1. Dr. Sharan . S. D

2.  Acute rheumatic fever is a multisystem disease
resulting from an autoimmune reaction to
infection with group A streptococcus.
 Although many parts of the body may be affected,
almost all of the manifestations resolve
completely.
 The exception is cardiac valvular damage
[rheumatic heart disease (RHD)], which may
persist after the other features have disappeared

4.  ARF is mainly a disease of children aged 5–15 years. Initial
episodes become less common in older adolescents and
young adults and are rare in persons aged >30 years.
 By contrast, recurrent episodes of ARF remain relatively
common in adolescents and young adults.
 This pattern contrasts with the prevalence of RHD, which
peaks between 25 and 40 years.
 There is no clear gender association for ARF, but RHD more
commonly affects females, sometimes up to twice as
frequently as males.

6.  ARF is exclusively caused by infection of the upper
respiratory tract with group A streptococci.
 Although classically, certain M-serotypes (particularly
types 1, 3, 5, 6, 14, 18, 19, 24, 27, and 29) were
associated with ARF, in high-incidence regions.
 It is now thought that any strain of group A
streptococcus has the potential to cause ARF.
 Potential role of skin infection and of groups C and G
streptococci are currently being investigated.

7.  Approximately 3–6% of any population may be
susceptible to ARF, and this proportion does
not vary dramatically between populations.
 Findings of familial clustering of cases and
concordance in monozygotic twins—
particularly for chorea—confirm that
susceptibility to ARF is an inherited
characteristic.

8.  Particular human leukocyte antigen (HLA)
class II alleles appear to be strongly associated
with susceptibility.
 Associations have also been described with
high levels of circulating mannose-binding
lectin and polymorphisms of transforming
growth factor 1 gene and immunoglobulin
genes

9.  High-level expression of a particular
alloantigen present on B cells, D8-17, has been
found in patients with a history of ARF in many
populations, with intermediate-level
expression in first-degree family members--
marker of inherited susceptibility.

10.  When a susceptible host encounters a group A
streptococcus, an autoimmune reaction
results, which leads to damage to human
tissues as a result of cross-reactivity between
epitopes on the organism and the host

11.  Cross-reactive epitopes are present in the streptococcal
M protein (M1,M5,M6,M19)and the N-
acetylglucosamine of group A streptococcal
carbohydrate and are immunologically similar to
molecules in human myosin, tropomyosin, keratin,
actin, laminin, vimentin, and N-acetylglucosamine.
 It is currently thought that the initial damage is due to
cross-reactive antibodies attaching at the cardiac valve
endothelium, allowing the entry of primed CD4+ T cells,
leading to subsequent T cell-mediated inflammation

14.  There is a latent period of 3 weeks (1–5 weeks) between the
precipitating group A streptococcal infection and the
appearance of the clinical features of ARF …
 The exceptions are indolent carditis and chorea, which may
follow prolonged latent periods lasting up to 6 months …
 A prior sore throat, the preceding group A streptococcal
infection is commonly subclinical; in these cases it can only
be confirmed using streptococcal antibody testing …
 The most common clinical presentation of ARF is
polyarthritis and fever…

15.  Polyarthritis is present in 60–75% of cases and
carditis in 50–60% …
 The prevalence of chorea in ARF varies
substantially between populations, ranging
from 10-15%…
 Erythema marginatum and subcutaneous
nodules are now rare, being found in <5% of
cases …

16.  Up to 60% of patients with ARF progress to RHD …
 The endocardium, pericardium, or myocardium may be
affected. Valvular damage is the hallmark of rheumatic
carditis …
 The mitral valve is almost always affected, sometimes
together with the aortic valve; isolated aortic valve
involvement is rare …
 Early valvular damage leads to regurgitation. Over ensuing
years, usually as a result of recurrent episodes, leaflet
thickening, scarring, calcification, and valvular stenosis may
develop …

