Rheumatic Fever and Rheumatic Heart Disease - Causes, Symptoms and Treatments

Often recurrent, acute rheumatic fever is a systemic inflammatory disease of childhood that follows a group A beta­hemolytic streptococcal infection. Rheumatic heart disease refers to the cardiac manifestations of rheumatic fever, and includes pancarditis (myocarditis, pericarditis, and endocarditis) during the early acute phase, and chronic valvular disease later.

Long-term antibiotic therapy can minimize recurrence of rheumatic fever, reducing the risk of permanent cardiac damage and eventual valvular deformity. However, severe pancarditis occasionally produces fatal heart failure during the acute phase. Of the patients who survive this complication, about 20% die within 10 years. Although rheumatic fever tends to run in families, this may merely reflect contributing environmental factors. For example, in lower socioeconomic groups, incidence is highest in children between ages 5 and 15, probably as a result of malnutrition and crowded living conditions.

This disease strikes most often during cool, damp weather in the winter and early spring. In the United States, it's most common in the northern states.

Causes of Rheumatic Fever and Rheumatic Heart Disease:

Rheumatic fever appears to be a hypersensitivity reaction to a group A beta­hemolytic streptococcal infection, in which antibodies manufactured to combat streptococci react and produce characteristic lesions at specific tissue sites, especially in the heart and joints. Because very few people (about 0.3%) with streptococcal infections ever contract rheumatic fever, altered host resistance must be involved in its development or recurrence.

Signs and symptoms of Rheumatic Fever and Rheumatic Heart Disease:

In 95% of patients, rheumatic fever characteristically follows a streptococcal infection that appeared a few days to 6 weeks earlier. A temperature of at least 100.4 F (38 C) occurs.

Most patients complain of migratory joint pain or polyarthritis. Swelling, redness, and signs of effusion usually accompany such pain, which most commonly affects the knees, ankles, elbows, or hips.

In 5% of patients (generally those with carditis), rheumatic fever causes skin lesions such as erythema marginatum. This nonpruritie, macular, transient rash gives rise to red lesions with blanched centers.

Rheumatic fever may also produce firm, movable, non tender, subcutaneous nodules about 3 mm to 2 cm in diameter, usually near tendons or bony prominences of joints (especially the elbows, knuckles, wrists, and knees) and less often on the scalp and backs of the hands. These nodules persist for a few days to several weeks and, like erythema marginatum, often accompany carditis.

Later, rheumatic fever may cause transient chorea, which develops up to 6 months after the original streptococcal infection.

Mild chorea may produce hyperirritability, a deterioration in handwriting, or an inability to concentrate. Severe chorea causes purposeless, nonrepetitive, involuntary muscle spasms; poor muscle coordination; and weakness. Chorea always resolves without residual neurologic damage.

The most destructive effect of rheumatic fever is carditis, which develops in up to 50% of patients. It may affect the endocardium, myocardium, pericardium, or the heart valves.

Pcricarditis causes a pericardial friction rub and, occasionally, pain and effusion. Myocarditis produces characteristic lesions called Aschoff's bodies (in the acute stages) and cellular swelling and fragmentation of interstitial collagen, leading to formation of a progressively fibrotic nodule and interstitial scars.

Endocarditis causes valve leaflet swelling, erosion along the Jines ofleaflet closure, and blood, platelet, and fibrin deposits, which form beadlike vegetations. Endocarditis affects the mitral valve most often in females; the aortic valve most often in males. In both sexes, endocarditis affects the tricuspid valves occasionally and the pulmonic valve only rarely.

Severe rheumatic carditis may cause heart failure with dyspnea, upper right quadrant pain, tachycardia, tachypnea, significant mitral and aortic murmurs, and a hacking, nonproductive cough.

The most common of such murmurs include a systolic murmur of mitral insufficiency (high-pitched, blowing, holosystolic, loudest at apex, possibly radiating to the anterior axillary line); a midsystolic murmur caused by stiffening and swelling of the mitral leaflet; and, occasionally, a diastolic murmur of aortic insufficiency. Valvular disease may eventually result in chronic valvular stenosis and insufficiency, including mitral stenosis and insufficiency and aortic insufficiency. In children, mitral insufficiency remains the major sequela of rheumatic heart disease.

