Acute Rheumatic Fever and Rheumatic Heart Disease, are two common conditions in children between 3-15 years of age following a Group B Streptococcal throat infection. We discuss these two conditions in the slides above, as well as their management.

1. Acute Rheumatic Fever and
Rheumatic Heart Disease
By Godwin Ivan Candia & Ndibarema Muhimbura

2. Acute Rheumatic Fever
Definition:
• Rheumatic fever is a non-suppurative, acute inflammatory complication of group
A streptococcal (GAS) pharyngeal infection, causing combinations of arthritis,
carditis, subcutaneous nodules, erythema marginatum and chorea.
• a pharyngeal streptococcal infection usually precedes acute rheumatic fever by
2-6 weeks.

3. Epidemiology
• The first attack usually occurs between ages of 3-15 years (peak years for pharyngeal
streptococcal pharyngitis)
• 470,000 new cases of acute rheumatic fever each year worldwide.
• 282,000 new cases of rheumatic heart disease each year globally.
• In patients with prior ARF, rate of recurrence in untreated GAS pharyngitis is 50%.
• ARF in Uganda has prevalence of between 1.5% - 3.5% in children aged between 5-15
years.
• RHD accounts for at least 345000 deaths worldwide, with a female: male ratio of 2:3

4. Etiology
• Group A streptococcal (GAS) pharyngitis is the etiologic precursor of ARF.
• GAS M Proteins share epitopes (antigenic-determinant sites that are
recognized by antibodies) with proteins found in synovium, heart muscle,
and heart valves.
• This molecular mimicry by GAS antigens from rheumatogenic strains
contributes to the arthritis, carditis, and valvular damage.
• This occurs by a hypersensitivity reaction when the anti-streptococcal
antibodies then attack and destroy the normal tissue that contains these
mimicked antigens.

5. Risk Factors
• Previous group A streptococcal pharyngitis
• A family history of rheumatic fever
• Genetic (D8/ 17 B-Cell antigen and certain class II histocompatibility
antigens)
• Undernutrition
• Overcrowding
• Lower socioeconomic status

6. Pathogenesis
• The joints, heart, skin and central nervous system (CNS) are most often
affected. Pathology varies by site.
Joints
• It causes non-specific synovial inflammation which sometimes contains
Aschoff- like bodies.
• The abnormalities of the joint are not chronic and do not leave scarring or
residual abnormalities. (‘’ARF licks the joints but bites the heart’’)
• It affects the large joints, is asymmetrical and usually manifests as a
polyarthritis.

7. Cont.
Heart
• Manifests as carditis, typically affecting the heart valves, endocardium, then
myocardium and pericardium finally.
• Aschoff bodies often develop in the myocardium.
• Potentially dangerous valve changes may occur.
• Acute interstitial valvulitis may cause valvular edema.
• Major morphological changes of the valves include; commissural fusion,
shortening and fusion of the chordae tendinae and leaflet thickening.
• The most common cardiac manifestations are mitral regurgitation,
pericarditis and sometimes, aortic regurgitation.

8. Cont.
Skin
• It causes subcutaneous nodules on the skin and erythema
marginatum.
CNS
• Causes Sydenham chorea which manifests as hyper perfusion and
increased metabolism in the basal ganglia.
• Increased levels of antineuronal antibodies have also been shown.

9. Cont.

10. Clinical Features
• Arthritis (migrating asymmetric polyarthritis)
• Acute rheumatic carditis, signs of cardiac failure, murmurs and
pericarditis
• Subcutaneous nodules
• Chorea (involuntary movements of limbs)
• Skin rash (erythema marginatum)
• Other minor signs/symptoms: fever, arthralgia

11. Diagnostic criteria (revised Jones criteria)
• Acute Rheumatic Fever is diagnosed using the clinical and laboratory
findings of the revised Jones criteria.
• A history of recent group B streptococcal infection is obtained and is
confirmed by either increased or rising antistreptolysin O titre or other
anti-streptococcal antibodies or a positive throat swab for group A beta-
hemolyticus streptococcus.
• The presence of either two major criteria or one major and two minor
criteria, along with evidence of an antecedent streptococcal infection,
confirm a diagnosis of acute rheumatic fever.