17.  Acute rheumatic carditis usually presents as
tachycardia & cardiac murmur,with or without evidence
of myocardial or pericardial involvment…
 Moderate to severe rheumatic carditis can results in
cardiomegaly or CCF with hepatomegaly ,peripheral
&pulmonary edema…
 ECHO findings includes pericardial effusion,decreased
ventricular contractility & aortic or mitral
regurgitation…

18.  Therefore the characteristic manifestation of
carditis in previously unaffected individuals is
mitral regurgitation, sometimes accompanied by
aortic regurgitation …
 Myocardial inflammation may affect electrical
conduction pathways, leading to P-R interval
prolongation (first-degree AV block or rarely
higher-level block) and softening of the first heart
sound …

19.  To qualify as a major manifestation, joint involvement in
ARF must be arthritic, i.e., objective evidence of
inflammation with hot, swollen, red and/or tender
joints, and involvement of more than one joint
(i.e., polyarthritis) …
*The typical arthritis is migratory, moving from one joint to
another over a period of hours …
ARF almost always affects the large joints—most commonly
the knees, ankles,wrist,and elbows—and is asymmetric. The
pain is severe and usually disabling until anti-inflammatory
medication is commenced …

20.  Involvement of spine small joints of hand & feet or
hips is uncommon…
 The joint involvment is characteristically
migratory in nature,severly inflammed joint can
become normal within 1-3days without
treatment,as 1 or more other large joints become
involved…
 Monoarticular arthritis is unusual unless anti-
inflammatory theraphy is initiated…

21.  joint involvement that persists more than 1 or 2 days
after starting salicylates is unlikely to be due to ARF …
 Conversely, if salicylates are commenced early in the
illness, before fever and migratory polyarthritis have
become manifest, it may be difficult to make a diagnosis
of ARF …
 For this reason, salicylates and other NSAIDs should be
withheld—and pain managed with acetaminophen or
codeine—until the diagnosis is confirmed …

22.  Occurs in about 10-15% of patient with ARF…
 Sydenham's chorea commonly occurs in the absence of
other manifestations, follows a prolonged latent period
after group A streptococcal infection, and is found
mainly in females …
 The choreiform movements affect particularly the head
(causing characteristic darting movements of the
tongue) and the upper limbs …
 They may be generalized or restricted to one side of the
body (hemi-chorea) …

23.  The chorea varies in severity …
 In mild cases it may be evident only on careful
examination, while in the most severe cases
the affected individuals are unable to perform
activities of daily living and are at risk of
injuring themselves…
 Chorea eventually resolves completely, usually
within 6 weeks…

24.  Clinical maneuvers to elicit features of chorea include
 (1) demonstration of milkmaid's grip (irregular
contractions of the muscles of the hands while squeezing
the examiner's fingers).
 (2) spooning and pronation of the hands when the
patient's arms are extended.
 (3) wormian darting movements of the tongue upon
protrusion.
 (4) examination of handwriting to evaluate fine motor
movements.

25.  Usually presents as an isolated, frequently
subtle, neurologic behavior disorder.
 Emotional lability, incoordination, poor
school performance, uncontrollable
movements, and facial grimacing,
exacerbated by stress and disappearing with
sleep, are characteristic.

27.  The classic rash of ARF is erythema
marginatum which begins as pink macules
that clear centrally, leaving a
serpiginous, spreading edge.
 It occurs usually on the trunk, sometimes on
the limbs, but almost never on the face.

