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Rheumatic Heart Disease: SMF Jantung Pembuluh Darah RSU UKI
Rheumatic Heart Disease: SMF Jantung Pembuluh Darah RSU UKI
Disease
Robert E Saragih SpJP FIHA
Learning Objectives:
To understand the pathogenesis of acute rheumatic fever and
rheumatic heart disease
To appreciate the burden of disease
To recognize the features of a streptococcal sore throat
To know the treatment regimens of a streptococcal sore throat
To be aware of secondary prevention measures
Performance Objectives:
Group A streptococcal
infections
Beta-haemolytic streptococci can be divided into a
number of sero-logical groups on the basis of their cellwall polysaccharide antigen.
Those in serological group A (Streptococcus pyogenes )
can be further subdivided into more than 130 distinct M
types, and are responsible for the vast majority of
infections in humans.
Only pharyngitis caused by group A streptococci has
been linked with the etiopathogenesis of RF and RHD.
Other streptococcal groups (e.g. B, C, G and F) have
been isolated from human subjects and are some-times
associated with infection; and streptococci in groups C
and G can produce extracellular antigens (including
streptolysin-O) with similar characteristics to that
produced by group A streptococci .
Streptococcal Mprotein
M-protein is one of the best-defined
determinants of bacterial virulence.
The streptococcal M-protein extends from
the surface of the streptococcal cell as
coiled coil dimer, and shares structural
homology with cardiac myosin and other
alpha-helical coiled coil molecules, such
as tropomyosin, keratin and laminin.
This homology is responsible for the
pathological findings in acute rheumatic
carditis.
Streptococcal
superantigens
The role of the superantigen-like activity of Mprotein fragments , as well as the
streptococcal pyrogenic exotoxin, in the
pathogenesis of RF.
Superantigenic activation is not limited to the
T-cell compartment alone.
Streptococcal erythrogenic toxin may behave
like a superantigen for the B-cell, leading to
the production of autoreactive antibodies.
Extracellular
products
and
cell-wall
components represent the virulence of group
A streptococci.
Host-pathogen
interaction
Infection by streptococci begins with the
binding of bacterial surface ligands to specific
receptors on host cells, and subsequently
involves specific processes of adherence,
colonization and invasion.
The binding of bacterial surface ligands to
host surface receptors is the most crucial
event in the colonization of the host, and it is
initiated by fibronectin and by streptococcal
fibronectin-binding proteins .
Streptococcal lipoteichoic acid and M-protein
also play a major role in bacterial adherence .
Diagnosis of rheumatic
fever
The Jones criteria were introduced in 1944 as a
set of clinical guidelines for the diagnosis of
rheumatic fever (RF) and carditis .
The clinical features of RF were divided into major
and minor categories, based on the prevalence
and specificity of manifestations .
Major manifestations were
included carditis,
joint symptoms, subcutaneous nodules, and
chorea.
A history of RF or preexisting rheumatic heart
disease (RHD) was considered to be a major
criterion .
Chronic rheumatic
heart disease
1. Mitral stenosis(MS)
. In developing countries, MS progresses much
more rapidly, because of more severe or repeated
streptococcal infections, genetic influences, or
economic conditions, and may lead to symptoms
in the late teens and early twenties .
. Survival is >80% at 10 years for untreated
patients who are asymptomatic or minimally
symptomatic (New York Heart Association (NYHA)
Functional Class I/II) at time of diagnosis
Mitral stenosis
Mitral regurgitation(MR)
The volume load of chronic MR can be well
tolerated for several years.
Symptoms and/or signs of left ventricular systolic
dysfunction (defined by an ejection fraction
<0.60, or an end-systolic dimension 4.5 cm) are
indications for surgery .
Compared with patients with predominant
stenosis, patients with isolated MR are less
susceptible to thromboembolism with AF, but are
more prone to infective endocarditis.
Aortic stenosis(AS)
The well-known natural history of AS has long
dictated that surgery be undertaken once
symptoms appear.
Survival without valve replacement after the
onset of angina, syncope, or heart failure is
generally measured at five, three, and two years,
respectively .
For patients with severe AS (valve area 1.0cm2)
who develop heart failure and who are not
candidates for surgery, diuretics can be provided
to alleviate congestion.
Aortic regurgitation(AR)
Patients with chronic, severe AR usually enjoy a
long,yet variable compensated phase characterized
by an increase in left ventricular end-diastolic
volume, an increase in chamber compliance,and a
combination of both eccentric and concentric
hypertrophy.
Preload reserve is maintained, ejection performance
remains normal, and the enormous increase in
stroke volume allows preservation of forward output
.
AR thus leads both to volume and pressure overload
.
Preoperative left ventricular function is the most
important predictor of postoperative survival
Antimicrobial therapy
Eradication of the pharyngeal streptococcal
infection is mandatory to avoid chronic repetitive
exposure to streptococcal antigens .
Ideally, two throat cultures should be performed
before starting antibiotics.
However, antibiotic therapy is warranted even if
the throat cultures are negative.
The eradication of pharyngeal streptococci should
be followed by long-term secondary prophylaxis to
guard against recurrent pharyngeal streptococcal
infections.
Contra-indications to
surgery
Relative contra-indications include manifestations
of end-stage valve disease, such as very poor LV
function in association with a regurgitant lesion,
severe fixed pulmonary hypertension or extensive
extraannular
tissue
destruction
due
to
uncontrolled endocarditis
Treatment options
1. Balloon valvotomy (commissurotomy)
2. Surgical treatment
Secondary prevention of
rheumatic fever
TERIMAKASIH