Diagnosis of Rheumatic Fever and Rheumatic Heart Disease:

Recognition of one or more of the classic symptoms (carditis, polyarthritis, chorea, erythema marginatum, or subcutaneous nodules) and a detailed patient history allow diagnosis,

Supportive laboratory data include the following:

1. White blood cell count and erythrocyte sedimentation rate may be elevated (during the acute phase) and blood studies show slight anemia from suppressed erythropoiesis during inflammation.

2. C-reactive protein is positive (especially during the acute phase).

3. Cardiac enzyme levels may be increased in severe carditis.

4. Antistreptolysin-O titer is elevated in 95% of patients within 2 months of onset.

5. Electrocardiography changes aren't diagnostic, but the PR interval is prolonged in 20% of patients.

6. Chest X-rays show normal heart size (except with myocarditis, heart failure, or pericardial effusion).

7. Echocardiography helps evaluate valvular damage, chamber size, and ventricular function.

8. Cardiac catheterization evaluates valvular damage and left ventricular function in severe cardiac dysfunction.

Several other conditions may have similar signs and symptoms and need to be considered, including systemic lupus erythematosus, sickle cell anemia, leukemia, serum sickness, traumatic arthritis, gonococcal arthritis, systemic juvenile rheumatoid arthritis, and congenital heart disease.

Treatment of Rheumatic Fever and Rheumatic Heart Disease:

Effective management eradicates the streptococcal infection, relieves symptoms, and prevents recurrence, reducing the chance of permanent cardiac damage.

During the acute phase, treatment includes penicillin or (for patients with penicillin hypersensitivity) erythromycin. Salicylates such as aspirin relieve fever and minimize joint swelling and pain; if carditis is present or salicylates fail to relieve pain and inflammation, corticosteroids may be used.

Supportive treatment requires strict bed rest for about 5 weeks during the acute phase with active carditis, followed by a progressive increase in physical activity, depending on clinical and laboratory findings and the response to treatment.

After the acute phase subsides, a monthly I.M. injection of penicillin G benzathine or daily doses of oral sulfadiazine or penicillin G may be used to prevent recurrence, Such preventive treatment usually continues for 5 to 10 years.

Heart failure necessitates continued bed rest and diuretics. Severe milral or aorlic valvular dysfunction causillg persistent heart failure requires correclive valvular surgery, including commissurotomy (separation of the adherent, thickened leaflets of the mitral valve), valvuloplasty (inflation of a balloon within a valve), or valve replacement (with a prosthetic valve). Corrective valvular surgery is rarely necessary before late adolescence.

Special considerations and Prevention Tips:

1. Teach the patient and family about this disease and its treatment.

2. Before giving penicillin, ask the parents if the child has ever had a hypersensitivity reaction to it. Even if the patient has never had a reaction to penicillin, warn that such a reaction is possible, Tell the child's parents to stop the drug and immediately report the development of a rash, fever, chills, or other signs of allergy at any time during penicillin therapy.

3. Instruct the parents to watch for and report early signs of heart failure, such as dyspnea and a hacking, nonproductive cough.

4. Stress the need for bed rest during the acute phase and suggest appropriate, physically undemanding diversions.

5. After the acute phase, encourage family and friends to spend as much time as possible with the child to minimize boredom. Advise the parents to secure a tutor to help the child keep up with schoolwork during his long convalescence.

6. Help the parents overcome any guilt they may feel about their child's illness. Tell them that failure to seek treatment for streptococcal infection is common, because this illness often seems no worse than a cold.

7. If the child has severe carditis, help parents prepare for permanent changes in the child's lifestyle.

8. Warn the parents to watch for and immediately report signs of recurrent streptococcal infection - sudden sore throat,diffuse throat redness and oropharyngeal exudate, swollen and tender cervical lymph glands, pain on swallowing, a temperature of 101 to 104° F (38.3° to 40° C), headache, and nausea, Urge them to keep thc child away from people with respiratory tract infections.

9. Arrange for a visiting nurse to oversee home care if necessary.

Explain the importance of good dental hygiene in preventing gingival infection, which can make the gums prone to bleeding, thus putting the patient at risk for infection. Make sure the child and his family understand the need to comply with prolonged antibiotic therapy and follow­up care and the need for additional antibiotics for dental or oral surgical procedures likely to cause gingival bleeding, for upper respiratory tract surgery, and for surgery or instrumentation of the GU and lower GI tracts.