eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Rheumatic Heart Disease: Treatment & Medication
Updated: Oct 10, 2008
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Treatment
Medical Care
Medical therapy in rheumatic heart disease is directed toward eliminating the group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment for congestive heart failure. Group A streptococcal vaccines are still years away from being available. Oral penicillin V remains the drug of choice for treatment of group A streptococcal pharyngitis. When oral penicillin is not feasible or dependable, a single dose of intramuscular benzathine penicillin G is therapeutic. For patients who are allergic to penicillin, administer erythromycin or a first-generation cephalosporin. Other options include clarithromycin for 10 days, azithromycin for 5 days, or a narrow-spectrum (first-generation) cephalosporin for 10 days. As many as 15% of penicillin-allergic patients also are allergic to cephalosporins. Tetracyclines and sulfonamides should not be used to treat group A streptococcal pharyngitis.
- For recurrent group A streptococcal pharyngitis, a second 10-day course of the same antibiotic can be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides.
- In general, antimicrobial therapy is not indicated for pharyngeal carriers of group A Streptococcus. Exceptions include the following:
- Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis
- Family history of rheumatic fever
- Outbreaks of group A streptococcal pharyngitis in a closed community
- When considering tonsillectomy for chronic group A streptococcal carriage
- Multiple episodes of documented group A streptococcal pharyngitis within a family despite appropriate therapy
- Following group A streptococcal toxic shock syndrome or necrotizing fasciitis in a household contact
- Carriage is difficult to eradicate with conventional penicillin therapy. Thus, oral clindamycin (20 mg/kg/d in 3 divided doses for 10 days) is recommended.
- Treatment of the acute inflammatory manifestations of acute rheumatic fever consists of administering salicylates and steroids. Aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea, and the response is typically dramatic. In fact, if rapid improvement is not seen after 24-36 hours of therapy, the diagnosis of rheumatic fever should be questioned.
- Attempts are made to obtain aspirin blood levels at 20-25 mg/dL; however, due to variable GI absorption of the drug, stable levels may be difficult to achieve during the inflammatory phase. Aspirin is maintained at anti-inflammatory doses until the signs and symptoms of acute rheumatic fever are resolved or subsiding (6-8 wk) and the acute phase reactants have returned to normal levels.
- Anti-inflammatory doses of aspirin may be associated with abnormal liver function test findings and GI toxicity; adjusting the aspirin dosage may be necessary. When discontinuing therapy, aspirin should be gradually withdrawn over weeks while monitoring the acute phase reactants for evidence of rebound.
- If moderate-to-severe carditis is indicated by cardiomegaly, congestive heart failure, or third-degree heart block, oral prednisone should be added to salicylate therapy.
- Prednisone should be continued for 2-6 weeks, depending on the severity of the carditis, and tapered during the last week of therapy.
- Adverse effects can be minimized by discontinuing prednisone therapy after 2-4 weeks and maintaining salicylates for an additional 2-4 weeks.
- Additional treatment for patients with acute rheumatic fever and congestive heart failure should include digoxin, diuretics, supplemental oxygen, bed rest, and sodium and fluid restriction.
- Digoxin should be initiated only after checking electrolyte values and correcting abnormal findings in serum potassium levels. The total loading dose is 20-30 mcg/kg orally with 50% of the dose given initially, followed by 25% of the dose 8 and 16 hours after the initial dose.
- Maintenance doses are typically 8-10 mcg/kg/d orally in 2 divided doses. For older children and adults, the total loading dose is 1.25-1.5 mg orally, and the maintenance dose is 0.25-0.5 mg/d orally.
- Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL.
- The diuretics most commonly used in conjunction with digoxin for children with congestive heart failure include furosemide and spironolactone, both at doses of 1-2 mg/kg/dose twice per day.
- In patients with moderate-to-severe mitral valve regurgitation, enalapril therapy was associated with significant reduction in left ventricular diameter and mitral regurgitation volume.
- Surgery is indicated to decrease valve insufficiency when heart failure persists or worsens during the acute phase after aggressive medical therapy. Mitral valve repair (posterior collar annuloplasty, commissurotomy, cusp level chordal shortening, cusp thinning, cleft suture, and cusp excision or plication) has also been shown to be feasible in children with chronic rheumatic mitral valve disease.
- Preventive and prophylactic therapy is indicated after rheumatic fever and rheumatic heart disease to prevent further damage to valves. The initial course of antibiotics given to eradicate the streptococcal infection also serves as the first course of prophylaxis. An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary prevention for most patients in the United States. The same dosage should be given for 3 weeks in areas where rheumatic fever is endemic, in patients with residual carditis, and in high-risk patients.
- Although oral penicillin prophylaxis is also effective, data from the World Health Organization suggest that the recurrence risk for group A streptococcal pharyngitis is lower when penicillin is parentally administered.
- The duration of antibiotic prophylaxis is controversial. Antibiotic prophylaxis should be continued indefinitely for patients at high risk (eg, health care workers, teachers, daycare workers) for recurrent group A streptococcal infection.
