Rheumatic fever and Rheumatic heart diseases in Children-A.S.,AR, MS, MR, TS, TR, PS, PR

2. Lecture- 12
Rheumatic Fever and Rheumatic Heart
Diseases in Children
Prof. Dr. Sunil Natha Mhaske
Dean
Dr. Vithalrao Vikhe Patil Foundation’s Medical College and Hospital,
Ahmednagar (M.S.) India-414111
Mo- 7588024773
Mail-sunilmhaske1970@gmail.com

3.  Rheumatic fever was described for the first time in 1898 in
London by William Cheadle.
 In 1904, Aschoff described typical histopathological lesions in
rheumatic heart disease.
 The first RF diagnostic criteria were developed in 1944 by
Jones, then they were modified by AHA in 1992.
 RF is an autoimmune disease associated with group A β-
hemolytic streptococcal infection.
 It usually appears in children between the ages of 5 and 15.

4.  The patients develop carditis (50–78%), arthritis (35–88%), chorea
(2–19%), erythema marginatum (< 6%) and subcutaneous nodules
(< 1–13%)
 Acquired heart defects can be a consequence of RF.
 approximately 60% of RF patients in endemic countries develop
chronic rheumatic heart disease, which is a complication of RF
 The risk of chronic rheumatic heart disease is 1.6–2 times higher in
female patients.
 The course of the disease is characterized by relapses, where after
the first episode more may follow, thus increasing the risk of heart
defects. The risk is the higher the younger the patient is.
 Rheumatic fever symptoms occur 2–3 weeks after streptococcal
pharyngitis, never after streptococcal dermatitis.

5. Thomas Duckett Jones (d. 1954) :
• He was the director of research in
rheumatic fever and rheumatic heart
disease at the House of the Good
Samaritan in Boston for 20 years.
• He worked clinically at Massachusetts
General Hospital and was on faculty
at Harvard Medical School.
• Dr. Jones was appointed to the
inaugural National Advisory Heart
Council and also served as vice-
president of the American Heart
Association, chairman of the AHA's
Council on Rheumatic Fever, and
president-elect of the National Health
Council.

6. • Polyarthritis (a temporary migrating inflammation of the large
joints, usually starting in the legs and migrating upwards) is
considered as a major criterion in low-risk populations, whereas
monoarthritis, polyarthritis and polyarthralgia are all included as
major criteria in high-risk populations.
• Carditis: can involve the pericardium, myocardium, endocardium
in the form of valvulitis.
• Sydenham's chorea (St. Vitus' dance): A characteristic series of
involuntary rapid movements of the face and arms. This can occur
very late in the disease for at least three months from onset of
infection. Milk maid grip, spooning and pronation of extended
hands, wormian movements of tongue etc.

7. • Subcutaneous nodules: Painless, firm collections of collagen
fibers over bones or tendons. They commonly appear on the back
of the wrist, the outside elbow, and the front of the knees.
• Erythema marginatum: A long-lasting reddish rash that begins
on the trunk or arms as macules, which spread outward and clear
in the middle to form rings, which continue to spread and coalesce
with other rings, ultimately taking on a snake-like appearance.
This rash typically spares the face and is made worse with heat.

8. Diagnostic criteria– modified 1992 Jones criteria
Major criteria Minor criteria
Carditis
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Hyperpyrexia
Arthralgia, without other signs
of inflammation
Laboratory indicators of acute
phase:
ESR, CRP
Prolonged PR interval in ECG
And evidence of antecedent streptococcal infection
– Throat swab culture or rapid antigen test
– Elevated/increasing anti-streptococcal antibody titer in serum

9. • Major criteria -main clinical presentation of the disease.
• Minor criteria - clinical presentation and laboratory tests.
• The diagnosis of a first RF episode -2 major criteria or 1 major and
2 minor criteria, along with evidence of antecedent group A β-
hemolytic streptococcal infection.
• Rheumatic fever can also be diagnosed if the Jones criteria are not
met, in the case of isolated chorea or carditis with an insidious
onset, long-term course and inconspicuous progression of lesions,
after other causes have been excluded.
• In the revised 2015 Jones criteria , a low, medium and high-risk
population was identified. A low risk population is one in which
cases of acute RF occur in ≤ 2/100 000 school-age children or
rheumatic heart disease is diagnosed in ≤ 1/1000 patients at any
age during one year .

