This document provides information on the management of rheumatic fever and rheumatic heart disease. It discusses the causes, diagnostic criteria, clinical features, and treatment approaches for various manifestations of rheumatic fever including carditis, arthritis, chorea, and heart valve disease. Medical management involves antibiotics to treat streptococcal infections, anti-inflammatory drugs, and management of heart failure. Surgical interventions like valve repair or replacement are indicated for severe valve disease. Secondary prophylaxis with antibiotics aims to prevent recurrent rheumatic fever.

1. Management of Rheumatic Fever & Rheumatic
Heart Disease
Department of Cardiology
Yangon General Hospital

2. Rheumatic Fever (RF) & Rheumatic Heart Disease
(RHD)
 Pharyngeal infection with Lancefield group A ß-
haemolytic streptococci triggers rheumatic fever
2-4 weeks later.
 Rheumatic Heart Disease - Non-suppurative
complications of Group A streptococcal
pharyngitis due to a delayed immune response

3. Rheumatic Fever (RF) & Rheumatic Heart Disease
(RHD)
 Peak incidence : 5-15 yrs
 True infection (rising antibody response) or
carrier state (no rising antibody)
 True infection : at risk of developing RF and of
spreading the organism to close contacts
 Socioeconomic & environmental factors play an
indirect but important role in the magnitude and
severity of RF & RHD

4. Diagnostic criteria for rheumatic fever – modified
2015 Jones criteria ; Major criteria
Low risk population
 Carditis (clinical or
subclinical)
 Arthritis – only
polyarthritis
 Chorea
 Erythema marginatum
 Subcutaneous nodules
High risk population
 Carditis (clinical or
subclinical)
 Arthritis –
monoarthritis or
polyarthritis
 Polyarthralgia
 Chorea
 Erythema marginatum
 Subcutaneous nodules

5. Diagnostic criteria for rheumatic fever – modified
2015 Jones criteria ; Minor criteria
Low risk population
 Polyarthralgia
 Hyperpyrexia (≥ 38.5ºC)
 ESR ≥ 60 mm/h and/or
CRP ≥ 3.0 mg/dl
 Prolonged PR interval
(after taking into account
the differences related to
age; if there is no carditis
as a major criterion)
High risk population
 Monoarthralgia
 Hyperpyrexia (≥ 38.0ºC)
 ESR ≥ 30 mm/h and/or
CRP ≥ 3.0 mg/dl
 Prolonged PR interval
(after taking into account
the differences related to
age; if there is no carditis
as a major criterion)

6. Diagnosis of RF
 First episode of the disease –
 Two major criteria or
 one major and two minor criteria
 with evidence of antecedent group A β-
hemolytic
streptococcal infection
 Subsequent episodes -
 Two major criteria or
 one major and two minor criteria or
 three minor criteria

7. Clinical features of Rheumatic Carditis
 Pericarditis: (in primary episode or recurrence of RF) -rub
supported by echo evidence of PE and simultaneous
valvular involvement
 Myocarditis: unexplained CHF or cardiomegaly, almost
always associated with valvular involvement. RHD patients
– CHF, minor criteria, ↑ASO provide Rh: carditis
 Endocarditis/ valvulitis: Apical PSM ± MDM (Carey
Coombs), basal EDM in pt: who do not have RHD
 Pt: with previous RHD, change in the character of
murmur or the appearance of a new significant murmur
indicates the presence of carditis
 Role of Echo in diagnosis of carditis is essential

8. Medical Management of RF
General:
 Hospital admission- to confirm a diagnosis
 Bed rest – to monitor closely for the onset of carditis
 Rest period at least 4 weeks for carditis
 Investigations: throat culture, ASO, acute phase reactants;
ESR, CRP, CXR, ECG, Echo, blood culture to exclude IE
 Antimicrobial Tx: Eradication of the pharyngeal strept:
infection
 Two throat cultures before starting A/B

9. Medical Management of RF
 Suppression of the inflammatory process
 Should avoid premature administration of salicylates
 Aspirin 100 mg/kg/day divided into 4-5 doses
 (125 mg/kg/day in children) for adequate response but
avoid toxicity
 Reduce to 60-70 mg/kg/day for 3-6 weeks
 Naproxen 10-20 mg/kg/day if intolerant or allergic to
aspirin
 Corticosteroids: Not respond to Aspirin or for pericarditis
or HF
 Prednisolone 2 mg/kg/day (80 mg/day) or
methylprednisolone 2-3 wk, overlap with aspirin

