Rheumatic heart disease is a major complication of rheumatic fever, triggered by group A streptococcal throat infections, leading to inflammation of the heart and potential valve damage. It primarily affects children in developing nations and is characterized by symptoms such as carditis, polyarthritis, and chorea, with significant laboratory findings including elevated inflammatory markers and echocardiographic evidence of heart involvement. Long-term effects include severe valvular diseases, particularly affecting the mitral valve, which can result in heart failure and other serious complications.

1. RHEUMATIC HEART
DISEASE
MODERATOR -- DR. SANJEEVA.G.N
PRESENTER – DR.SANDEEP.R

2. RHEUMATIC FEVER – [ Rheumatic – Joints]
Rheumatic fever is an acute, immunologically mediated
multisystemic inflammatory disease that follows group A beta
haemolytic streptococcus infection of the throat.
“Licks the JOINT, bites the HEART”
- Lasegue

3. • Rheumatic fever emerged from the potpourri of “Rheumatism"
in the seventeenth century
• Guillaume de Baillou in France described it under the name of
"acute articular rheumatism“
• Deformities of the heart valves, first described anatomically by
Morgagni in Italy in 1761
• In 1886 Cheadle had described the full rheumatic fever
syndrome: carditis, polyarthritis, and chorea, as well as
subcutaneous nodules and erythema marginatum.
BRIEF HISTORY OF RHEUMATIC FEVER
Ref- MILTON MARKOWITZ Book of Rheumatic fever

4. EPIDEMIOLOGY -
Incidence-
Incidence of acute rheumatic fever varies with geographical location and
population which ranges from 3 to 61 per 1 lakh school children. In India
incidence of rheumatic fever by eco studies is 0.5 to 11 per thousand
Predisposing factors
1. Low socioeconomic status
2. Overcrowding
3. Poor medical care
Ref- Piyush Guptha PG Text book PEDIATRICS

5. ETIOLOGY
Agent-
• It is caused by the Group A beta-hemolytic streptococci
and more specifically by group A betahemolytic
streptococcal infections of the throat.
• Group A streptococcal infections of the throat always
precede the development of rheumatic fever, whether
first attacks or recurrences
• To initiate a rheumatic fever attack, group A streptococci
must cause an infection of the pharynx, not just a
superficial colonization.

6. GROUP A BETA HEMOLYTIC STREPTOCOCCI
 Gram-positive coccoid shaped bacteria grows in chains.
 Broadly classified by their hemolytic activity
 Beta hemolytic -
SEQUELA M TYPE
ACUTE RHEUMATIC FEVER 1, 3, 5, 6, 18, 29 [ pharyngeal
serotypes]
ACUTE GLOMERULONEPHRITIS
Throat 1, 4, 12
Skin 49, 55, 57, 60.
Ref- Nelson 21ST Edition.

7. Climate-
Temperate Countries – WINTER
Tropical Countries- RAINY SEASON
Host factors-
• Age- It is peak between 5 and 15 years
• Sex – both sexes equally effected
• Patients who have already had one attack of the disease
tend to have recurrences
Ref- Nelson 21ST Edition.

8. PATHOGENESIS AND PATHOLOGY
 Non suppurative sequel of streptoccal
infection
 Post streptococcal
 WHY? only streptococcal throat infection
leads to rheumatic fever?

9. CONTINUE.....
 To cause an infection the streptococci must first of all attach to
the epithelial cells in the pharynx of the host
 Virulent streptococci have fimbriae, avirulent ones don't
 streptococci get attached to epithelial cells by means of
lipoteichoic acid on the streptococcal side, and of a receptor for
lipoteichoic acid on the epithelial cell side
 The M proteins, which are also located on the fimbriae, make it
difficult for phagocytes to ingest the strep, thus further
increasing virulence

10. CONTINUE....
 A hyaluronic acid capsule, which further hinders phagocytosis
and thus enhances virulence, is often found on rheumatogenic
strains, especially in epidemic or near-epidemic streptococcal
outbreaks
o As the streptococcal products diffuse out of the pharyngeal
epithelium they encounter, of course, lymphoid cells and
stimulate antibody responses. Several streptococcal antigens
happen to cross-react immunologically with human tissue
antigens

11. Virulent strains
Fimbriae- M protein
Capsule- hyaluronic
acid
Non virulent
strains
Colonization f/b infection of the throat
Products diffuse out and there occurs cross reaction b/w streptoccal
antigens and human tissue antigens
Lipoteichoic acid
Attachment of GAS to the epithelial cells of
pharynx

13. LABORATORY MANIFESTATIONS
 Evidence of a recent streptococcal infection

14.  Evidence of systemic inflammation—
 ESR and CRP reflect the magnitude of the inflammatory
process ("rheumatic activity")
 are useful to find out if the inflammatory process is still going on
after the symptoms and signs have subsided
 If these tests are abnormal, such patients should be
reexamined at close intervals for other evidence of disease.

