Acute rheumatic fever is a non-suppurative complication of Streptococcus pyogenes which only licks the joints, however bites the heart valves, particularly the mitral, aortic or both, rarely the tricuspid valve, resulting in valvular endothelial ulceration, collagen degeneration, neovascularization, interstitial calcification and fibrosis associated with lymphocytic infiltration . Currently in Turkey, it is believed that there are tens of thousands of heart valve diseased adult patients half of whom are class III or higher according to New York Heart Association (NYHA) classification . It is stated that the other half is in mild or moderate heart failure condition, ie. Class I or II, possibly due to previous interventions . Currently, the incidence of rheumatic fever (RF) and rheumatic heart disease (RHD) is really lowat certain parts of the globe compared to the incidence in Fiji, South Africa, New Zealand etc., and Turkey, with a high incidence following right after these countries . Many countries have recognized the problem and tried to manage the disease so the diagnosis could be made earlier, using hand held echocardiography, as a screening test . Some others have integrated using computer assisted auscultation in addition to two dimensional (2D) echocardiography, in an effort to improvethe diagnostic methods. The reason behind such efforts has been to initiate secondary prophylaxis without delay, hoping to minimize the upcoming valvular damage. Once the valve is damaged, valvular mechanics of the beating heart damages the valve even further. Hence, the valve will requirea suitable type of intervention, such as repair, balloon valvuloplasty and/or prosthetic valve replacement. In Turkey, the most frequent etiology of heart valve disease(HVD) is still of rheumatic original . The question is, is this disease preventable ? Many publications support the fact that RF and RHD are preventable. For many years, the prevalance of the disease has been shown to decline dramatically within decades in certain populations at a certain place when certain precautions are carried out. Recently, the decline in RF wasreported to be in association with the changes in M protein of Group A Streptococci, which is one of the main
portions of the microorganism responsible for the mechanism of antigenic mimicry within the host . Certain strategies should be proposed in order to increase the possibility of earlierdiagnosis and to keep track of the patient’s compliance to secondary prophylaxis, as well as to infective endocarditis prophylaxis. Due to variations of the disease incidence between regions, even within the same country, it is our belief that many doctors had to make their clinical judgements as a personal initiative. One of the main reasons for this has been the inadequacy of the criteria used within the last few decades . However, the disease is not only a problem of how healthcare is provided to the patient; but also mainly ralated to low economic income , crowded living conditions, low socioeconomic status overall. The aim is to discuss the most current recommendations in the diagnosis of definite and/or probable RF with special emphasis on subclinical carditis and preventive measures.
Antecedent group a beta streptococcal ınfection months of the infection . Currently, in our daily practice, serial
evaluation of rising ASO titers are the optimal tool for the evidence
of antecedent streptococcal throat infection.
The most recent major Jones criteria have been revised
according to the risk of the population; ie. high and low risk
populations .Low risk has been defined as RF incidence of
<2/100.000 school age children; or, all age rheumatic heart
disease prevalance of <1/1000 population per year.For the high
risk populations, monoarthritis, instead of polyarthritis , and
polyarthralgia (in the absence of arthritis) are now considered
as major criteria . On the other hand, polyarthritis only is
considered as a major criterion in the low risk populations. We
have been considering monoarthritis as a major criteria in our
country . For the high risk populations, polyarthralgia, low grade
fever of 38 or more, sedimentation rate of 30 mm/hour or more,
CRP: 3.0 mg/dl or more, PR prolongation in the absence of carditis
have been considered as minor criteria. On the other hand, for the
low risk populations, fever of 38.5 or more, sedimentation rate of
60 mm/hour or more, CRP>3.0 mg/dl or more, polyarthralgia (in
the absence of arthritis), prolonged PR on ECG (in the absence of
carditis) are considered as minor criteria . Chorea, erythema
marginatum and subcutaneous nodules are the other major
criteria in both risk groups. In the presence of chorea, elevation
of sedimentation rate and CRP shold not be expected. Chorea by
itself is diagnostic for RF and may be associated with carditis up to
half of the patients with chorea.
Pancarditis is the most important factor for morbidity and
mortality in RF. The valvular endothelial surface is effected in up
to 70-80% of the patients involving one or more cardiac valves.
Myocardial and pericardial involvement may be seen. Myocarditis
in RF is not associated with necrosis .
Troponin levels are not elevated in the pediatric RF patients
. Pericardial involvement is extremely rare in RF, unlike other
rheumatic diseases, eg. systemic lupus erythematosus in which
pericarditis may be the first manifestation of carditis.