10. Medical Management of RF
 HF in RF: bed rest, steroids, if severe symptoms,
Diuretics, ACEI, digoxin
 For chorea: self-limiting benign disease, no
threapy, Neuroleptics, benzodiazepines, anti-
epileptics (Haloperidol, Diazepam,
Carbamazepine)
 Steroids are not beneficial for chorea

11. Primary prevention of RF: Recommended
treatment for Streptococcal pharyngitis
Phenoxymethyl
penicillin
> 40 kg – 2–3 MIU/day
< 40 kg – 100,000 to 200,000
IU/kg/day
PO in 2 divided doses every 12
hours for 10 days
Benzylpenicillin >40 kg – 1.2 MIU
< 40 kg – 600,000 IU.
intramuscularly at a single dose
Cefadroxil > 40 kg – 1 g
< 40 kg – 30 mg/k
hypersensitivity to penicillin
single dose for 10 days
Cefalexin adults 500 mg BD
children 25– 50 mg/kg/day in 2 doses
hypersensitivity to penicillin
for 10 days
Erythromycin > 40 kg – 0.2–0.4 g
< 40 kg – 30–50 mg/kg/day
every 6–8 hours for 10 days
Clarithromycin > 40 kg – 250–500 mg every 12 hours
< 40 kg – 15 mg/kg/day in 2 doses
10 days
Azithromycin > 40 kg – 500 mg on the first day, then
250 mg for three consecutive days

12. Secondary prevention of RF
 Secondary prevention - prevention of subsequent
rheumatic fever relapses
 Duration of secondary prevention - determined
individually
 From 5 to 10 years from the last RF relapse, or up to 21
years of age (whichever is longer)

13. Suggested Duration of Secondary Prophylaxis
Patients without proven carditis For 5 yrs after the last attack or
until 18 yrs of age (whichever is
longer)
Patients with carditis
(mild MR or healed carditis)
For 10 yrs after the last attack or
at least until 25 yrs of age
(whichever is longer)
More severe valvular disease Lifelong
After valve surgery Lifelong

14. Antibiotics used in secondary prophylaxis of RF
Benzathine benzylpenicillin 1 200 000 units, IM, 3-4 weeks
600 000 units for children
Penicillin V 250 mg BD
Sulphonamide e.g sulphadoxine,
sulphadiazine
1 gm daily
500 mg daily for children
Erythromycin 250 mg BD

15. Surgical referrals or percutaneous valvotomies
 Chronic rheumatic valve disease
 Determined by the severity of patient’s symptoms
and significantly impaired cardiac function
 To prevent irreversible damage to the LV and
irreversible pulmonary hypertension
 Echo is essential for an assessment and follow up
of valvular disease

16. Referrals for further assessment
 > NYHA Class II. Note: with AS, all symptomatic patients
 Progressive LV enlargement on clinical or CXR
 Cardiac failure not due to episode of rheumatic carditis
 PHT with clinical signs and ECG evidence of RVH, and CXR evidence
of pulmonary artery dilatation
 TR complicates mitral valve disease
 Development of AF
 Thromboembolism
 Endocarditis is suspected to contribute to cardiac decompensation

17. Treatment Options
 Balloon valvotomy (commissurotomy)
 Surgical treatment
 Closed mitral commissurotomy
 Valve repair
 Valve replacement

18. Management of heart failure with
rheumatic valvular heart disease
 Investigations:
 Baseline blood tests : Haemoglobin,
electrolytes, creatinine, liver function test
 ECG
 CXR
 Assessment of rheumatic activity

19. Management of heart failure with
rheumatic valvular heart disease
 Life style modification: restriction of salt intake
 Avoidance of precipitating factors
 Diuretics: Frusemide, Spironolactone, Metolazone
 Heart rate control : digoxin, beta blocker
 Anticoagulation: warfarin
 Therapeutic INR  2 to 2.5
 Metallic valve : Singe valve  2 to 2.5
 Metallic valve : Double valve  2.5 to 3
 Prophylaxis : Penicillin

20.  ACEI/ ARB – recommended only for non
rheumatic valvular regurgitations
 Patients should be referred to tertiary centres for
assessment of valvuloplasty or valve replacement
Management of heart failure with
rheumatic valvular heart disease

21. MITRAL STENOSIS

22. Medical management
 Salt intake restriction and oral diuretics
 In AF: digoxin, B-blocker, or calcium-channel
blocker for rate control.
 Anticoagulation
 Endocarditis prophylaxis is no longer
recommended
Balloon valvotomy
Surgical treatment - Valvotomy, Mitral repair, MVR

23. MITRAL REGURGITATION

24. Medical management
 Asymptomati mild MR are managed conservatively with
serial echocardiograms.
 Vasodilators in symptomatic patients to increase
forward CO and reduce regurgitant volume.
 AF: rate control and anticoagulation
Surgical treatment
 Symptomatic severe MR on optimum medical
management.
 Asymptomatic patients with severe MR may need
surgery if
worsening of LV function (EF 30–60 % or LV end-systolic
dimension > 40 mm), new AF , pulmonary hypertension.

