Pediatric Rheumatic Heart Disease Treatment & Management

Medical therapy in rheumatic heart disease includes attempts to prevent rheumatic fever (and thus rheumatic heart disease). In patients who develop rheumatic heart disease, therapy 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. Following the resolution of the acute episode, subsequent therapy is directed towards preventing recurrent rheumatic heart disease in children and monitoring for the complications and sequelae of chronic rheumatic heart disease in adults.

Prevention of rheumatic fever in patients with group A beta hemolytic streptococci (GABHS) pharyngitis

For patients with GABHS pharyngitis, a meta-analysis supports a protective effect against rheumatic fever when penicillin is used following the diagnosis.^[5]

Oral (PO) penicillin V remains the drug of choice for treatment of GABHS pharyngitis, but ampicillin and amoxicillin are equally effective.

When PO penicillin is not feasible or dependable, a single dose of intramuscular benzathine penicillin G or benzathine/procaine penicillin combination 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 patients who are allergic to penicillin are also allergic to cephalosporins.

Do not use tetracyclines or sulfonamides to treat GABHS pharyngitis.

For recurrent group A streptococci (GAS) pharyngitis, a second 10-day course of the same antibiotic may be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides.

Control measures for patients with GABHS pharyngitis are as follows:

• Hospitalized patients: Place hospitalized patients with GABHS pharyngitis of pneumonia on droplet precautions, as well as standard precautions, until 24 hours after initiation of appropriate antibiotics.
• Exposed persons: People in contact with patients having documented cases of streptococcal infection first should undergo appropriate laboratory testing if they have clinical evidence of GABHS infection and should undergo antibiotic therapy if infected.
• School and childcare centers: Children with GABHS infection should not attend school or childcare centers for the first 24 hours after initiating antimicrobial therapy.

GABHS carriage is difficult to eradicate with conventional penicillin therapy. Thus, PO clindamycin (20 mg/kg/d PO in 3 divided doses for 10 days) is recommended.

In general, antimicrobial therapy is not indicated for pharyngeal carriers of GABHS. Exceptions include the following:

• Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis
• Family history of rheumatic fever
• During outbreaks of GAS pharyngitis in a closed community
• When tonsillectomy is considered for chronic GABHS carriage
• When multiple episodes of documented GABHS pharyngitis occur within a family despite appropriate therapy
• Following GAS toxic shock syndrome or necrotizing fasciitis in a household contact

Treatment for patients with rheumatic fever and rheumatic heart disease

Therapy is directed towards eliminating the GABHS pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment of congestive heart failure.

Treat residual GABHS pharyngitis as outlined above, if still present.

Treatment of the acute inflammatory manifestations of acute rheumatic fever consists of salicylates and steroids. Aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea, and the response is typically dramatic.

If rapid improvement is not observed after 24-36 hours of therapy, question the diagnosis of rheumatic fever.

Attempt to obtain aspirin blood levels from 20-25 mg/dL, but stable levels may be difficult to achieve during the inflammatory phase because of variable GI absorption of the drug. Maintain aspirin at anti-inflammatory doses until the signs and symptoms of acute rheumatic fever are resolved or residing (6-8 wk) and the acute phase reactants (APRs) have returned to normal.

Anti-inflammatory doses of aspirin may be associated with abnormal liver function tests and GI toxicity, and adjusting the aspirin dosage may be necessary.

When discontinuing therapy, withdraw aspirin gradually over weeks while monitoring the APRs for evidence of rebound. Chorea is most frequently self-limited but may be alleviated or partially controlled with phenobarbital or diazepam.

If moderate to severe carditis is present as indicated by cardiomegaly, third-degree heart block or congestive heart failure, substitute PO prednisone for salicylate therapy. Continue prednisone for 2-6 weeks depending on the severity of the carditis, and taper prednisone during the final week(s) of therapy. Weaning prednisone therapy after a shorter period (2-4 weeks) while initiating and maintaining salicylates for several weeks can minimize adverse effects of the steroids while preventing rebound of the carditis.

Include digoxin and diuretics, afterload reduction, supplemental oxygen, bed rest, and sodium and fluid restriction as additional treatment for patients with acute rheumatic fever and heart failure. The diuretics most commonly used in conjunction with digoxin for children with heart failure include furosemide and spironolactone. Initiate digoxin only after checking electrolytes and correcting hypokalemia.

The total digitalizing dose is 20-30 mcg/kg PO, with 50% of the dose administered initially, followed by 25% of the dose 12 hours and 24 hours after the initial dose. Maintenance doses typically are 8-10 mcg/kg/d PO in 2 divided doses. For older children and adults, the total loading dose is 1.25-1.5 mg PO, and the maintenance dose is 0.25-0.5 mg PO every day. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL.

Afterload reduction (ie, using ACE inhibitor captopril) may be effective in improving cardiac output, particularly in the presence of mitral and aortic insufficiency. Start these agents judiciously. Use a small, initial test dose (some patients have an abnormally large response to these agents), and administer only after correcting hypovolemia.

When heart failure persists or progresses during an episode of acute rheumatic fever in spite of aggressive medical therapy, surgery is indicated and may be life-saving for severe mitral and/or aortic insufficiency.

Treatment for patients following rheumatic heart disease (RHD)

Preventive and prophylactic therapy is indicated after rheumatic fever and acute rheumatic heart disease to prevent further damage to valves.

Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as the first course of secondary prophylaxis (prevention of recurrent rheumatic fever and rheumatic heart disease).

An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary prophylaxis for most US patients. Administer the same dosage every 3 weeks in areas where rheumatic fever is endemic, in patients with residual carditis, and in high-risk patients.

Although PO penicillin prophylaxis is also effective, data from the World Health Organization indicate that the recurrence risk of GABHS pharyngitis is lower when penicillin is administered parentally.

The duration of antibiotic prophylaxis is controversial. Continue antibiotic prophylaxis indefinitely for patients at high risk (eg, health care workers, teachers, daycare workers) for recurrent GABHS infection. Ideally, one could argue for continuing prophylaxis indefinitely, because recurrent GABHS infection and rheumatic fever can occur 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 aged 21 years, whichever is longer.^[7] 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 with carditis and valve disease should receive antibiotics for at least 10 years or until age 40 years.

Patients with rheumatic heart disease and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis. Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis. Do not use penicillin, ampicillin, or amoxicillin for endocarditis prophylaxis in patients already receiving penicillin for secondary rheumatic fever prophylaxis (relative resistance of PO streptococci to penicillin and aminopenicillins). Alternate drugs recommended by the American Heart Association for these patients include PO clindamycin (20 mg/kg in children, 600 mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in children, 500 mg in adults). The guidelines for endocarditis prophylaxis in patients with valve damage from rheumatic heart disease have changed. Antibiotic prophylaxis is no longer recommended.^[7]

A recent study investigated the difference in clinical manifestations and outcomes between first episode and recurrent rheumatic fever.^[8] The study concluded that subclinical carditis occurred only in patients experiencing the first episode, and that all deaths occurred in patients with recurrent rheumatic fever, emphasizing the need for secondary prophylaxis.

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 heart disease 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.

In addition to cardiology consultation, complications may require cardiothoracic surgery consultation (heart failure and progressive valve insufficiency) and neurology consultation (chorea, PANDAS).

The diet should be nutritious and without restrictions except in the patient with congestive heart failure. In these patients, fluid and sodium intake should be restricted. Potassium supplementation may be necessary if steroids or diuretics are used.

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.