This document provides an overview of acute rheumatic fever (ARF) and rheumatic heart disease (RHD). It discusses the epidemiology, pathogenesis, clinical manifestations, diagnosis, complications and management of ARF as well as the manifestations, diagnosis and management of RHD. ARF is an autoimmune response following group A streptococcal infection that can lead to RHD, which is permanent heart valve damage. Mitral valve involvement is most common in RHD. Management involves secondary prophylaxis to prevent recurrent ARF as well as treatment of valve lesions and heart failure.

1. Acute rheumatic fever
and RHD
MARC KAVAKURE
SUPERVISED BY: Dr Francois

2. Outlines
• Introduction
• Epidemiology
• Pathophysiology
• Clinical features and manifestations
• Investigations
• Differential diagnosis
• Complications
• Management plan

3. Overview on acute rheumatic fever
• The complications of group A streptococcal pharyngeal infection includes:
suppuratives ( peritonsillar abscess, sinusitis or otitis media) and non
suppurative inflammatory ( ARF, scarlet fever or acute glomerunephritis)
• ARF is a delayed autoimmune response to untreated group A beta hemolytic
streptococcal pharyngitis.
• The first signs or symptoms of ARF occurs after 2 to 4 weeks of latent
period following the initial GAS pharyngitis.

4. Epidemiology
• Rheumatic fever and RHD are diseases of poverty, commonly in developing
and being the leading cause of cardiovascular death in the first five decades.
• Environmental conditions, quality and availability of health care determine
number of affected child.
• In recent century, ARF was rare in developed countries.
• ARF can occur at any age but the most cases occur in children
between 5 and 15 years.

5. Pathogenesis
• Streptococcal pharyngeal infection absolute requirement in genetically
susceptible individual.
• Following GAS pharyngeal infection, activation of innate immune system
leads to GAS antigen presentation to B and T cells.
• IgG and IgM production followed by activation of CD4+T cells.
• In susceptible individuals, cross-reactive immune response thought to be
mediated by molecular mimicry involving both cellular and humoral
component of adaptive immunity.

6. Two manifestation of ARF

7. Clinical features
• This cross-reactive response leads to the clinical features of rheumatic fever (modified
JONES criteria)
• The five major manifestations:
• Carditis and valvulitis (eg, pancarditis) that is clinical or subclinical – 50 to 70 percent
• Arthritis (usually migratory polyarthritis predominantly involving the large joints) – 35 to 66
percent
• Central nervous system involvement (eg, Sydenham chorea) – 10 to 30 percent
• Subcutaneous nodules – 0 to 10 percent
• Erythema marginatum – <6 percent

8. Image of erythema marginatum

9. • The four minor manifestations are:
• Arthralgia
• Fever greater or equal to 38.5oc
• Elevated acute phase reactants (erythrocyte sedimentation rate [ESR] , C-
reactive protein [CRP])
• Prolonged PR interval on electrocardiogram
• Family hx, RHD, jaccoud arthropthy

10. Jaccoud arthropthy

11. Diagnosis
• Modified JONES criteria; for joint( arthritis or arthralgia), for cardiac( carditis or
prolonged PR interval)should be counted once.
• Two major or one major plus two minor sufficient when there is evidence of a
preceding GAS infection.
• Evidence of GAS infection helpfully but not extreme requirement:
• Positive throat culture for group A beta-hemolytic streptococci
• Positive rapid streptococcal antigen test
• Elevated or rising antistreptococcal antibody titer – Either antistreptolysin O (ASO)
or antideoxyribonuclease B (ADB)

12. Diagnosis
• Laboratory investigations : CRP, ESR, FBC with differential for evidence of
anemia or leukocytosis
• Cardiac evaluation: including ECG and echocardiogram

13. Differential diagnosis
• Septic arthritis
• Connective tissue and other
autoimmune disease
• Viral arthropathy
• Lyme disease
• Sickle cell anemia
• Mitral valve prolapse
• Infective endocarditis
• Viral pericarditis
• Drug intoxication
• Intracranial tumor

14. Management of ARF
• Symptomatic relief of acute disease manifestations (eg, arthritis)
• Eradication of group A beta-hemolytic Streptococcus (GAS)
• Prophylaxis against future GAS infection to prevent progression of cardiac
disease
• Provision of education for the patient and patient’s caregivers