15.  Laboratory evidence of heart involvement
 Enlargement of the heart, pericardial effusion, and valve
deformities are all to be looked for
 Radiography of the heart, echocardiography, and
electrocardiography are the methods most often used to detect
heart involvement
 Prolongation of the P-R interval occurs in 20-40% of the
patients;
 Doppler ultrasonography has proven to be useful to identify
evidence of valvular involvement

16. DIAGNOSIS
 Jones criteria-
Ref- WHO Guidelines Rheumatic fever- 2004

19. MODIFIED JONES CRITERIA
Ref- AHA Journals- 2015

20. SUB CLINICAL CARDITIS
 It is an echocardiographic evidence of mitral or
aortic valvulitis in the abscnce of ascultatory
findings
 Current version of Jones criteria focused on this
concept
Ref- Nelson 21ST Edition.

21. DURATION OF THE RHEUMATIC ATTACK
 Duration as a whole varies according to the criterion used to
determine it, and to the clinical manifestations present
 It is the shortest in attacks characterized by arthritis alone; it is
longer in the presence of chorea and longest in the presence
of carditis.
 The duration of an initial attack of rheumatic fever ranges from
less than 6 weeks (in one-third of cases) to 3 months
 In patients with severe carditis, the active rheumatic process
may continue for 6 months or more. These patients have
"chronic" rheumatic fever

22. ACTIVE RHEUMATIC FEVER???
 Arthritis
 New origanic murmur
 Enlarged heart size
 Sleeping tachicardia
 Congestive heart failure in the absence of long standing sever
valvular disease
 Subcutaneous nodules.
 Persistence of an elevated sedimentation rate (ESR) for more
than 6 months should not be considered a sign of rheumatic
activity if no clinical signs are present
Ref- MILTON MARKOWITZ Book of Rheumatic fever

23. 1. ARTHRITIS 60-75%
2. CARDITIS 50-60%
3. CHOREA 10-15%
4. ERYTHEMA MARGINATUM 1-2%
5. SUB CUTANEOUS NODULES <1%
INCIDENCE OF 5 MAJOR CRITERIA
Ref- Nelson 21ST Edition.

24. RHEUMATIC HEART DISEASE
“It is an non suppuartive immune mediated
complication of group A streptococcal
infection characterised by inflammation of all
layers of the heart”
It is the most common acquired heart disease in
children
It is the long term sequelae of acute rheumatic
fever
Most commonly its is manifested as valvular
disease

25. EPIDIMIOLOGY-
a) Industrialised nations
 Incidence -less than 0.5 per 1 lakh
 Prevalence -less than 0.05/1000 children
b) Developing countries
 Prevalence – 0.4 -21/1000 children
Ref- Piyush Guptha PG Text book PEDIATRICS

26. PATHOGENSIS
Molecular Mimicry – antibodies against M proteins of
certain streptococcal strains binds to protein in
myocardial valves and causes injury by activation of
complements .
CD4 + T cells that recognises streptococcal peptide can
also cross react with host antigen leading to cytokine
mediated inflammatory response
Latent period - 2-3 weeks

28. PATHOLOGICAL MORPHOLOGY
Acute rheumatic fever is characterised by an discrete myocardial
inflammatory lesion called ASCHOFF BODIES - pathognomic for
rheumatic fever
Aschoff bodies are collection of lymphocytes, scattered plasma
cells and anitschkow cells ( activated macrophages) occasionally
punctuating zone of fibrinoid necrosis
Aschoff bodies can be found in any of the 3 layers of the heart
hence rheumatic fever said to cause PANCARDITIS

30. 1. Pericardium – fibrinous exudates – generally resolves without
squealae
2. Myocardium – myocarditis – in the form of scattered aschoff
bodies within the intertial connective tissue
3. Endocardium - valvular lesions – in the form of verrucae
(fibrinoid necrosis and fibrin deposition along the lines of
closure- 1-2 mm )
The most Important functional consequence of rheumatic
heart disease is valvular lesions ( insufficiency/stenosis)
PANCARDITIS FEATURES

