Acute rheumatic heart

disease

Commonest form of acquired heart disease

in children especially in the developing
countries.
Contributes to the larger extent of the heart
disease in adults.
The long term outcome of the acute
rheumatic process depends on the state of
the patient during the initial attack. Those
who do not have carditis nearly always
remain free of chronic valvar heart disease.

Epidemiology
Prevalence in (1937)western countries 45/1000 children dropped to 2.9/100000 in
the late 1960s.
In India, the prevalence was 6-11/1000
(padmavathi,1978).
In south india a study done by Dr.Koshy et
el in 1981 showed the prevalence of
rheumatic fever and heart disease was
4.9/1000 in a survey in school children.

Streptococcal throat infection peaks in

winter months in western countries but
there was o seasonal variation in tropical
countries.
The incidence of rheumatic fever following
the documented streptococcal upper
respiratory infection varies from 0.3%
during sporadic cases to 3% during the
epidemics.

Attack rate is higher in individuals who

have had a previous rheumatic episode
than in the general population.
There are rebound and recurrences in
rheumatic fever. Rebound usually occurs
within two weeks of stopping the treatment.
Recurrence occur around two months or
more after discontinuationn of treatment
and always follow a new streptococcal
infection.

The recurrences frequently show the

appearance of c/f present in the first
attack ie.,mimetic or true to type.
There is lower incidence of carditis
during the recurrences in patients
initially free of it.
Rheumatogenic strains M type of
group A beta hemolytic streptococci
-3,5,6,12,18,19,24.

Etiology
Rheumatic fever follows 2-4weeks after a
Group A streptococcal infection of the
throat and not of the skin.
The infection can be identified in about
25% by bacteriological and in over 90% by
serological methods.
Individuals with vigorous antibody
response develop rheumatic fever with
greater frequency.

Prerequisites for development of ARF

group A streptococcus infection
especially of URI, streptococcal
antibody response and relative
persistence of the organism at the
site of infection.
Following is the picture of structure of
Streptococcus

Immunological cross reaction b/w streptococcal

component and mammalian tissue
components(Mccarty,1972)
Streptococcal
component

Mammalian tissue
component

Streptococcal hyaluronic
acid

Mammalian hyaluronicacid
& protein polysaccharide
Glycoproteins of heart
valves.
Sarcolemma of cardiac and
skeletal muscles.
Sarcolemma of cardiac and
skeletal muscles.

Group A carbohydrate
Protein cell wall
Protein of cell menbrane

Glycoprotein of the
cell membrane
Antigen of the cell
membrane

Glycoprotein of the
glomerular
basement
membrane
Histocompatibility
antigen.

Modified Jones criteria

Major
Carditis
Polyarthritis
Chorea
Subcutaneous
nodules
Erythema
marginatum

Minor
Fever
Arthralgia
Prolonged PR
interval on ECG
Increased
ESR,leukocytois or
CRP.

Essential criteria
Evidence of recent streptococcal
infection elevated streptococcal
antibodies.
Positive throat culture for group A
streptococcus.

Diagnosis
2 major or 1 major and two minor
criteria indicate high probability of
acute rheumatic process.
These criteria are guidelines not
meant to substitute clinician s
judgement .

Acute Rheumatic heart disease

Carditis is the most serious manifestation
of the acute rheumatic process since it
causes significant morbidity and mortality.
It is the common manifestation occuring in
1/3rd -1/2 of the patients with ARF.
It is a pancarditis.
Endocarditis valvar malfunction
Myocarditis CCF
Pericarditis Fibrinoid pericarditis or
pericardial effusion.

Cardiac tamponade was unusual.

Cardiac involvement tend to appear early
in the attack of acute process and rarely
delayed for up to 3 weeks.
At times, the carditis may be initially sub
clinical and may become overt during a
recurrent episode.
A history of rheumatic fever without carditis
does not confer immunity from future
rheumatic heart disease.

On examination
Apical murmur- pan systolic murmur of the
mitral regurgitation is heard. It is soft and
are transmitted to the axilla.
The intensity and duration of the murmur
can wane and may disappear towards
recovery.
The development of the low pitched apical
mid diastolic murmur (carey coombs)
murmur which is attributed to mitral
valvulitis helps to confirm the organic
significance of MR murmur.It tend to
disappear during recovery.

AR- a early decrescendo murmur .It

tends to be more persistent and
indicates permanent valvar injury.
But development of severe AR
following a single episode of ARF is
unusual.
Murmurs due to valvar stenosis are
seldom seen in initial attack of active
carditis.

The murmurs in acute rheumatic carditis

typically change from day to day depending
on the alteration in the myocardial and
valvar function with or without treatment.
S1- may be diminished in about half the
children with MR in the presence of
carditis. It is attributed to lengthened
conducted time which allows for early
closure of mitral valve and diminishes the
valvar component of the first heart sound.

In a patient with earlier RHD or past h/o

ARF documentation of a change in the
established murmur or appearance of new
murmur is significant.
CCF occurs with varying frequency with
cardiomegaly, persistent elevation of the
sleeping pulse rate and a gallop rhythm.
They donot occur without organic murmur.

The sudden onset of CCF with

cardiomegaly may the first
manifestation in acute rheumatic
valvar disease
Cardiac failure alone should not be
accepted as sole evidence of active
carditis without supporting evidence.

Pericarditis always associated with

valvar involvement.
Commonly manifests as friction rub
heard over the pericardium, especially
over the sternum, with a to and fro
character in systole and diastole.
It lasts from a few days to weeks.
Pericardial effusion is not uncommon.

In the presence of CCF with

myocarditis , pericardial effusion may
be unnoticed.
ECG abnormal ST segment and T
wave changes in the left praecardium
ECHO helps to confirm the
diagnosis.

During active phase of carditis, chest radiograph

may show rapid changes in the heart size,
significant pulmonary congestion or even frank
pulmonary edema.
It doesnot carry grave prognosis for immediate
outcome or longterm valve dysfunction.
ECG show disproportionate sinus tachycardia, a
prolonged P-R interval or a prolonged QT interval.
Conduction defects due to AV or fascicular blocks
may develop transiently but are rarely permanent.

Prolonged PR interval occurs in

40% of patients with ARF.
In isolation, of much help.
It does not correlate with active
carditis, prognosis or residual heart
disease.

Natural history
TOMPKIN et el 1972 reported that about
2/3rd of patients with mitral regurgitation or
AR became free of murmurs within 5-10
years.
Mitral stenosis did not develop in any
patient who was on regular penicillin
prophylaxis.
New valvular lesions do not develop
usually in the absence of recurrences,
even in the patient who had carditis as an
initial attack but old valvar lesions may
evolve further.