RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

Posted: Aug 31, 2010

By: Dr Izharul Hasan 

Rheumatic heart disease is now uncommon in western Europe and North America, but is an
important historical cause of what was once the bulk of valvular heart disease. It is still
important in the developing world as a disease of childhood and a major cause of heart
disease. Its decline in western countries started before the widespread use of antibiotics,
and probably reflects increased standards of nutrition and hygiene and, possibly, a decline
in the virulence and prevalence of Lancefield group A hemolytic streptococci.

Rheumatic fever and chorea are the exclusive causes of chronic rheumatic heart disease, in
which there is interstitial muscle fibrosis as well as the heart valve disease. However, some
50% of patients with chronic rheumatic heart disease give no history of either condition.
Rheumatic fever develops in about 3% of young people exposed to an epidemic strain of (3-
haemolytic Lancefield group A streptococcus. These patients usually have pharyngitis, but
may have scarlet fever.

After an apparent recovery they develop fever, sweats, skin rashes, arthritis and,
sometimes, cardiac involvement from what appears to be an autoimmune disease starting
2-4 weeks after the initial sore throat and lasting 6-8 weeks, but occasionally as long as 26
weeks. Some patients give no definite history of an antecedent sore throat.
Following the attack of rheumatic fever, some 70% of patients will eventually have evidence
of rheumatic valvular heart disease, although the time interval can be as long as 50 years.
On the other hand, the valve disease may become manifest during the attack of rheumatic
fever, especially where regurgitation develops. Rheumatic fever may be recurrent, and the
chances of valve disease increase with the number of attacks.

Pathology
Although rheumatic fever follows a streptococcal infection, no bacteria are found in the
lesions; however, they may still be present in the pharynx. There is an acute inflammatory
reaction, with edema in the affected tissues, and then granulomatous lesions develop in the
myocardium(Aschoff nodules). Edema of the valve cusps is followed by veracious fibrin
deposits developing along the lines of apposition of the valve cusps, which eventually
adhere at the commissures. In the acute illness there is an acute non-bacterial sinusitis in
the joints, and granulomata may develop subcutaneously on extensor surfaces of the limbs
and over the Achilles tendons (rheumatic nodules).There is usually a high antistreptolysin O
titre, and other antistreptococcal titres may also be high. It is thought that cross-reactivity
between the streptococcal antigens and host tissue antigens leads to an autoimmune
inflammatory process.
Clinical features and diagnosis
The diagnosis of rheumatic fever depends on criteria proposed by Duckett-Jones, two major
criteria, or one major and two minor criteria, establish a high probability for the diagnosis.
The patient is almost always between the ages of 3 and30 years (usually 5-15); drenching
sweats and a prolonged feverish illness occur, with extremely painful arthritis that moves
from joint to joint (usually involving large joints such as the knee and elbow), which become
hot, red, swollen and extremely painful for a few days and then recover fully. The flitting
arthritis is characteristic, as is the classic skin rash, erythematic marginatum. Subcutaneous
nodules may develop after several weeks, usually over the elbows.
Carditis may be pericarditis, myocarditis or, more importantly for the eventual appearance
of valve disease,endocarditis. All may occur at once as pancarditis. The pericarditis is similar
to any acute pericarditis, with primordial pain, ECG changes (raised ST segments) and often
an increase in cardiac silhouette on chest X-ray.Myocarditis is manifest by a tachycardia
disproportionate for the degree of pyrexia, gallop rhythm and heart failure;there are also
ECG changes, such as prolonged PR intervalor greater degrees of AV block and T-wave
flattening or inversion, and raised serum creatine phosphokinase AV.Death occasionally
occurs from myocarditis.
Endocarditic is indicated by transient or changing murmurs. The classic murmur, which is
specific for thecondition, is the Carey Coombs murmur; this is a short rumbling diastolic
murmur indicating mitral valvulitis.

Sydenham's chorea
Sydenham's chorea (St Vitus' dance) is usually a separate disease entity but, like rheumatic
fever, appears to be related to recent p-haemolytic streptococcal infection andan
autoimmune process; occasionally it accompanies therheumatic fever, although it may
follow some weeks later. It is characterized by sudden, jerky, purposeless movements of
the face and limbs, such that the child is thought to be clumsy and grimacing. Pure chorea,
even when unaccompanied by other evidence of rheumatic fever, is followed by a high
incidence of rheumatic valve disease, indistinguishable from that following conventional
rheumatic fever.

