naproxen has been used (10-20mg/kg).
Aspirin 100mg/kg/day in 4-5 divided doses, Adults 6-8g/d, for 2
weeks then 60-70% for 4-6 more weeks. Should be continued till
disease activity settles (Clinical and acute phase reactants)
Steroids vs Aspirin – meta analysis shows no difference in long term
RHD outcome. But used in carditis
21. • Chorea
Severe chorea can lead to social withdrawal.
Recommended treatment Valpraote,
Carbamezapine, Haloperidol
Anti inflammatory drugs not effective
22. Prevention
• Improve living standards
• Primary prevention – treatment of strept.
Pharyngitis (Not proven to be cost effective)
• Secondary- Prophylactic antibiotics to prevent
recurrent strept sore throat and recurrence of
RF/RHD (Proven to be cost effective in
preventing RHD)
• Further studies needed