- Ideally, prophylaxis should be continued indefinitely because recurrent group A streptococcal infection and rheumatic fever are possible at any age.
- However, the American Heart Association currently recommends that patients with rheumatic fever without carditis receive prophylactic antibiotics for 5 years, or until age 21 years, whichever is longer.4
- Patients with rheumatic fever and carditis but no valve disease should receive prophylactic antibiotics for 10 years or well into adulthood, whichever is longer.
- Finally, patients with rheumatic fever and carditis and valve disease should receive antibiotics at least 10 years or until age 40 years.
- Patients with rheumatic heart disease require antibiotic prophylaxis before certain surgical and dental procedures to prevent bacterial endocarditis. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.5,6 Patients who have had rheumatic fever without valve disease do not need prophylaxis for prevention of endocarditis. Penicillin should not be used for prophylaxis of endocarditis in patients who are receiving secondary rheumatic fever prophylaxis because of relative resistance to penicillin. The recommended alternative for these patients is erythromycin.
Surgical Care
When heart failure persists or worsens after aggressive medical therapy for acute rheumatic heart disease, surgery to decrease valve insufficiency may be life-saving.
- Forty percent of patients with acute rheumatic fever subsequently develop mitral stenosis as adults.
- In patients with critical stenosis, mitral valvulotomy, percutaneous balloon valvuloplasty, or mitral valve replacement may be indicated.
- Due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve replacement appears to be the preferred surgical option.
Diet
The diet should be nutritious and without restrictions except in the patient with congestive heart failure, whose fluid and sodium intake should be restricted. Potassium supplementation may be necessary because of the mineralocorticoid effect of corticosteroid and the diuretics (if used).
Activity
Initially, patients should be placed on bed rest followed by a period of indoor activity before being permitted to return to school. Full activity should not be allowed until the acute phase reactants have returned to normal levels.
Medication
Medical therapy is directed at eliminating the group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment for congestive heart failure. The treatment and prevention of group A streptococcal pharyngitis outlined here is based on the current recommendations of the Committee on Infectious Disease (American Academy of Pediatrics). See the eMedicine article Pharyngitis.
Penicillin V is the drug of choice for treatment of group A streptococcal pharyngitis. Ampicillin or amoxicillin may be used instead of penicillin V but have no microbiologic advantage. Tetracyclines and sulfonamides should not be used to treat group A streptococcal pharyngitis. For recurrent group A streptococcal pharyngitis, a second 10-day course of the same antibiotic can be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides for 10 d. As many as 15% of patients allergic to penicillin are also allergic to cephalosporins.
Antibiotics
Antibiotics are used for the initial treatment of group A streptococcal pharyngitis to prevent the first attack of rheumatic fever (primary prophylaxis), for recurrent streptococcal pharyngitis, and for continuous therapy to prevent recurrent rheumatic fever and rheumatic heart disease (secondary prophylaxis).
Penicillin VK (Beepen-VK, Betapen-VK, Pen-Vee K)
DOC for treatment of group A streptococcal pharyngitis. Inhibits the biosynthesis of cell wall mucopeptide. Bactericidal against sensitive organisms when adequate concentrations are reached, and most effective during the stage of active multiplication. Inadequate concentrations may produce only bacteriostatic effects.
Adult
500 mg PO bid/tid for 10 d
Pediatric
Children: 250 mg (400,000 U) PO bid/tid for 10 d
Adolescents: Administer as in adults
Probenecid may increase effectiveness by decreasing clearance; tetracyclines are bacteriostatic, causing a decrease in the effectiveness of penicillins when administered concurrently
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in renal impairment; administer 1 h ac or 2 h pc
Penicillin, benzathine (Bicillin L-A) and procaine (Wycillin)
Used when PO administration of penicillin is not feasible or dependable. Discomfort of IM injection may be minimized if the penicillin G is brought to room temperature before injection or if a combination of benzathine penicillin G and procaine penicillin G (Bicillin CR) is used. Initial course of antibiotics given to eradicate the streptococcal infection also serves as the first course of prophylaxis. Benzathine penicillin G IM q4wk is recommended for secondary prevention for most United States patients. The same dosage should be used q3wk in areas where rheumatic fever is endemic, in patients with residual carditis, and in patients with high risk.
Adult
1.2 million U of benzathine penicillin G or a combination of 900,000 U of benzathine penicillin G with 300,000 U of procaine penicillin G single dose IM to eradicate streptococcal infection; for secondary prevention of rheumatic fever, administer the above dose q3wk (high-risk areas or patients) or q4wk (most areas in United States)
Pediatric
<27 kg: 600,000 U of benzathine penicillin G single dose IM to eradicate the streptococcal infection; for secondary prevention of rheumatic fever, administer the above dose q3wk (high-risk areas or patients) or q4wk (most areas in United States)
>27 kg: Administer as in adults
Increases risk of bleeding when administered concurrently with warfarin; ethacrynic acid, aspirin, indomethacin, and furosemide may compete with penicillin G for renal tubular secretion increasing penicillin serum concentrations
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Never use IV route to administer penicillin G procaine; perform cultures after treatment to confirm streptococci eradication
Erythromycin estolate (Ilosone) or ethyl succinate (E.E.S, EryPed)
Used to treat patients allergic to penicillin. Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest.