Investigation
Laboratory investigations show a raised ESR, C-reactive protein and antistreptolysin O titre.
Blood cultures are negative. Throat swab may be positive for p-haemolyticstreptococcus, if
the preceding pharyngitis has not be entreated with antibiotic.

Differential diagnosis
As there is no absolute diagnostic test it is extremely common to find patients who have
been diagnosed as having rheumatic fever on weak evidence, such as a childhood fever with
a few aches and pains and the presence of a systolic murmur. In one study some 50% of
patients with mild congenital heart disease claimed to have had rheumatic fever, suggesting
that a child with a heart murmur alone is very likely to be diagnosed as having rheumatic
fever during the course of a febrile illness.

Management
Aspirin is traditionally used to control the arthritis and fever; it is used in maximum doses.
Where the patient is extremely ill, corticosteroids are commonly used, but there is no
evidence that these reduce the incidence of endocarditisor subsequent development of
rheumatic valvular disease.

Prophylaxis
Anyone who has had acute rheumatic fever is at risk of recurrence and is usually maintained
on phenoxymethylpenicillin(250 mg orally daily, or an I’m. depot preparation of benzathine
penicillin) or, if allergic to penicillin, oralsulphonamide, either until the age of 21 or for 5
yearsafter the last attack of rheumatic fever, whichever comeslater.
SUMMARY

Rheumatic heart disease is now uncommon in western Europe and North America, but is an
important historical cause of what was once the bulk of valvular heart disease. It is still
important in the developing world as a disease of childhood and a major cause of heart
disease. Rheumatic fever and chorea are the exclusive causes of chronic rheumatic heart
disease, in which there is interstitial muscle fibrosis as well as the heart valve disease.
However, some 50% of patients with chronic rheumatic heart disease give no history of
either condition. Rheumatic fever develops in about 3% of young people exposed to an
epidemic strain of (3-haemolytic Lancefield group A streptococcus. These patients usually
have pharyngitis, but may have scarlet fever.

Following the attack of rheumatic fever, some 70% of patients will eventually have evidence
of rheumatic valvular heart disease, although the time interval can be as long as 50 years.
On the other hand, the valve disease may become manifest during the attack of rheumatic
fever, especially where regurgitation develops. Rheumatic fever may be recurrent, and the
chances of valve disease increase with the number of attacks.

Sydenham's chorea
Pure chorea, even when unaccompanied by other evidence of rheumatic fever, is followed
by a high incidence of rheumatic valve disease, indistinguishable from that following
conventional rheumatic fever.

Significance of the study

Rheumatic fever and rheumatic heart disease is one of the most dangerous childhood
disease. This article is all about giving information on how and why children or even adults
have these diseases. Nurses should know a lot of things with this diseases and also there
signs and symptoms.

Nursing Interventions
1. Monitor temperature frequently, and patient’s response to antipyretics.
2. Monitor the patient’s pulse frequently, especially after activity to determine degree of cardiac
compensation.
3. Auscultator the hear periodically for development of new heart murmur or pericardial or pleural
friction rub.
4. Observe for adverse effects of salicylate or nonsteroidal anti-inflammatory drug (NSAID)therapy,
such as stomach upset, tinnitus, headache, GI bleeding, and altered mental status.
5. Monitor the patient’s response to long-term activity restriction.
6. Restrict sodium and fluids and obtain daily weights as indicated.
7. Administer medications punctually and at regular intervals to achieve constant therapeutic blood
levels.
8. Explain the need to rest (usually prescribed for 4 to 12 weeks, depending on the severity of the
disease and health care provider’s preference) and assure the patient that bed rest will be imposed no
longer than necessary.
9. Assist the patient to resume activity very gradually once asymptomatic at rest and indicators of
acute inflammation have become normal.
10. Provide comfort measures.
11. Provide safe, supportive environment for the child with chorea.
12. Observe for the disappearance or any major or minor manifestations of the disease and report
signs of increased rheumatic activity as salicylates or steroids are being tampered.
13. Encourage continuous prophylactic antimicrobial therapy to prevent recurrence.