31. Ref- WHO Guidelines Rheumatic fever- 2004

32. Order of valve involvement in RHD-
1. Mitral valve 70%
2. Combined mitral and aortic disease 25%
3. Tricuspid – less frequently involved
4. Pulmonary valves – almost always escapes injury
Ref- MILTON MARKOWITZ Book of Rheumatic fever

33. MITRAL INSUFFICINECY
Mitral insufficient is the result of structural changes that may
include some loss of valvular substance and/ or changes to
sub valvular apparatus in the form of elongation of chordae.
In case of ARF with severe cardiac failure is secondary to
mitral insufficiency and pan carditis .

34. PATHOPHYSIOLOGY OF ACUTE SEVERE MITRAL
INSUFFIENENCY
Valve or Sub valvular damage
secondary to inflammatory process
MITRAL INSUFFICINCY
Increased volume load leading to left
ventricular dilatation. Increased left atrial pressure
resulting in pulmonary congestion
LEFT HEART FAILURE

35. CHRONIC SEVERE INSUFFIENCY
Leaflet and chordal thickening, chordal fussion and restricted
leaflet motion secondary to fibrosis
Persistent mitral insuffiency
Increased Pulmonary artery pressure[PAH]
Development of RA/RV dilatation
RIGHT HEART FAILURE

36. CLINICAL MAINFESTATION
Mild disease –
1. NO sign of heart failure
2. Precordium normal , apex normal
3. Ascultation - Normal S2
Holosystolic Murmur (best heard at apex,
radiating to axilla)

37. Severe Disease –
1. Signs of Heart failure
2. Apex beat – heaving apical impulse with cardiomegaly
3. Ascultation – wide split S2 , P2 may be louder if Pulmonary
hypertension develops , S3 present , CAREY COOMBS
MUMRMUR

38. ELECTROCARDIOGRAM AND CHEST
RADIOGRAPHS
1. Both are normal in mild disease
2. Severe disease –
• ECG – prominent ,long duration and often bifid ‘p’
waves .
• Chest radiography – LA/LV dilatation , prehilar
congestion if PAH is developed.

40. 2D ECHO FINDINGS IN SEVERE MITRAL
INSUFFIENCY
Acute phase
• Mitral annnular dilatation , chordal elongation and at times
evidence of chordal rupture – resulting in flail leaflet
Chronic phase
• Leaflet and chordal thickening, chordal fussion and
restricted leaflet motion leading to variable degree of
regurgitation

41. COMPLICATION OF MITRAL INSSUFIENCY
1. Cardiac failure
2. Atrial fibrilation
3. Arrythmias
4. Infective endocarditis

42. MITRAL STENOSIS
It results from firbrosis of mitral ring, commissural adhesions
and contracture of the valve leaflets, chordae and papillary
muscles.
• Pure or predominant MS occurs in approximately 40% of all
patients with rheumatic heart disease
•It is a chronic processes and often takes >10years for lesions to
become fully establish
• Two‐thirds of all patients with MS are female
• Reduction of valvular size to 25% or less
• Rarely it may occur in 6 months to 2 years (juvenile mitral

43. • 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

45. PATHOPHYSIOLOGY

46. CLINICAL MANIFESTATIONS-
SYMPTOMS
 Palpitations
 Cough
 Orthopnea
 PND
 Respiratory infections
 Hepatic congestion
 Edema
 hemoptysis
COMPLICATIONS
 Atrial fibrillations
 Systemic embolism
 Left heart failure
 Pulmonary
hypertension
 Right heart failure

47. RECOGNIZING MITRAL STENOSIS
Palpation:
• Small volume pulse
• Tapping apex‐palpable S1
• +/‐ palpable opening snap (OS)
• RV lift
• Palpable S2
ECG:
• LAE, A Fib, RVH, RAD

48. PHYSICAL EXAMINATION
• First heart sound (S1) is accentuated and snapping
S1_____________S2__OS________________S1
• Opening snap (OS) after aortic valve closure
• Low pitch diastolic rumble at the apex
• Pre‐systolic accentuation (esp. if in sinus rhythm)

49. PROGRESSION-
• Progressive / life long disease,
• Usually slow & stable in the early years.
• Progressive acceleration in the later years
• 20‐40 year latency from rheumatic fever to
symptom onset.