10. Diagnostic criteria– modified 2015 Jones criteria
Major criteria
Low risk population High risk population
Carditis (clinical or subclinical)
Arthritis – only polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Carditis (clinical or subclinical)
Arthritis – monoarthritis or polyarthritis
Polyarthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
Minor criteria
Low risk population High 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)
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)

11. Echocardiographiy is main diagnostic tool developed by AHA in 2012 -
I. Echocardiographic (Doppler) criteria:
 Pathological mitral regurgitation – 4 criteria (all must be met):
1.Visible at least in 2 projections.
2.Regurgitation jet length ≥ 2 cm at least in 1 projection.
3.Regurgitation peak velocity > 3 m/s.
4.Regurgitation pansystolic.
 Pathological aortic regurgitation – 4 criteria (all must be met):
1.Visible at least in 2 projections.
2.Regurgitation jet length ≥ 1 cm at least in 1 projection.
3.Regurgitation peak velocity > 3 m/s.
4.Regurgitation pandiastolic.

12. II. Echocardiographic (morphological) criteria:
 In acute mitral valve involvement:
1.Dilatation of mitral annulus.
2.Elongation of chordae tendineae.
3.Rupture of chorda tendinea with acute mitral regurgitation.
4.Prolapse of anterior (less often posterior) leaflet.
5.Nodular lesions on leaflets.

13.  In chronic mitral valve involvement (invisible in acute
involvement):
1.Thickening of leaflets.
2.Thickening of chordae tendinea, with their fusion.
3.Limited mobility of leaflets.
4.Calcifications.
 Lesions in acute and chronic aortic valve involvement:
1.Symmetrical or focal thickening of leaflets.
2.Disturbed leaflet coaptation (leaflet closing during systole).
3.Limited mobility of leaflets.
4.Prolapse of leaflets.

14. Diagnosis
• Blood tests
• C-reactive protein
• erythrocyte sedimentation rate.
• Electrocardiogram
• Echocardiogram
• Throat culture.

15. Treatment
Rest- Absolute bed rest if carditis present

16. Anti-streptococcal treatment-I. Primary prevention- treatment of streptococcal
pharyngitis-
 Phenoxymethylpenicillin orally , weight > 40 kg – 2–3 MIU/day in 2 divided doses
every 12 hours for 10 days, children with a body weight < 40 kg – 100,000 to
200,000 IU/kg/day in 2 divided doses every 12 hours for 10 days.
 Benzylpenicillin, intramuscularly at a single dose, weight > 40 kg – 1.2 MIU,
children with a body weight < 40 kg – 600,000 IU.
 Cefadroxil: weight > 40 kg – 1 g, children with a body weight < 40 kg – 30 mg/kg,
in a single dose for 10 days.
 Cefalexin: children 25–50 mg/kg/day in 2 doses for 10 days.
 Erythromycin: weight > 40 kg – 0.2–0.4 γ every 6–8 hours, weight < 40 kg – 30–50
mg/kg/day in 3–4 doses, for 10 days.
 Clarithromycin: weight > 40 kg – 250–500 mg every 12 hours, weight < 40 kg – 15
mg/kg/day in 2 doses, for 10 days.
 Azithromycin: weight > 40 kg – 500 mg on the first day, then 250 mg for three
consecutive days, weight < 40 kg – a single daily dose of 12 mg/kg/day for 5 days

17. II. Secondary prevention - prevention of subsequent rheumatic fever
relapses –
 Secondary prevention should be administered from 5 to 10 years
from the last RF relapse, or up to 21 years of age (whichever is
longer)
 In RF cases with carditis leading to chronic valvular heart disease,
the prevention should be administered for 10 years or until 40
years of age (whichever is longer)
 Secondary prevention makes use of benzathine benzylpenicillin,
intramuscularly: weight > 20 kg – 1.2 MIU, weight < 20 kg –
600,000 IU every 4 weeks
 Phenoxymethylpenicillin is administered orally at a dose of 2 ×
250 mg (i.e. 2 × 400,000 IU).