Clinical scenario of rheumatic carditis is the presence of a
systolic or diastolic murmur. Rapid heart rate at rest, fatiguability
and chest painmay accompany. Low grade fever, monoarthritis or
polyartralgia, elevated both sedimentation rate and CRP is present
typically. Sometimes, 1st degree atrio-ventricular (AV) block or
rarely higher degrees of AV block may be present on ECG.Recently,
the presence of 2 major or 1 major and 2 minor or 3 minor criteria
is considered diagnostic for recurrent disease.
As per se, monoarthritis was extrapolated from the major
criteria not to overdiagnose RF once, Doppler echocardiographic
criteria set by World Health Organization (WHO) are considered
too strict , or even may be considered inadequate . Recently
many countries have developed their own echocardiographic
criteria in order not to underdiagnose the entity of silent carditis
[7-10]. In my experience, in children more than 10 years or older,
the observation of mitral valve prolapse especially at the tip of
the valve and/or anterior valve thickening, whether associated
with regurgitation or not , should be further questionedfor family
history. Recently, the presence of E148Q mutation on the second
exon of MEFV gene was found to be associated with carditis in the
Turkish population. In case of lack of of family history, a personal
history for epistaxis, frequent upper respiratory infections and
a latent period or an event of self resolving discomfort or pain
while moving medium to large sized joints should be obtained.
History should be followed by a complete physical examination
andsupportive laboratory data. Echocardiography is the most
important diagnostic tool for detection and prevention of
sequela in any patient suspected of RF. Every child with acute
rheumatic fever must undergo echocardiography at initial
presentation followed byanother at the end of the second week.
Morphological changes such as mitral annular dilation, chordal
elongation, anterior mitral leaflet prolapse just at the tip of
the valve, nodularity of the leaflet tips may not be evident on
the first echocardiographic examination. Therefore a second
exam preferably at the end of the second week of the disease is
recommended. Serial echocardiographic follow up is mandatory
in the presence of valvular regurgitation and presence of any of
the other changes. Among the echocardiographic and clinical
parameters, predictors for the development of chronic RHD were
initial big left ventricular end diastolic dimension and an audible
murmur at the first presentation .
Adult cardiology uses the following ehocardiographic criteria
in the diagnosis and managementof chronic valvulitis: right atrial
and coronary sinus volumes, mitral valve area, ejection fraction,
vena contracta, effective orifice area,etc.However in the pediatric
age group, the presence of such criteria would be extraordinarily
rare. Many of the pediatric patients fall into the category of,at
risk of developing mitral regurgitation. Before progressive mitral
regurgitation develops ,it is important to define which patients
are to be administered secondary prophylaxis. Regarding this,
the most recent guidelines from AHA/ACC have introduced
the concept of definite RF/probable RF in relavance to the
administration of secondary prophylaxis. In case of probable RF,
secondary prophylaxis is recommended for at least one year .
Such an approach is satisfactory, however, may be inadequate in
some cases where the expert has to put his/her initiative regarding
whether to continue or discontinue the prophylaxis. The weighted
pooled prevalence of silent carditis has been published as 17%.
However, without appropriate management, the disease is known
to progress over time . If compliance to secondary prophylaxis
is established 80% of mild mitral regurgitation is known to
diminish within 5 years . Within 3 years of follow-up, mitral regurgitation diminished in patients with good compliance to
secondary prophylaxis . Although secondary prophylaxis
with Peniciilin G is inadequate during the third week, a 3 weekly
regimen is satisfactory . We recommend a 2 weekly Penicillin
G prophylaxis during the 1st 6 months period following aninitial
acute attack and to be continued life long every 3 weeks.
In the etiopathogenesis, viral infections have never been
proven, however Coxsackie B, enterovirus, Hepatitis B particles
were found to a greater extent in the myocardium of the patients
with rheumatic carditis compared to normal population.The
Australian RF Group recommends influenza vaccine for patients
on ASA . In our practice,influenza vaccineand infective
endocarditis prophylaxis is recommended to all rheumatic carditis
patients with valvar insufficiency in the long term follow-up.
In conclusion, rheumatic carditis continues to be a health
issue particularly in crowded, low income parts of the World. The
prognostic importance of early diagnosis and treatment of silent
carditis using echocardiography and the need for initiation of a
policy to increase the awareness to the disease to minimize the
extent of the disease worldwide is emphasized.