28.  Rare < 3% of patients with ARF but characteristic
rash of ARF…
 It consist of erythematous,serpiginous,macular
lesions with pale centre that are not pruritic…
 It occur primarily on the trunk & extremities,but
not on the face & can be accentuated by warming
the skin…

31. Subcutaneous nodules occur as painless, small (0.5–
2 cm), mobile lumps beneath the skin overlying
bony prominences, particularly of the
hands, feet, elbows, occiput, and occasionally the
vertebrae …
*Rare less 1% of patients with ARF.
* MC along extensor surface of tendon near bony
prominences…

32.  They are a delayed manifestation, appearing
2–3 weeks after the onset of disease, last for
just a few days up to 3 weeks, and are
commonly associated with carditis …

34.  Arthralgia in the absence of polyarthritis…
 Fever typically temp >102*F…
 The two LAB minor criteria are elevated
acute phase reactants ( CRP,ESR ) and
prolonged PR interval on ECG (1st degree
heart block)

35.  With the exception of chorea and low-grade
carditis, both of which may become manifest
many months later, evidence of a preceding
group A streptococcal infection is essential in
making the diagnosis of ARF.
 As most cases do not have a positive throat
swab culture or rapid antigen test, serologic
evidence is usually needed.

36.  The most common serologic tests are the anti-
streptolysin O (ASO) and anti-DNase B (ADB)
titers.
 Where possible, age-specific reference ranges
should be determined in a local population of
healthy people without a recent group A
streptococcal infection.

39.  All patients with acute rheumatic fever should
be placed on bed rest & monitored for evidence
of carditis…
 Patients with carditis requires longer periods of
bed rest…

40.  Once a diagnosis of ARF has been established &
regardless of throat culture results,the patient
should receive 10 days of orally administered
penicillin or erythromycin or single intramuscular
injection of benzathine penicillin to eradicate GAS
from upper respiratory tract…
 After this initial course of antibiotic therapy,the
patient should be started on long term antibiotics
prophylaxis…

41.  Anti-inflammatory
agents(eg:salicylates,corticosteroids) should be
withheld if arthralgia or atypical arthritis is
only clinical manifestation of presumed ARF…
 Early treatment with one of these agents may
interfere with development of characteristic
migratory polyarthritis & thus obscure the
diagnosis of ARF…

42.  Agents such as acetaminophen can be used
to control pain & fever while the patient is
being observed for more definative signs of
ARF & for evidence of another disease…

43.  Patients with typical migratory polyarthritis &
those with carditis without cardiomegaly or
CCF should be treated with oral salicylates…
 Usual dose of aspirin is 100 mg/kg/day in 4
divided doses PO for 3-5 days ,followed by
75mg/kg/day in 4 divided doses PO for 4 weeks

44.  Patients with carditis & cardiomegaly or CCF
should receive corticosteroids…
 Usual dose in 2mg/kg/day in 4 divided doses for
2-3 week f/b tapering dose of 5mg/day for
every 2-3 days…
 Aspirin should be at 75mg/kg/day in 4 divided
doses for 6 weeks

45.  Supportive therapies for patients with
moderate to severe carditis includes
digoxin,fluid and salt restriction,diuretics and
oxygen…
 Cardiac toxicity of digoxin is enhanced with
myocarditis

46.  Sedative may be helpful early in course of
chorea : Phenobarbital (16-32 mg every 6-8 hr
PO) is drug of choice…
 If phenobarbital is ineffective,then
haloperidol(0.001-0.003mg/kg/24hrs divided
bid PO ) or chlorpromazine (0.5mg/kg every 4-
6 hr PO ) should be started…

47.  Prognosis for patients with ARF depends on initial
clinical manifestation present at time of initial
episodes,severity of initial episodes,presence or
recurrence…
 Approximately 70% of patients with carditis
during initial episodes of ARF recover with no
residual heart disease…
 More severe the initial episodes are greater risk is
for residual heart disease

48.  Patients who had ARF are susceptible to
recurrent attacks following reinfection of upper
respiratory with grp A streptococcus.Therefore
patients require long-term continous
prophylaxis….
 Approximately 20% of patients who presents
with pure chorea who are not given secondary
prophylaxis develop RHD within 20 years…

49.  Approximately antibiotic theraphy instituted
before the 9th day of symptoms of acute grp A
streptococcus pharyngitis is highly effective in
preventing 1st attack of ARF from that
episode…
 About 30 % of patients with ARF don’t recall a
preceeding episodes of pharyngits…