18. Anti-inflammatory treatments-
Heart involvement, glucocorticosteroids (GCs) are used prednisone
at a dose of 1–2 mg/kg/day for 2–3 weeks, then the dose should be
reduced gradually.
 The total duration of GCs treatment is 6 weeks.
 During the period of prednisone dose reduction, acetylsalicylic
acid should be initiated – at 60 mg/kg/day.
Carditis with out- Cardiomegaly & or CCF-Aspirin 100mg/Kg/24hr
qid 4 day, 75 mg/24hrs/ qid 4 week

19. • Supportive therapy- when required Digoxin, Diuretics, Oxygen,
Fluid & salt restriction.
• Chronic heart lesions- Prophylaxis against bacterial endocarditis
during surgical procedures.
• Sydenham’s chorea –
- Phenobarbitol 15-30 mg tds or qid oral is the drug of choice
- Haloperidol 0.01- 0.03mg/kg/24hrs bd oral
- Chlorpromazine 0.5mg/kg every 4-6 hrs oral

20. Complications -
- Rheumatic heart disease.
- Aortic valve stenosis.
- Aortic regurgitation.
- Atrial fibrillation.
- Heart failure.
- stroke

21. Rheumatic Heart Diseases in Children

22.  Commonest acquired heart disease.
 Rheumatic heart disease is the most serious complication
of rheumatic fever.
 Acute rheumatic fever and rheumatic heart disease are thought to
result from an autoimmune response, but the exact pathogenesis
remains unclear.
 Rheumatic heart disease is the result of permanent heart valve
damage secondary to acute rheumatic fever and the resultant
rheumatic carditis involving pericarditis, myocarditis, or valvulitis.
 With chronic rheumatic heart disease, patients develop mitral valve
stenosis with varying degrees of regurgitation, atrial dilatation,
arrhythmias, and ventricular dysfunction.
 Although the mitral valve is involved in most cases of rheumatic
heart disease, the aortic and tricuspid valves can be involved as
well.
 MV  AV  TV  PV

23. Rheumatic Mitral stenosis
• One of the grave consequences of rheumatic heart disease.
• Mitral valve involvement is seen mainly as mitral regurgitation and
less commonly mitral stenosis.
• A narrowing of the heart's mitral valve.
• This abnormal valve doesn't open properly, blocking blood flow
into the left ventricle
• The main cause of mitral valve stenosis is an infection called
rheumatic fever.
• Rarely congenital
• 40% of all patients with rheumatic heart disease
• Two-thirds of all patients with MS are female.

24. Right Heart Failure:
Hepatic Congestion
JVD
Tricuspid Regurgitation
RA Enlargement
 Pulmonary HTN
Pulmonary Congestion
LA Enlargement
Atrial Fib
LA Thrombi
 LA Pressure
RV Pressure Overload
RVH
RV Failure LV Filling
Normal valve area: 4-6 cm2
Mild mitral stenosis:
MVA 1.5-2.5 cm2
Minimal symptoms
Mod mitral stenosis
MVA 1.0-1.5 cm2 usually does not produce symptoms at rest
Severe mitral stenosis
MVA < 1.0 cm2

25. Signs and Symptoms -
 Fatigue
 Shortness of breath with exertion or when lying flat
 Shortness of breath and coughing during the night
 Swollen ankles and feet
 Palpitations
 Heavy coughing which may produce blood-stained mucus
• Palpation:
- Small volume pulse
- Tapping apex-palpable S1
+/- palpable opening snap (OS)
- RV lift
- Palpable S2
• Auscultation:
- Loud S1
- Diastolic rumble: length proportional to severity

26. • First heart sound (S1) is accentuated and snapping
• Opening snap (OS) after aortic valve closure
• Low pitch diastolic rumble at the apex
• Pre-systolic accentuation (esp. if in sinus rhythm)
• ECG: LAE, RVH, RAD
• Cardiac murmur,
• Echocardiogram,
• Chest X-ray,
• Transesophageal echocardiogram
• Cardiac catheterization

27. Complications –
• Atrial dysrhythmias
• Systemic embolization (10-25%)
• Congestive heart failure
• Pulmonary infarcts
• Hemoptysis
• Endocarditis
• Pulmonary infections
Treatment -
• Medical
Diuretics for LHF/RHF
Digitalis/Beta blockers/CCB: Rate control in A Fib
Anticoagulation
Endocarditis prophylaxis
• Balloon valvuloplasty
• Mitral valve repair or replacement surgery.