50. AORTIC INSUFFIENCY
•It is the most common complication of rheumatic aortic
valvitis.
•Isolated A.R is seen in 1/12th
•Combined mitral and aortic insuffiency in acute ARF is more
common presentation.
•Most common in men
•Patients with recurrent rheumatic episodes will have more
rapid progression.

51. PATHOPHYSIOLOGY
ACUTE – Poor coaptation of leaflet or leaflet prolapse
Regurgitation of blood leading to LV volume overload
Compensatory mechanisms-
Increased chamber compliance and both
concentric and eccentric hypertrophy of ventricles.
degree of dilatation directly proportional to
severity of A.R.

52. Chronic Aortic Regurgitation
Increase in the ventricular dilatation over an
period of time leading to increased diastolic
compliance and permits ventricles to accommodate
a large regurgitationt blood without increase in end
diastolic pressure.
Aortic lesion were associated with
increased peripheral vasodilatation

53. Rapid dissipation of blood into the dilated perpheral vasculature
resulting in low diastolic pressure.
Reduced coronary flow Inadequate nutrition to
peripheral tissue
Peak systolic pressure increases as a compensation
Leading to increase in MEAN ARTERIAL PRESSURE
Signs of Aortic Regurgitation.

54. CLINICAL FEATURES
Symptoms seen in severe in aortic insuffiency with mitral
involvement or Myocardial dysfunction
• Palpitation is the earliest symptom in patient of AR
• Wide pulse pressure
• Exertional angina
• Heaving apex
• Blowing early diastolic murmur in aortic area‐ best heard in
expiration
• AUSTIN FLINT MURMUR – apical presystolic murmur due to
large flow across mitral valve

55. Signs of Aortic Regurgitation.

56. INVESTIGATION
Chest radiograph – Enlargement of left ventricle
and aorta
ECG – normal in majority of cases , in advance case
it reveals signs of LV hypertrophy with a strain
pattern and prominent ‘p’ waves
2D ECHO – dilatation of left ventricle and diastolic
mitral valve flutter or oscillation caused by aortic
regurgitant flow hitting the valve leaflet

57. TRICUSPID VALVE DISEASE
Involvement is rare in both acute and chronic
phases of rheumatic fever
It is more common secondary to RV dilatation ,
resulting from significant left sided cardiac lesion

58. CLINICAL MANIFESTATIONS-
•Prominent pulsation of jugular veins
•Systolic pulsation of liver
•Blowing holosystolic murmur at lower left sternal border that
increases in intensity during inspiration
Concomitant signs of mitral or aortic valve disease
with or without AF

59. PULMONARY VALVE DISEASE
•It is rare and occurring on functional basis secondary to
pulmonary hypertension
•It is an late findings of mitral stenosis
•It is confirmed by 2D Echo and Doppler findings

60. MURMURS OF RHD

61. CARDIAC CONDITIONS SIMULATING RHEUMATIC
CARDITIS AND RHEUMATIC HEART DISEASE
 Innocent murmurs –
• Always systolic
• Best heard in pulmonary / lower left sternal
border
ex- Pulmonic murmur
Stills murmur

62. Infective Endocarditis-
May be mistaken for a recurrent attacks of rheumatic
fever in a patient with established rheumatic heart disease
 Confusion occurs because often IE affects the joints as well
as heart
 Differentiating points-
• Joint involvement is monoarticular
• High grade fever
• Associated with extra cardiac manifestations

63. Mitral Valve Prolapse [floppy mitral valve, click
syndrome, or Barlow's syndrome ]
• Benign disorder
• It can be distinguished from its rheumatic counterpart
clinically on the basis of the characteristic systolic click and
late systolic murmur.
• Echocardiography is the most reliable way to confirm the
diagnosis.