Adult
1 g/d PO divided bid/qid for 10 d
Pediatric
20-40 mg/kg/d PO divided bid/qid for 10 d
Coadministration may increase toxicity of theophylline, digoxin, carbamazepine, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin, increases risk of rhabdomyolysis
Documented hypersensitivity; hepatic impairment
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur
Anti-inflammatory agents
The manifestations of acute rheumatic fever (including carditis) typically respond rapidly to therapy with anti-inflammatory agents. Aspirin, in anti-inflammatory doses, is the drug of choice. Prednisone is added when evidence of worsening carditis and heart failure is noted.
Aspirin (Anacin, Ascriptin, Bayer Aspirin)
Also called acetylsalicylic acid. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. Start immediately after the diagnosis of rheumatic fever has been made. Initiation of therapy may mask manifestations of the disease.
Adult
4-8 g/d PO in 4-6 divided doses; maintain salicylate serum levels in a 20-25 mcg/dL range until all symptoms resolve and the acute phase reactants return to normal values
Pediatric
80-100 mg/kg/d PO in 4-6 divided doses; maintain salicylate serum levels in a 20-25 mcg/dL range until all symptoms resolve and the acute phase reactants return to normal values
Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, asthma; because of association of aspirin with Reye syndrome, do not use in children (<16 y) with flu
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Pregnancy category D in third trimester; risk of salicylate intoxication and poisoning; watch for hyperventilation with prolonged expiratory phase with respiratory alkalosis and metabolic acidosis; risk of tinnitus, hepatic dysfunction, GI discomfort, and ulceration; taken during pregnancy increases the risk of pulmonary hypertension in the neonate
Prednisone (Deltasone, Orasone)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. If moderate to severe carditis is indicated by cardiomegaly, congestive heart failure, or third-degree heart block, 2 mg/kg/d PO should be used in addition to, or in lieu of, salicylate therapy. Prednisone should be continued for 2-4 wk, depending on the severity of the carditis, and tapered during the last week of therapy. Adverse effects can be minimized by discontinuing prednisone therapy after 2 wk and adding or maintaining salicylates for an additional 2-4 wk.
Adult
2 mg/kg/d PO for 2-4 wk
Pediatric
Administer as in adults
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI ulceration or bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Angiotensin-converting enzyme (ACE) inhibitors
These agents are competitive inhibitors of ACE. They reduce angiotensin II levels and thus decrease aldosterone secretion.
Enalapril (Vasotec)
Indicated for chronic aortic and/or mitral regurgitation. Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased plasma renin levels and a reduction in aldosterone secretion. Helps control blood pressure and proteinuria. Decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance. Has favorable clinical effect when administered over a long period. Helps prevent potassium loss in distal tubules. Body conserves potassium; thus, less oral potassium supplementation needed. Goal is to decrease afterload to left ventricle (by reducing systemic blood pressure and by peripheral vasodilatation).
Adult
2.5 mg PO bid initially; therapeutic range within 2.5-20 mg/d in 2 divided doses; not to exceed 40 mg/d
Pediatric
0.1 mg/kg PO bid/qid; not to exceed 40 mg/d
Alternatively, 5-10 mcg/kg IV qid, infuse slowly over 5 min
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment, valvular stenosis, or severe congestive heart failure
More on Rheumatic Heart Disease |
| Overview: Rheumatic Heart Disease |
| Differential Diagnoses & Workup: Rheumatic Heart Disease |
 Treatment & Medication: Rheumatic Heart Disease |
| Follow-up: Rheumatic Heart Disease |
| Multimedia: Rheumatic Heart Disease |
| References |
| « Previous Page | Next Page » |
References
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Further Reading
Keywords
rheumatic heart disease, acute rheumatic carditis, rheumatic fever, pharyngitis, heart failure, valve insufficiency, pericarditis, chronic rheumatic heart disease, valve stenosis, regurgitation, atrial dilation, arrhythmias, ventricular dysfunction, mitral valve stenosis, valve replacement, pancarditis, group A beta-hemolytic Streptococcus, Streptococcus pyogenes, impetigo, cellulitis, myositis, pneumonia, sepsis, endocarditis, myocarditis, polyarthritis, chorea, subcutaneous nodules, erythema marginatum, chest pain, edema, orthopnea, mitral insufficiency, congestive heart failure, Carey-Coombs murmur, hepatomegaly, arthralgias, epistaxis, Huntington chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, PANDAS, Sydenham chorea, aortic stenosis