28. Rheumatic Mitral Regurgitation
• Mitral valve doesn't close tightly.
• Allowing blood to flow backward in
heart.
• Often mild and progresses slowly.
• Chronic LV volume overload 
compensatory LVE initially
maintaining cardiac output
• Decompensation (increased LV wall
tension)  CHF
• LVE  annulus dilation  increased
MR
• Backflow  LAE, A. fib, Pulmonary
HTN

29. • Similar to MS
• Dyspnea
• Orthopnea
• PND
• Fatigue
• Pulmonary HTN
• right sided failure
• Hemoptysis
• Systemic embolization in A Fib
• Pulse: brisk, low volume
• Apex:
• hyperdynamic
• laterally displaced
• palpable S3 +/- thrill
• late parasternal lift
• S 1 soft or normal
• S 2 wide split (early A2)
• Murmur-Fixed MR:
• pan systolic
• loudest apex to axilla
• no post extra-systolic
accentuation
• Murmur-Dynamic
MR(MVP)
• mid systolic
• S 3 / flow rumble if severe

30. Complications-
 Heart failure.
 Atrial fibrillation.
 Pulmonary hypertension.
ECG:
LA enlargement
LVH
RVH (15%)
Combined hypertrophy (5%)
CXR:
 LV
 LA
 pulmonary vascularity
CHF
Only effective
treatment is valve
repair/replacement

31. Mitral Valve Prolapse
•Common in girls and thin children
•May be inherited as autosomal dominant
•Associated with Marfan’s syndrome, Pectus excavatum
•May not be recognized till adolescent
•Symptoms are chest pain or palpitation
•On auscultation, systolic ejection click may be present
•Arrhythmias may occur
•Echocardiography is diagnostic

32. Aortic valve stenosis
 Aortic stenosis is narrowing of the aortic valve, impeding delivery
of blood from the heart to the body.
 Aortic stenosis can be caused by congenital bicuspid aortic valve,
scarred aortic valve of rheumatic fever, and wearing of aortic valve
in the elderly.
 prevents the valve from opening fully, which reduces or blocks
blood flow from your heart into the main artery to aorta and to the
rest of body.
Normal aortic valve area 2.5-3.5 cm2
Mild stenosis 1.5-2.5 cm2
Moderate stenosis 1.0-1.5 cm2
Severe stenosis < 1.0 cm2

33. Signs and symptoms -
 Abnormal heart sound
 heart murmur
 Chest pain
 angina
 dizzy or fainting
 Shortness of breath.
 Fatigue
 palpitations
 Not gaining weight
Complications-
 Heart failure
 Stroke
 Blood clots
 Bleeding
 arrhythmias
 endocarditis
 Death

34. Diagnosis-
• low volume pulse (pulsus parvus et tardus)
• delay between the first heart sound and the corresponding pulse in the carotid artery ( apical-
carotid delay).
• delay between the appearance of each pulse in the brachial artery and the radial artery
• The first heart sound may be followed by ejection click best heard at the lower left sternal
border and the apex.
• systolic, crescendo-decrescendo murmur is heard loudest at the upper right sternal border, at
the 2nd right intercostal space and radiates to the carotid arteries bilaterally.
• second heart sound tends to become decreased and softer as the aortic stenosis becomes more
severe.
• fourth heart sound due to the stiff ventricle.
• sustained, heaving apex beat
 precordial thrill
 narrowed pulse pressure
• Electrocardiogram- left ventricular hypertrophy
• Chest X-ray- enlarged left ventricle and atrium.