12. Cont.

13. Differential Diagnosis
• Any form of arthralgia/arthritis
• Pyrexia with cardiac failure
• Arthritis: Sickle cell disease, Hemophilia, Still’s disease, Septic arthritis
• Rash: Henoch- Schönlein purpura (HSP), Kawasaki disease, Lyme disease,
Systemic lupus erythematosus, juvenile idiopathic arthritis
• Carditis: Cardiac disease, Cardiomyopathy, Infective endocarditis
• Chorea: Wilson’s disease, Adverse drug reactions, Huntington’s disease
(very rare in children)
• Malaria

14. Investigations
• CBC (raised ESR and CRP)
• Chest X-ray
• ECG
• Echocardiography
• Antistreptolysin O titre (ASOT)
• Throat swab(culture)
• Rapid streptococcal antigen test

15. Treatment of ARF
• Bed rest
• To eradicate streptococci, phenoxymethylpenicillin 125mg per dose every 6
hours for 10 days for a child.
• Or Benzathine benzylpenicillin dose 1.2 MU IM stat
Child < 30 kg: 0.6 MU
Child > 30 kg: 1.2 MU
• To treat the inflammation, acetylsalicylic acid 80-100mg/kg/day in 3 doses.
Add magnesium trisilicate compound 2-4 tablets every 8 hours and must be
taken 30 minutes after the acetylsalicylic acid tablet. If allergic to aspirin,
give a low dose steroid.
• If chorea, valproate 10-20mg/kg/day

16. Prophylaxis
To prevent further episodes
• Pen V 500 mg 12 hourly
Child: 125-250 mg 12 hourly
• Or Benzathine benzylpenicillin 1.2 MU IM every 4 weeks
Child <30 kg: 0.6 MU
• If allergic to penicillin:
Erythromycin 250 mg 12 hourly
Child: 10 mg/kg twice a day
Duration of prophylaxis depends on severity of disease:
Rheumatic fever without carditis: for 5 years or until age 18 or 21 years old
Carditis but no residual heart disease: for 10 years or until age 25 years old
Carditis with residual heart disease: until age 40-45 years or for life

17. Prevention
• Early diagnosis and treatment of group A Streptococcus throat
infection
• Avoid overcrowding, good housing
• Good nutrition

18. Rheumatic Heart Disease
Pathogenesis
• Valve inflammation leads to further binding of cross reactive
antibodies to the valve, leading to endocarditis.
• Lack of production of regulatory cytokines contributes to permanent
valve damage.
• Thus RHD can occur due to fibrotic healing of acute inflammatory
lesions and turbulent flow induced by ongoing valve damage.
• Major morphological changes of the valves include; commissural
fusion, shortening and fusion of the chordae tendinae and leaflet
thickening.

19. Clinical Features
• Heart failure
• Heart murmurs depending on valves affected and nature
of effect caused. The murmurs are usually different
depending on the valves affected. A tricuspid stenosis
mumur is usually scratchy in character, short in duration,
and usually augmented by inspiration. A mitral stenosis
murmur is usually rumbling and low pitched in character,
long in duration and usually augmented by exercise.

20. Cont'd
• The murmur of tricuspid regurgitation is frequently not heard. When
evident, it is a holosystolic murmur heard best at the left middle or
lower sternal border or at the epigastrium.The murmur may be high-
pitched if TR is trivial and due to pulmonary hypertension, or it may
be medium-pitched if TR is severe

21. Cont'd
• Arrhythmias, palpitations
• Thromboembolic problems e.g. stroke
• The patient may be asymptomatic and the valvular lesion discovered
as an incidental finding
• Increased cardiac demand as in pregnancy and anemia may present
as congestive cardiac failure

22. Investigations
• Echocardiogram
• Electrocardiogram(ECG)
• Chest X-ray
• Cardiac MRI
• Blood tests to look for infection and inflammation.