64. Congenital heart disease-
 The systolic murmur of a ventricular septal defect due to
a small left-to-right shunt can resemble the murmur of mild
mitral regurgitation.
 Atrial septal defects associated with a deformed mitral
valve can mimic rheumatic mitral regurgitation.
 Congenital bicuspid aortic valve- diastolic murmur of aortic
regurgitation, which can occur in patients with ventricular
septal defect and aortic insufficiency

65. Viral Carditis-
 A number of viral agents, especially Coxsackie B and
Arboviruses, can cause myocarditis with cardiac
enlargement, arrhythmias, and heart failure.
 The absence of significant heart murmur generally
excludes rheumatic fever as the etiology

66. ARTHRITIS-
 Most Common – 30 to 50%
 Early manifestation
 Pain > swelling
 Large joint involvement
 Pathogenesis- The articular and periarticular structures are swollen and
edematous, but there is never erosion of the joint surfaces or pannus
formation. The exudate is turbid but never purulent.
Migratory Polyarthritis ?????
 No Permanent Sequelae
 Resolves in Six weeks

67.  Arthritis often affects the legs first and then spreads to the arms.
The knees are most frequently affected (75%) followed by the
ankles (50%), elbows, wrists, hips, and small joints of the feet
(each 12-15%), shoulders and small joints of the hand (7-8%).
 Sacroiliac, Temporo‐mandibular and Cervical joints involvement
is rare.
Ref- MILTON MARKOWITZ Book of Rheumatic fever

68. JACCOUD'S ARTHRITIS
 Jaccoud's arthropathy appears after multiple, prolonged, and
severe attacks of rheumatic fever
 Jaccoud's arthritis is also called, more descriptively, chronic
post-rheumatic fever arthropathy. It is a rare, indolent, slowly
progressive process that deforms the fingers and sometimes the
toes. The deformity consists of ulnar deviation of the fingers,
flexion of the metacarpophalangeal joints, and hyperextension
of the proximal interphalangeal joints

69. JACCOUD'S ARTHRITIS

70. SYDENHAM’S CHOREA
 10 – 15 % of patients
 Usually delayed/often sole manifestation of ARF
 Sydenham's chorea, chorea minor, or "St Vitus' dance" is a
neurological disorder consisting of involuntary movements,
muscular weakness, and emotional disturbances.
 It disappear during sleep, but may occur at rest and may
interfere with voluntary activity

71.  In exceptional cases the psychological manifestations may be
quite severe, and result in transient psychosis "chorea
insaniens"
 The neurological examination does not reveal sensory losses or
pyramidal tract involvement.
 Chorea is unusual after puberty, and does not occur in adults,
with the exception ofrare cases during pregnancy "chorea
gravidarum"

72.  Milkmaid's grip – irregular contractions of the muscles of the
hands while squeezing the examiner's fingers
 Spooning and pronation of the hands when the patient's arms
are extended(St. Vitus Dance)
 Wormian movements of the tongue upon protrusion -Jack in the
Box
 Handwriting to evaluate fine motor movements

73. St. Vitus Dance

74. Pronator sign

75. ERYTHEMA MARGINATUM
 Occurs 1-2% of Patients
 Macular non pruritic rash
 Serpiginous border, raised edges, central
clearing
 Most common on trunk less common on
face

76. ERYTHEMA MARGINATUM

77. SUBCUTANEOUS NODULES
 Seen in around <1%
 Small, pea sized, 0.5 to 2 cms in diameter , Firm,
mobile, Painless
 Seen over the extensor surface of Wrist, Elbow,
Spine
 Usually seen in individuals with long standing
carditis

78. SUBCUTANEOUS NODULES

79. POINTS TO REMEMBERED...
 Rheumatic fever is an acute, immunologically mediated
multisystemic inflammatory disease that follows group A beta
haemolytic streptococcus { 1, 3, 5, 6, 18, 29 [ pharyngeal
serotypes]} infection of the throat.
 In India incidence of rheumatic fever by eco studies is 0.5 to 11
per thousand, most common age group is 5-15
 Diagnosis is based on MODIFIED JONES CRITERIA
 Exceptions for Jones criteria is Carditis, recurrence of
Rheumatic fever and Choria.

80.  ARTHRITIS 60-75%,CARDITIS 50-60%, CHOREA 10-
15%, ERYTHEMA MARGINATUM 1-2%, SUB CUTANEOUS
NODULES <1%
 RHD can lead to pancarditis in which ENDOCARDITIS is
must.
 Pathgnomic of RHD is ASCHOFF BODIES
 Sub clinical carditis is an echocardiographic evidence of
mitral or aortic valvulitis in the abscnce of ascultatory findings

81.  Valvitis -Mitral valve 70%, Combined mitral and aortic
disease 25%, Tricuspid – less frequently involved
Pulmonary valves – almost always escapes injury .
 Clinical history and physical examination remains the
mainstay for dianosing RF and RHD particularly in resource
poor settings
 Infective endocarditis remains a major treat for individuals
with rheumatic valvular disease.

82. THANK YOU