Rheumatic fever and rheumatic heart disease

Rheumatic fever and rheumatic heart disease: Excerpt from Handbook of Diseases

Often recurrent, acute rheumatic fever is a systemic inflammatory disease of childhood that follows a group A beta-hemolytic streptococcal infection. Rheumatic heart disease refers to the cardiac manifestations of rheumatic fever, including 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.

Causes

Rheumatic fever appears to be a hypersensitivity reaction to a group A beta-hemolytic streptococcal infection, in which antibodies manufactured to combat streptococci react and produce characteristic lesions at specific tissue sites, especially in the heart and joints. About 3% of patients with untreated streptococcal infections develop rheumatic fever.

Although rheumatic fever tends to run in families, this may merely reflect contributing environmental factors. It primarily affects children between ages 6 and 15, usually within 1 to 5 weeks after strep throat or scarlet fever. The disease strikes most often during cool, damp weather in winter and early spring. In the United States, it’s most common in the northern states.

Signs and symptoms

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.

Joint pain

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.

Skin lesions and nodules

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

Rheumatic fever may also produce firm, movable, nontender, subcutaneous nodules ⅛" to ¾" (0.5 to 2 cm) in diameter, usually near tendons or bony prominences of joints (especially the elbows, knuckles, wrists, and knees) and less commonly 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.

Chorea

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.

Carditis

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.

Pericarditis causes a pericardial friction rub and, occasionally, pain and effusion. Myocarditis produces characteristic lesions called Aschoff 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 lines of leaflet closure, and blood, platelet, and fibrin deposits, which form beadlike vegetations. Endocarditis usually affects the mitral valve in females and the aortic valve 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, right-upper-quadrant pain, tachycardia, tachypnea, significant mitral and aortic murmurs, and a hacking, nonproductive cough.

The most common murmurs include:

❑ a systolic murmur of mitral insufficiency (high-pitched, blowing, holo-systolic, loudest at apex, possibly radiating to the anterior axillary line)

❑ a midsystolic murmur caused by stiffening and swelling of the mitral leaflet

❑ 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 after-effect of rheumatic heart disease.

Diagnosis

Recognition of one or more classic signs or symptoms (carditis, polyarthritis, chorea, erythema marginatum, or subcutaneous nodules) and a detailed patient history allow diagnosis. The following laboratory data support the diagnosis:

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

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

❑ Cardiac enzyme levels may be increased in those with severe carditis.

❑ Antistreptolysin O titer is elevated in 95% of patients within 2 months of onset. (Rising antiDNase B test results can also detect recurrent streptococcal infection.)

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

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

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

❑ Cardiac catheterization evaluates valvular damage and left ventricular function in those with severe cardiac dysfunction.

Treatment

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

Treatment in acute phase

During the acute phase, treatment includes low doses of antibiotics, such as penicillin, sulfadiazine, or erythro-mycin. Salicylates, such as aspirin, can help relieve fever and minimize joint swelling and pain; if carditis is present or the salicylate fails 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 patient’s response to treatment.

Preventive treatment

After the acute phase subsides, the patient is maintained on low-dose antibiotic therapy, especially during the first 3 to 5 years after the initial episode of rheumatic fever, to prevent recurrence. Such preventive treatment usually continues for 5 to 10 years.

Surgery and other measures

Heart failure necessitates continued bed rest and diuretic therapy. Severe mitral or aortic valvular dysfunction causing persistent heart failure requires corrective 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

❑ Teach the patient and his family about this disease and its treatment.

❑ Before giving penicillin, ask the parents whether 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 parents that if the child develops a rash, fever, chills, or other signs or symptoms of allergy at any time during penicillin therapy, they should  stop the drug and immediately contact the physician.

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

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

❑ 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.

❑ Tell the parents that failure to seek treatment for streptococcal infection is common, because the illness may seem no worse than a cold.

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

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

CLINICAL TIP: Explain the importance of good dental hygiene in preventing gingival infection.

❑ Make sure the child and his family understand the need to comply with prolonged antibiotic therapy and follow-up care and the need for additional antibiotics during dental surgery.

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

Book Source Details

• Book Title: Handbook of Diseases
• Author(s): Springhouse
• Year of Publication: 2003
• Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.

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More About This Book: Title: Handbook of Diseases Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2003 ISBN: 1-58255-266-5

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