23. Treatment of RHD
• Treat heart failure if present
• Prophylaxis for life as in rheumatic fever
• Cardiac surgery if necessary e.g. commissurotomy (only at national
referral hospital.)

24. Diagnosis & treatment of:
Common valve lesions like
1. Mitral regurgitation
2. Aortic valve regurgitation
3. Mitral stenosis
4. Tricuspid valve stenosis

25. Mitral regurgitation
• Echocardiogram. This test is commonly used to diagnose
mitral valve regurgitation. In this test, sound waves directed at
your heart from a wandlike device (transducer) held on your
chest produce video images of your heart in motion.
• This test assesses the structure of your heart, the mitral valve
and the blood flow through your heart. An echocardiogram
helps your doctor get a close look at the

26. Cont'd
• Doctors may conduct another type of echocardiogram called a
transesophageal echocardiogram. In this test, a small
transducer attached to the end of a tube is inserted down your
esophagus, which allows a closer look at the mitral valve than a
regular echocardiogram does.
• Electrocardiogram (ECG). Wires (electrodes) attached to
adhesive pads on your skin measure electrical impulses from
your heart. An ECG can detect enlarged chambers of your
heart, heart disease and abnormal heart rhythms.

27. Cont.
• Chest X-ray. This enables your doctor to determine whether the
left atrium or the left ventricle is enlarged — possible indicators
of mitral valve regurgitation — and the condition of your lungs.
• Cardiac MRI. A cardiac MRI uses magnetic fields and radio
waves to create detailed images of your heart. This test may be
used to determine the severity of your condition and assess the
size and function of your lower left heart chamber (left
ventricle).

28. Cont.
• Cardiac CT. A CT angiogram may be performed of the chest,
abdomen and pelvis to determine whether you're a candidate
for robotic mitral valve repair.
• Exercise tests or stress tests. Different exercise tests help
measure your activity tolerance and monitor your heart's
response to physical exertion. If you are unable to exercise,
medications to mimic the effect of exercise on your heart may
be used.

29. • Cardiac catheterization. This test isn't often used to diagnose
mitral valve regurgitation. This invasive technique involves
threading a thin tube (catheter) through a blood vessel in your
arm or groin to an artery in your heart and injecting dye through
the catheter to make the artery visible on an X-ray.

30. Aortic valve regurgitation
• Diagnosis
• Echocardiogram. Sound waves directed at your heart from a
wandlike device (transducer) held on your chest produces video
images of your heart in motion. This test can help doctors
closely look at the condition of the aortic valve and the aorta. It
can help doctors determine the cause and severity of your
condition, and see if you have additional heart valve conditions.
Doctors may also use a 3-D echocardiogram.

31. Cont.
• Doctors may conduct another type of echocardiogram called a
transesophageal echocardiogram to get a closer look at the
aortic valve. In this test, a small transducer attached to the end
of a tube is inserted down the tube leading from your mouth to
your stomach (esophagus).

32. Cont.
• Electrocardiogram (ECG). In this test, wires (electrodes)
attached to pads on your skin measure the electrical activity of
your heart. An ECG can detect enlarged chambers of your
heart, heart disease and abnormal heart rhythms.
• Chest X-ray. This enables your doctor to determine whether
your heart is enlarged — a possible indicator of aortic valve
regurgitation — or whether you have an enlarged aorta. It can
also help doctors determine the condition of your lungs.

33. Cont.
• Exercise tests or stress tests. Exercise tests help doctors see
whether you have signs and symptoms of aortic valve disease
during physical activity, and these tests can help determine the
severity of your condition. If you are unable to exercise,
medications that have similar effects as exercise on your heart
may be used.
• Cardiac MRI. Using a magnetic field and radio waves, this test
produces detailed pictures of your heart, including the aorta and
aortic valve. This test may be used to determine the severity of
your condition.