15. Treatement of ARF
Long-term Management:
 Regular secondary prophylaxis
 Regular medical review
 Regular dental review
 Echocardiogram following each episode of ARF, and routine echocardiogram:
• every 2 years for children (sooner if there is evidence of cardiac symptoms)
• every 5 years for adults (sooner if there is evidence of cardiac symptoms

16. Management of ARF
 Benzathine penicillin G single injection, or
 Oral Penicillin V for 10 days (Erythromycin if penicillin allergy)
 Relief of symptoms and signs with NSAID (Aspirin…) or corticosteroids (prednisolone)
 Carbamazepine or Valproic acid can be given for severe cases of Sydenham chorea
 If Heart Failure:
• Bed rest
• Anti-failure medication (e.g. Diuretics, ACEi, Digoxin)
• Anti-coagulation medication if atrial fibrillation is present

17. Secondary prophyllaxis
• Benzathine Penicillin G IM every 3 to 4 weeks:
Standard dose:
• 1,200,000 units for patients ≥30kg
• 600,000 units for children <30kg
• Penicillin V can be used if Benzathine penicillin injections are not tolerated or if injections are contraindicated.
standard dose : 250mg oral, twice-daily.
• Erythromycin is given if there is a proven allergy to Penicillin.
standard dose : 250mg oral, twice-daily.

18. Duration of secondary prophyllaxis

19. RHEUMATIC HEART DISEASE
• RHD is defined as permanent heart valve damage subsequent to ARF.
• Most important form of acquired heart disease in children and young adults living
in developing countries
• It accounts approximately 15% of all patients with heart failure living in endemic
area.
• Poverty, unemployment, overcrowding, poor access to health care increase the
incidence of RHD.
• RHD was significantly decreased in industrialized countries.

20. Introduction
• Early diagnosis of RHD is very important so that secondary prophylaxis.
• Echocardiography is essential to confirm the diagnosis and to detect any
progression of valvular disease.
• Transition from rheumatic carditis to RHD with chronic valvular lesions
evolves over years following one or more episodes of ARF.

21. Clinical manifestations
• Rheumatic carditis; spectrum of lesions ranging from pericarditis,
myocarditis and valvulitis during episodes of ARF.
• Transition from rheumatic carditis to RHD with chronic valvular lesions that
evolve over years following one or more episodes of ARF.
• Valvulitis is generally a prominent manifestation of rheumatic carditis.
• The clinical correlate of valvulitis is pathologic valvular regurgitation, which
may be detected clinically (as regurgitant murmurs) or only on
echocardiography.

22. Manifestation
• Mitral regurgitation (MR) may be detected as an apical holosystolic murmur
and may or may not be accompanied by an apical mid-diastolic murmur
(Carey-Coombs murmur)
• Aortic regurgitation (AR; which may be detected as a basal early diastolic
murmur).
• Approximately 10 percent of patients with ARF develop severe acute
valvulitis with mitral and/or aortic insufficiency after the first episode of
ARF.

23. Manifestation
• Pericarditis occurs in approximately 15 percent of cases of ARF and may
present with precordial chest pain and a pericardial friction rub.
• Heart failure (HF) and left ventricular (LV) dilation in patients with ARF are
caused chiefly by severe valve disease (mainly MR with or without AR)

24. Manifestation
• Rheumatic carditis detected only by echocardiography (as valve disease with valvular
regurgitation) is termed subclinical carditis.
• MR is the most common early valvular manifestation and may be accompanied by
AR and/or uncommonly by tricuspid regurgitation (TR).
• Isolated AR is rare
• Mechanisms of MR in ARF are postulated to be the combination of annulitis with
annular dilatation, chorditis with chordal elongation, and valvulitis resulting in
typically anterior mitral leaflet prolapse and pathologic MR

25. Affected valves
• Mitral valve is affected in over 90% of cases of RHD.
• The next most commonly affected valve is the aortic valve; usually disease
of the aortic valve is associated with disease of the mitral valve.
• Tricuspid and pulmonary valves are rarely directly affected but tricuspid
regurgitation may occur in advanced mitral valve disease.