52.  Valvular lesions in rheumatic fever begins as
small verrucae composed of fibrin & blood cells
along the borders of one or more of the heart
valves…
 Mitral valve is affected most often,f/b aortic
valve;right sided heart manifestations are
rare…

53.  As the inflammation subsides verrucae tend to
disappear & leave scar tissue…
 With repeated attacks of rheumatic fever,new
verrucae form near the previous ones,and the
mural endocardium & chordae tendineae
becomes involved…

54.  PATHOPHYSIOLOGY :
Mitral insufficiency is result of structural
changes that usually include some loss of
valvular substance & shortening & thickening
of chordea tendineae…

55.  During acute rheumatic fever with severe cardiac
involvement,heart failure is caused by a
combination of mitral insufficiency coupled with
inflammatory disease of
preicardium,myocardium,endocardium &
epicardium…
 Because of high volume load & inflammatory
process,the left ventricle becomes enlarged…
 The left atrium dilated as blood regurgitates into
this chamber…

56.  Increased left atrial pressure results in pulmonary
congestion & symptoms of left heart failure…
 More than half of patients with acute mitral
insufficiency no longer have the mitral murmur at 1
year…
 Severe chronic mitral insufficiency,pulmonary arterial
pressure becomes elevated,the right ventricle & atrium
become enlarged, & right-sided heart failure
subsequently develops …

57.  In mild disease,signs of heart failure are not
persent,the precordium is quiet,a high pitched
holosystolic murmur at apex that radiates to
axilla…
 Severe mitral insufficiency ,signs of chronic
heart failure may be noted,cardiomegaly with
a heaving apical left ventricular impulse &
often an apical systolic thrill…

58.  2nd HS may be accentuated if pulmonary HTN
in present…
 Holosystolic murmur is heard at the apex with
radiation to axilla…
 A short mid-diastolic rumbling murmur is
caused by increased blood flow across mitral
valve d/t insufficiency…

59.  Presence of diastolic murmur doesn’t
necessarily mean that mitral stenosis is
present…
 It take many years to develop MS & is
characterised by a diastolic murmur of greater
length,usually with presystolic accentuation…

60.  ECG,CXR is normal if lesion is mild…
 More severe insufficiency,the ECG show
prominent bifid P waves,signs of LVH,&
associated RVH if pulmonary HTN is present…
 CXR : prominence of left atrium &
ventricle,congestion of perihilar vessels,a sign of
pulmonary venous HTN may be evident…

61.  Calcification of mitral valve is rare in children…
 ECHO shows enlargment of left atrium &
ventricle,an abnormally thickened mitral
valve,DOPPLER demonstrate the severity of
MR…
 Heart catheterization & left ventriculography…

62.  Severe mitral insufficiency may result in
cardiac failure that may be precipitated by
progression of rheumatic process,onset of AF,or
infective endocarditis…
 Effect of chronic MR may manifest after many
years & include RVF, atrial & ventricular
arrhythmias …

63.  In mild MR prophylaxis against recurrences of
rheumatic fever is all that is required…
 Afterload-reducing agents(ACE inhibitors or
angiotensin receptor blocker ) may reduce the
reguritant volume & preserve left ventricular
function…

64.  Treat underlying heart
failure,arrhythmias,infective endocarditis…
 Surgical treatment is indicated for patients
who despite adequate medical theraphy have
persistent heart failure,dyspnea with moderate
activity,and progressive cardiomegaly,often
with pulmonary HTN…

65.  In patients with a prosthetic mitral valve
replacement,prophylaxis against bacterial
endocarditis is warranted for dental
procedure,as routine antiobiotics taken by
these patients for rheumatic fever prophylaxis
are insufficient to prevent endocarditis …

66.  PATHOPHYSIOLOGY :
Mitral stenosis of rheumatic origin results from
fibrosis of mitral ring,commissural
adhesion,contracture of valve leaflets,chordae
& papillary muscle over time…
Its take 10 years or more for lesion to become
fully established….