35. Treatment-
• Aortic valve repair
• Aortic valve replacement
• Transcatheter aortic valve replacement
• Balloon valvuloplasty
History-
Aortic stenosis was first described by French physician Lazare Rivière
in 1663

36. Aortic Regurgitation
• Leaking of the aortic valve of the heart that causes blood to flow
in the reverse direction during ventricular diastole, from
the aorta into the left ventricle.
Signs and symptoms-
 Dyspnea on exertion
 Orthopnea
 Paroxysmal nocturnal dyspnea
 Palpitations
 Angina pectoris
 Cyanosis (in acute cases)

37. Physical examination-
• S3 heart sound
• S3 gallop
• early diastolic and decrescendo- third left intercostal space and
radiate along the left sternal border.
• increased stroke volume of the left ventricle due to volume
overload, an ejection systolic 'flow' murmur may also be present
when auscultating the same aortic area.
• Austin Flint murmur- soft mid-diastolic rumble heard at the apical
area; it appears when a regurgitant jet of blood from severe aortic
insufficiency partially closes the anterior mitral leaflet.
• Widened pulse pressure : Systolic – diastolic = pulse pressure
• Aortic diastolic murmur (Severe)
• Apex: Enlarged, Displaced, Hyper-dynamic, Palpable S3 (Severe)

38.  Landolfi's sign
 Becker's sign
 Mayne's sign
 Rosenbach's sign
 Gerhardt's sign
 Lincoln sign
 Sherman sign
• Quincke’s sign: capillary pulsation
• Corrigan’s sign: water hammer pulse
(holding the middle of forearm or leg
and elevating it discloses a sharply
rising and abruptly falling pulse)
• De Musset’s sign: systolic head
bobbing
• Mueller’s sign: systolic pulsation of
uvula
• Durosier’s sign: femoral retrograde
bruits
• Traube’s sign: pistol shot femorals
• Hill’s sign:BP Lower extremity >BP
Upper extremity by
• > 20 mm Hg - mild AR
• > 40 mm Hg – mod AR
• > 60 mm Hg – severe AR
Peripheral physical signs

39. Diagnosis-
• transthoracic echocardiography
• Chest X-ray - left ventricular hypertrophy and dilated aorta.
• ECG typically indicates left ventricular hypertrophy.
• Cardiac chamber catheterization

40. Treatment-
• Vasodilators-ACE inhibitors or angiotensin II receptor
antagonists, nifedipine, and
• low sodium diet, diuretics, digoxin, calcium blockers and
avoiding very strenuous activity.
• Surgery- aortic valve replacement
Prognosis-
AI is fatal in 10 to 20%,who do not undergo surgery.

41. Pulmonary Valve Stenosis
 The pulmonary valve is located between right ventricle and the pulmonary arteries.
 The pulmonary valve is made up of three thin pieces of tissue called cusps that are
arranged in a circle.
 With each heartbeat, the valve opens in the direction of blood flow — into the pulmonary
artery and continuing to the lungs — then closes to prevent blood from flowing backward
into the heart's right ventricle.
 valve acts as a doorway that lets blood into and out of the heart.
 in pulmonary valve stenosis-One or more of the cusps may be defective or too thick, or
the cusps may not separate from each other properly. If this happens, the valve doesn't
open correctly, restricting blood flow.

42. Signs And Symptoms-
 Heart murmur
 Fatigue
 Shortness of breath, especially during exertion
 Chest pain
 Loss of consciousness
 prominent and enlarged jugular vein
 bluish tint to the skin
 palpitations
 failure to thrive
 sudden death

43. Risk factors-
 Carcinoid syndrome
 Rheumatic fever
 Noonan syndrome
 Pulmonary valve replacement
Complications-
 Infection- infective endocarditis
 Right ventricular hypertrophy
 Heart failure.
 Arrhythmia
Diagnosis-
 Chest X-ray
 Electrocardiogram
 Echocardiogram
 MRI scan

44. Treatment-
• Mild stenosis may improve with time
• Prostaglandins to improve blood flow
• Blood thinners to reduce clotting
• A surgical procedure - balloon valvuloplasty.
• Replace the pulmonary valve

45. Pulmonary Regurgitation
• The pulmonary valve is located at the junction of the distal end
of the right ventricular outflow tract and the pulmonary artery.
• It is comprised of three semilunar leaflets which are of equal
dimensions.
• These leaflets are joined by three commissures, which are the
attachments between the pulmonic wall and the leaflets.
• The pulmonic valve is not attached to papillary muscles, as
seen with atrioventricular valves. The pulmonic valves help in
delivering deoxygenated blood from the right ventricle to the
lung vasculature during systole when they open completely.
• They close completely during diastole to prevent regurgitant
flow.