26. Affected valves
• Mitral regurgitation (MR): found most commonly in children and young
adults.
• Mitral stenosis (MS) represents longer term chronic changes to the mitral
valve, more commonly seen in adults. Older children can present both MS
and MR.
• Aortic regurgitation is not uncommon, aortic stenosis is almost never seen
as an isolated lesion

27. Symptoms
• The symptoms of RHD depend on the valve lesion and its severity, may not
show for many years until valve disease becomes severe.
• Initial symptoms of RHD are the symptoms of early heart failure:
Breathlessness on exertion, general weakness
Orthopnoea, paroxysmal nocturnal dyspnoea
 Signs of chronic malnutrition may occur as complication due to long term heart
condition.
Peripheral edema

28. Symptoms
• Specific symptoms:
Palpitations: in case of atrial fibrillation (AF), particularly with mitral
stenosis (associated with increased thromboembolic risk)
 Stroke: cerebral embolism in case of AF and/or severe mitral stenosis,
infective endocarditis of mitral or aortic valve
 People with aortic valve disease may experience angina and syncope in
addition to heart failure symptoms

29. Physical examination
• signs of heart failure: ( tachypnoea, tachycardia, oedema, raised jugular
venous pressure, hepatomegaly, lung crackles)
• Bulging and hyperactive precordium are common in case of severe and
chronic mitral regurgitation
• the presence of atrial fibrillation
• stroke

30. Physical examination
• Auscultation:
Mitral regurgitation: pansystolic murmur heard loudest at the apex and radiating
laterally to the axilla.
Mitral stenosis: low-pitched, diastolic rumble heard best at the apex
Aortic regurgitation: diastolic blowing decrescendo murmur best heard at the
left sternal border with the person sitting up
Aortic stenosis: murmur is a loud, low pitched mid-systolic ejection murmur
best heard in the aortic area, radiating to the neck.

31. Investigation
• Electrocardiography: essential to determine the cardiac rhythm and other
abnormal patterns
• Chest X-ray : helps to assess the size of the heart chambers and to detect
pulmonary congestion
• Echocardiography: to detect any rheumatic valve damage, help determine
its severity and assess left ventricular function
• Additional investigations include : FBC, Electrolytes, renal and liver
function tests

32. Complications
• Depending upon the affected valve:
Heart Failure, severe pulmonary hypertension
 Infective endocarditis
Atrial fibrillation
Stroke
Chronic malnutrition
Ventricular dysfunction

33. Differential diagnosis
• Infective endocarditis
• Congenital valvular heart disease
• SLE

34. Management of RHD
• GOALS:
Prevent disease progression and to avoid, or at least delay, valve surgery
Secondary prophylaxis for prevention of recurrent ARF is the main strategy.
Management of complications
Infective endocarditis prophyllaxis before dental or any surgical procedure
Regular dental care
Contraception in female adolescent

35. Management of congestive heart failure
• Due to severe mitral and/or aortic regurgitation: bed rest, diuretics and
ACEIs
• Due to severe mitral stenosis:
Diuretics, beta-blocker
Aspirin to prevent intra-atrial thrombus and stroke
Warfarin in case of atrial fibrillation
• Nutrition support often required

36. Valve surgery
• Indication for heart surgery is determined by:
• severity of symptoms,
• evidence that the heart valves are significantly damaged,
• LV chamber size and function.
• Types include: valve repair or replacement by bioprosthetic /mechanical
valve

37. Valve surgery
• Factors to absolute contra-indications to valve surgery:
• Poor LV function with valve regurgitation,
• severe pulmonary hypertension may pose an unacceptable risk for cardiac surgery.
• Good nutritional status improves post-operative outcomes.

38. Post-operative period: anticoagulation
 Indicated for patient who undergo valvular replacement
 Commonly used drug is coumadin ( warfarin), needs to be monitored following
replacement with mechanical valves.
 Good anticoagulation management requires standardized anticoagulation measurement,
using the International Normalised Ratio (INR)

39. Long-term postoperative management
 Heart valve surgery requires regular long-term follow-up,
 Conservative valve procedures (valve repair), require close observation to detect re-stenosis
or a recurrence of valve regurgitation,
 Ensure secondary prophylaxis is administered regularly ( Benzanthine Penicillin G IM every
3-4 weeks) to prevent recurrent attacks by ARF.
 It is also important to monitor LV and prosthetic function

40. Anti-Coagulation Goals

41. References
• Uptodate
• Amboss
• Lecture note of cardiology