34. Cont
• Cardiac catheterization. This test isn't often used to diagnose
aortic valve regurgitation, but it may be used if other tests aren't
able to diagnose the condition or determine its severity. Doctors
may also conduct cardiac catheterization prior to valve
replacement surgery to see if there are obstructions in the
coronary arteries, so they can be fixed at the time of the valve
surgery.

35. Mitral Stenosis

36. Tricuspid regurgitation
• Diagnosis
• Echocardiogram
• Mild tricuspid regurgitation is most often detected on
echocardiography done for other reasons.
• More moderate or severe TR may be suggested by history and
physical examination. Confirmation is by echocardiography.
• Severe TR is characterized echocardiographically by ≥ 1 of the
following:

37. • 2-Dimensional failure of coaptation or flail
• Large regurgitant jet on color Doppler
• Large flow convergence zone proximal to the valve
• Vena contracta width > 7 mm
• Systolic flow reversal in the hepatic veins (specific for severe TR)
• Transtricuspid E wave dominant > 1 cm/second
• Dense, triangular, early peaking, continuous wave Doppler of TR jet

38. • In secondary TR, a dilated annulus > 40 mm (measured in the apical
4-chamber view) predicts severe TR. When TR is moderate or severe,
the peak regurgitant velocity will underestimate pulmonary pressure.
Two-dimensional echocardiography detects the structural
abnormalities present in primary TR.

39. Cardiac MRI
• Cardiac MRI is now the preferred method for evaluating RV size and
function, which typically should be done when echocardiographic
image quality is inadequate.
• An ECG and chest x-ray are often done.

40. • An ECG is usually normal but, in advanced cases, may show tall
peaked P waves caused by right atrial enlargement, a tall R or QR
wave in V1 characteristic of RV hypertrophy, or AF
• A chest x-ray is normal but, in advanced cases with RV hypertrophy
or RV dysfunction–induced HF, may show an enlarged superior vena
cava, an enlarged right atrial or RV silhouette (behind the upper
sternum in the lateral projection), or pleural effusion.
• Laboratory testing is not needed but if done may show hepatic
dysfunction in patients with severe TR.

41. • Cardiac catheterization is indicated for accurate measurement of
pulmonary pressure when TR is severe and to evaluate coronary
anatomy when surgery is planned. Catheterization findings include a
prominent right atrial c-v pressure wave during ventricular systole.

42. Infective endocarditis
• Diagnosis
•Blood cultures
• Because symptoms and signs are nonspecific, vary greatly, and may
develop insidiously, diagnosis requires a high index of suspicion.
Endocarditis should be suspected in patients with fever and no
obvious source of infection, particularly if a heart murmur is present.

43. Cont
• Suspicion of endocarditis should be very high if blood cultures are
positive in patients who have a history of a heart valve disorder, who
have had certain recent invasive procedures, or who abuse IV drugs.
Patients with documented bacteremia should be examined
thoroughly and repeatedly for new valvular murmurs and signs of
emboli.

44. Echocardiography
• Echocardiography, typically transthoracic (TTE) rather than
transesophageal (TEE), should be done initially. TEE is more sensitive
(ie, capable of revealing vegetations too small to be seen on TTE).

45. Cont.
• Transesophageal echocardiography should be done when
• Patients have a prosthetic valve
• Transthoracic echocardiogram is nondiagnostic
• Diagnosis of infective endocarditis has been established clinically
(done to detect perforations, abscesses, and fistulas)

46. CT
• CT is used occasionally when TEE fails to fully define paravalvular
abscesses and for detection of mycotic aneurysms. Positron emission
tomography (PET) scanning is an emerging tool for the diagnosis of
endocarditis originating in prosthetic and intracardiac devices. CT and
PET abnormalities are now included as major criteria in the European
guidelines.

47. PREVENTION