67.  Significant mitral stenosis results in increased
pressure , enlargement & hypertrophy of left
atrium,pulmonary venous HTN,increase
pulmonary vascular resistance,pulmonary
HTN…
 RVH,RAD ensue & are followed by RVD,TR &
clinical sign of right-sided heart failure…

68.  Mild lesion are symptomatic …
 More severe degree of obstruction are
associated with exercise intolerance &
dyspnea…
 Critical lesions can result in
ortopnea,PND,overt pulmonary edema,atrial
arrhythmias…

69.  When pulmonary HTN has developed,right
ventricular dilatation may result in functional
TR,hepatomegaly,ascites & edema…
 Hemoptysis caused by rupture of bronchial or
pleurohilar veins & occasionally pulmonary
infarction may occur…

70.  Jugular venous pressure is increased in severe
disease with heart failure,tricuspid valve
disease,or severe pulmonary HTN…
 In mild cases,heart size is normal,in moderate
cardiomegaly is usual with severe MS…
 Cardiac enlargement can be massive when AF
& HF supervene …

71.  A parasternal RV lift is palpable when
pulmonary pressure in high…
 A loud 1st HS , opening snap of mitral valve,&
long,low-pitched,rumbling mitral diastolic
murmur with presystolic accentuation at the
apex …

72.  Mitral diastolic murmur may be virtually absent in
patient who are in significant HF…
 A holosystolic murmur secondary to TR may be
audible..
 In presence of pulmonary HTN,pulmonary
component of 2nd HS is accentuated…
 Early diastolic murmur may be caused by
associated AR or PR secondary to pulmonary HTN

73.  In severe cases prominent & notched P waves
& varying degrees of RVH become evident…
 AF is common late manifestation…
 Moderate or severe lesion CXR : signs of LA
enlargment & prominence of PA & right sided
chambers,calcification may be noted in mitral
valve …

74. • Doppler can estimate the transmitral pressure
gradient…
• ECHO : thickening of mitral valve,distinct
narrowing of mitral orifice during diastole & LA
enlargment…
• Cardiac catheterisation quantitates the
diastolic gradient across the mitral
valve,degree of elevation of PA pressure…

76.  Ballon valvuloplasty is indicated for
symptomatic,stenotic,pliable,noncalcified
valves of patients without atrial arrhythmias
or thrombi…

78.  AR leads to volume overload with dilatation
and hypertrophy of the LV…
 Combined MR & AR is more common than
aortic involvment alone…

79.  Symptoms are unusual except in severe AR…
 Large stroke volume & forceful left ventricular contraction
may result in palpitations…
 Sweating & heat intolerance are related to excessive
vasodilation…
 Dysnea on exertion can progress to orthopnea & pulmonary
edema,angina may be precipitated by heavy exercise…
 Nocturnal attacks with sweating,tachycardia,chest pain and
HTN may occur…

80.  Pulse pressure is wide with bounding peripheral
pulses…
 SBP is elevated,Diastolic BP is lowered…
 In severe AR ,heart is enlarged with a LV apical
heave…
 A diastolic thrill may be present…
 Typical murmur begins immediately with 2nd HS &
continues until late in diastole…

81.  Murmur best heard at upper & midleft sternal
border with radiation to apex & upper right
sternal border…
 A aortic systolic ejection murmur is frequent
because of increased stroke volume…
 An apical presystolic murmur(Austin flint
murmur) resembling that of MS …

82.  ECG : Signs of LVH & strain with prominent P
waves…
 ECHO : large LV & diastolic mitral valve flutter
or oscillation caused by regurgitant flow hitting
the valve leaflets…
 DOPPLER : degree of aortic runoff into LV…
 MRA