46.  A leaky pulmonary valve.
 This valve helps control the flow of blood passing from the right
ventricle to the lungs.
 A leaky pulmonary valve allows blood to flow back into the right
ventricle before it gets to the lungs for oxygen.
Symptoms -
• Heart murmur.
• Right ventricle enlarged.
• Heart failure
• Chest pain
• Fatigue
• lightheadedness or fainting.

47. Auscultation-
 The first heart sound (S) is normal.
 P2 is not audible .
 A systolic ejection click .
 An S3 and S4 audible.
 In the absence of pulmonary hypertension, the PR murmur is a
diamond-shaped, diastolic low pitched murmur which commences
as soon as the pulmonary artery and the right ventricular pressures
diverge .
 The Graham Steell murmur: It is heard when systolic pulmonary
artery pressure exceeds approximately 55 mm Hg, resulting in
dilatation of the pulmonary annulus causing high-velocity
regurgitant jet.

48. EKG-
- in the absence of pulmonary artery hypertension (PAH) are rSR configuration in
the right precordial leads, which reflects RV diastolic overload.
- If it is secondary to PAH, then a P-pulmonale (tall p waves- indicating right atrial
enlargement), increased r to s ratio in the right precordial leads along with right
axis deviation can be seen.
chest X-ray- both pulmonary artery and right ventricular enlargement are visible.
Echocardiography
 Mild PR - Normal right ventricular dimensions with thin (less than 10mm in
length) regurgitant jet width by color doppler
 Moderate PR - Normal or dilated right ventricle with intermediate regurgitant jet
width (less than 50% of pulmonic valve annulus)
 Severe PR- Dilated right ventricle (except in acute PR) with large regurgitant jet
width (greater than 50% of pulmonic valve annulus)
Angiography

49. Treatment -
• Symptomatic patients who are not surgical candidates- heart
failure therapy, especially diuretics, ACE inhibitors, and digoxin.
• Valve replacement
• transcatheter pulmonary valve replacement
• A bioprosthetic valve
• Percutaneous pulmonary valve implantation .

50. Tricuspid Valve Stenosis
 The tricuspid valve is located between the right atrium and right
ventricle
 Its role is to make sure blood flows in a forward direction from the
right atrium to the ventricle.
 abnormal function of the tricuspid valve.
 valve leaflets are stiff and do not open widely enough, causing a
restriction in the forward flow of blood.
Symptoms -
 Atrial fibrillation
 Fatigue
 fluttering discomfort in the neck
 Heart failure

51. Diagnosis-
• Mild diastolic murmur at left sternal border with rumbling
character and tricuspid opening snap with wide-splitting S2. It may
increase in intensity with inspiration (carvallo's sign).
• Echocardiograph
Treatment-
• Tricuspid valve stenosis itself usually doesn't require treatment.
• If stenosis is mild, monitoring the condition closely suffices
• Severe stenosis, or damage to other valves in the heart, may
require surgical repair or replacement.
• surgery -tricuspid valve replacement or percutaneous balloon
valvuloplasty.

52. Tricuspid regurgitation
• Tricuspid valve regurgitation is a condition in which the valve
between the right ventricle and right atrium doesn't close
properly.
• The malfunctioning valve allows blood to flow back into right
atrium
• Active pulsing in the neck veins
• Decreased urine output
• Fatigue
• General swelling
• ECG
• Echocardiogram
• Doppler echocardiography
• Right-sided cardiac catheterization
• CT scan or MRI of the chest (heart)

53. Treatment
• Treatment may not be needed if there are few or no symptoms.
• Heart failure- diuretics
• Repair or replace the tricuspid valve.