25. AORTIC STENOSIS

26. Medical management
 No medical treatments are proven to prevent or delay the
disease process in the AV leaflets.
 B -blockers reduce myocardial O 2 demand and may improve
coronary blood fl ow
 Cautious use of loop diuretics may relieve preload and help
with dyspnoea (avoid hypovolaemia)
 In CHF or dilated LV, digoxin may help with dyspnoea
(particularly if patient is in AF/fl utter)
 In severe AS, avoid negative inotropes and drugs that reduce
afterload (e.g. glyceryl trinitrate (GTN,) angiotensin-converting
enzyme inhibitors (ACE-Is)), as these may worsen the gradient
and cause syncope.

27. Surgical treatment
 Symptomatic AS.
 In asymptomatic severe AS, EF<50 %
 Aortic valve replacement (AVR) is reasonable for asymptomatic
moderate or severe AS when undergoing concomitant coronary
artery bypass graft (CABG), aortic, or valve surgery.
 Balloon aortic valvuloplasty
 Transcatheter aortic valve replacement

28. AORTIC REGURGITATION

29. Medical management
 Asymptomatic mild/moderate AR with normal LV —
routine follow-up (every 1–2 years) with ECHO.
 Asymptomatic severe AR with normal LV — frequent
(6-monthly) follow-up
 Severe AR with LV dysfunction or symptoms (and
patient not operative candidate) — symptoms of CCF
respond to loop diuretics and digoxin and vasodilators
(ACE-Is, calcium-channel blockers)
Surgical management
 AVR is indicated for patients with symptomatic severe
AR, or asymptomatic severe AR and EF < 50 % , severe
LV dilatation (LV end-diastolic dimension > 75 mm or
LV end-systolic dimension > 55 mm), or concomitant
CABG, valvular, or aortic surgery.

30. Tricuspid and pulmonary disease
Tricuspid regurgitation
Functional or secondary TR
Organic tricuspid lesions: endocarditis, transvalvular pacing
wires, Marfan’s syndrome, Ebstein anomaly, rheumatic heart
disease, carcinoid.
In the absence of pulmonary hypertension, TR is well tolerated
and may not require specifi c treatment
TV annuloplasty
Valve replacement
Tricuspid stenosis
Extremely rare
surgical valvuloplasty/ replacement

31. Pulmonic stenosis
Balloon valvuloplasty — treatment of choice for stenosis at
valvular level.
Surgical – valvulotomy, Pulmonic valve replacement
Pulmonary regurgitation
Supportive treatment

32. Prosthetic heart valves
 Mechanical: Bileaflet (St Jude Medical ® , Carbomedics
® ) most common today. Ball and cage (Starr–Edwards
® ) or tilting disc (Medtronic Hall ® )
 Bioprosthetic: Porcine (stented or stentless) or bovine
pericardium (Carpentier–Edwards)
 Non prosthetic valves: Homograft (preserved cadaveric
human valve), autograft (pulmonary valve — Ross
procedure)
Anticoagulation ( target INR )

33. Planning & implementation of national programs
for the prevention & control of RF & RHD
 A strong commitment at policy level ( Ministries of H&E)
 A national advisory committee: Cardiologists,
paediatricians, family physicians, internal medicine
specialists, epidemiologists and nurses
 Stepwise program implementation: one or more defined
local areas (phase I) to provincial (phase II) & national
coverage (phase III)
 Service orientated and emphasize active secondary
prevention, integrated into PHC
 Support from the microbiology lab at peripheral,
intermediate and national levels
 Suspected outbreaks of gp A strept: infection should be
controlled and studied

34. Main Components of A National Program
 Secondary prevention activities aimed at preventing the
recurrence of acute RF and severe RHD
 (case finding, referral, registration, surveillance, follow-up,
secondary prophylaxis)
 Primary prevention activities aimed at preventing the first
attack of acute RF
 (early detection, correct dx, appropriate tx of strept:
pharyngitis)
 Health education activities (P&S prevention, recognizing
and reporting sore throats)
 Training of health care providers
 Epidemiological surveillance
 Community involvement

35. THE END