Rheumatic fever and rheumatic heart disease
Acute rheumatic fever is a systemic inflammatory disease of childhood, in many cases recurrent, that follows a group A beta-hemolytic 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 the 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 and incidence
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. Because very few persons (3%) with streptococcal infections ever contract rheumatic fever, altered host resistance must be involved in its development or recurrence. Although rheumatic fever tends to be familial, 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 generally during cool, damp weather in the 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, and 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, a nonpruritic, macular, transient rash that gives rise to red lesions with blanched centers. Rheumatic fever may also produce firm, movable, nontender, 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 (Sydenham’s 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 and 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 affects the mitral valve most often in females; the aortic, most often in males. In both females and males, endocarditis affects the tricuspid valves occasionally and the pulmonic only rarely.
Severe rheumatic carditis may cause heart failure with dyspnea; right upper quadrant pain; tachycardia; tachypnea; a hacking, nonproductive cough; edema; and significant mitral and aortic murmurs. 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 due to stiffening and swelling of the mitral leaflet
❑ occasionally, a diastolic murmur of aortic insufficiency (low-pitched, rumbling, almost inaudible). 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
Diagnosis depends on recognition of one or more of the classic symptoms (carditis, rheumatic fever without carditis, polyarthritis, chorea, erythema marginatum, or subcutaneous nodules) and a detailed patient history. 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 due to suppressed erythropoiesis during inflammation.
❑ C-reactive protein is positive (especially during acute phase).
❑ Cardiac enzyme levels may be increased in severe carditis.
❑ Antistreptolysin-O titer is elevated in 95% of patients within 2 months of onset.
❑ Electrocardiogram changes aren’t diagnostic; but 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 severe cardiac dysfunction.
Treatment
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, sulfadiazine, or 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, low-dose antibiotics may be used to prevent recurrence. Such preventive treatment usually continues for 5 years or until age 21 (whichever is longer). Heart failure necessitates continued bed rest and diuretics. Severe mitral or aortic valve dysfunction that causes 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 prosthetic valve). Such surgery is seldom necessary before late adolescence.
Special considerations
Because rheumatic fever and rheumatic heart disease require prolonged treatment, the care plan should include comprehensive patient teaching to promote compliance with the prescribed therapy.
❑ Before giving penicillin, ask the patient or his parents if he has ever had a hypersensitive reaction to it. If he hasn’t, warn that such a reaction is possible. Tell them to stop the drug and call the physician immediately if he develops a rash, fever, chills, or other signs of allergy at any time during penicillin therapy.
❑ Instruct the patient and his family 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 his family and friends to spend as much time as possible with the patient to minimize boredom. Advise his parents to secure tutorial services to help the child keep up with schoolwork during the long convalescence.
❑ Help his parents overcome any guilt feelings they may have 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. Encourage the child and his parents to vent their frustrations during the long, tedious recovery. If the child has severe carditis, help them prepare for permanent changes in his lifestyle.
❑ Teach the patient and his family about this disease and its treatment. Warn 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, 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.
❑ Promote good dental hygiene to prevent gingival infection. Make sure the patient 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 or procedures. Arrange for a home health nurse to oversee home care if necessary.
❑ Teach the patient to follow current recommendations of the American Heart Association for prevention of bacterial endocarditis. Antibiotic regimens used to prevent recurrence of acute rheumatic fever are inadequate for preventing bacterial endocarditis.
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2008 Williams & Wilkins.
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