Pediatric Rheumatic Heart Disease Treatment & Management

Updated: Dec 01, 2019
  • Author: Thomas K Chin, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Treatment

Medical Care

As of 2007, the American Heart Association no longer recommends subacute bacterial endocardial prophylaxis in patients with aortic or mitral valve abnormalities secondary to rheumatic heart disease. [42, 43]

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 (GAS) pharyngitis if still present and providing supportive treatment for congestive heart failure. Following resolution of the acute episode, subsequent therapy is directed toward 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

Patients who have positive rapid-antigen testing or throat culture for GAS in the setting of symptomatic pharyngitis should be treated with appropriate antibiotics. For patients with GABHS pharyngitis, a meta-analysis supports a protective effect against rheumatic fever when penicillin is used following the diagnosis. [44]

At this time, oral (PO) penicillin V (250 mg twice [BID] or thrice daily [TID] if < 27 kg and 500 mg BID or TID if >27 kg) is the drug of choice for treatment of GABHS pharyngitis. Amoxicillin (50 mg/kg/day; maximum of 1000 mg/day) is an acceptable alternative and has the benefit of once-daily dosing. Another option is a single dose of intramuscular (IM) benzathine penicillin G (0.6 million units if < 27 kg or 1.2 million units if >27 kg) or a benzathine/procaine penicillin combination. This option ensures adherence, and because the noncompliance rates for 10 days of PO penicillin are high, some clinicians recommend this choice. Furthermore, benzathine penicillin G is the only drug studied for the prevention of acute rheumatic fever.

For patients with a mild penicillin allergy, cephalosporins can be used, typically given for a 10-day course, and demonstrate a high efficacy rate. Options include cephalexin, cefuroxime, cefpodoxime, and cefdinir. In children with more severe reactions to penicillin, including anaphylaxis or other immunoglobulin (Ig)E-mediated reaction, macrolides (azithromycin for a 5-day course or clarithromycin for a 10-day course) can be used. In instances of severe reaction to penicillins where there is concominant concern for macrolide resistance, clindamycin (10-day course) is another alternative therapy. Do not use tetracyclines or sulfonamides to treat GABHS pharyngitis.

For recurrent GAS pharyngitis, a detailed history will help to guide further management. Other etiologies such as alternative bacterial sources and viruses should be considered. Generally, a second, repeat 10-day course of the same antibiotic may be used. If the cause of symptoms is suspected to be due to treatment failure from noncompliance, the single IM injection of benzathine penicillin G may be a good option. If choosing an alternative antibiotic, one with improved beta-lactamase activity relative to the original antibiotic is preferred, such as amoxicillin-clavulanate or a later-generation cephalosporin. 

Control measures for patients with GABHS pharyngitis

  • Hospitalized patients: Place hospitalized patients with GABHS pharyngitis or pneumonia on droplet precautions, as well as standard precautions, until 24 hours after the 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; if infected, these individuals should undergo antibiotic therapy.
  • School and childcare centers: Children with GABHS infection should not attend school or childcare centers for the first 24 hours after the initiation of antimicrobial therapy.

Chronic carriers of GABHS

In general, animicrobal therapy is is not indicated in chronic GABHS carriers. These patients are unlikely to develop complications, specifically acute rheumatic fever. [45]  Exceptions to this include the following:

  • Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis
  • Family history of rheumatic fever
  • During outbreaks of GAS pharyngitis in a closed community or when multiple episodes of documented GABHS pharyngitis occur within a family despite appropriate therapy
  • When tonsillectomy is considered for chronic GABHS carriage
  • Following GAS toxic shock syndrome or necrotizing fasciitis in a household contact
  • GABHS carriage is difficult to eradicate. Options include clindamycin, amoxicillin-clavulanate, and penicillin + rifampin (rifampin given only last 4 days of therapy).

Treatment for patients with rheumatic fever and rheumatic heart disease

Therapy is directed toward eliminating the GABHS pharyngitis (if still present) and providing supportive treatment of congestive heart failure.

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

Previously, treatment of the acute inflammatory manifestations of acute rheumatic fever included salicylates, nonsteroidal anti-inflammatory agents (NSAIDs), steroids, and intravenous immunoglobulin (IVIG). However, current data do not show improved cardiac outcomes with the use of these interventions. [46]  Thus, these agents are not recommended for the treatment of carditis associated with acute rheumatic fever. However, some of these agents may be of benefit for the arthritis associated with acute rheumatric fever.

In certain instances of severe carditis associated with acute rheumatic fever, glucocorticoids such as prednisone (1-2 mg/kg/day with a maximum of 80 mg) can be used. Oftentimes, these patients have extremely elevated inflammatory markers. Typically, if a patient is also taking aspirin or other NSAIDs, these medications are stopped once glucocorticoids are initiated and then resumed once the course is completed. It is always important to remember to gradually taper the steroid dose if needed for a prolonged period to prevent adrenal insufficiency. 

Patients with heart failure due to acute rheumatic fever should be treated appropriately. Include the use of digoxin and diuretics, afterload reduction, supplemental oxygen, bed rest, and sodium and fluid restriction.

Loop diuretics (furosemide) are the most commonly used diuretics in children. Thiazide diuretics such as chlorothiazide can be used in conjunction with loop diuretics. Spironolactone (a mineralocorticoid receptor antagonist) can also be added for further diuresis, specifically in patients with hypokalemia. 

Afterload reduction with an agent such as an angiotensin-converting enzyme (ACE)-inhibitor 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 them only after correcting hypovolemia. Angiotensin-receptor blockers (ARBs) are an alternative to ACE-inhibitors in patients who cannot tolerate them; however, data are limited in the pediatric population for this class of medication.

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.

Prophylaxis for patients following rheumatic heart disease)

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

Primary prevention of rheumatic fever consists of prompt diagnosis and treatment of GABHS pharyngitis.

Secondary prevention of acute rheumatic fever is very important, as patients who have had acute rheumatic fever are at high risk for recurrence. [86] Furthermore, rheumatic heart disease becomes more severe with each recurrent episode. Some studies have shown that recurrence of acute rheumatic fever can result from nonsymptomatic episodes of GAS pharyngitis. [47]  Given this, any patients with a history of acute rheumatic fever and evidence of rheumatic heart disease should receive continuous antibiotic prophylaxis.

The preferred medication for secondary prophylaxis of acute rheumatic fever is an injection of 0.6-1.2 million units (dosing based upon weight) of IM benzathine penicillin G every 4 weeks. 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. [2] Other studies evaluting parenteral versus PO antibiotics as secondary prophylaxis have demonstrated similar results. [48]  Some oral alternatives include penicillin V (preferred PO agent, dosed at 250 mg BID), macrolides,and sulfadiazine. If a penicillin allergy has been confirmed in a patient, azithromycin 250 mg or 5 mg/kg once daily is usually the preferred agent used.

The duration of antibiotic prophylaxis is controversial. Continue antibiotic prophylaxis indefinitely for patients at high risk (eg, healthcare 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 recommends that patients with rheumatic fever without carditis receive prophylactic antibiotics for 5 years or until aged 21 years, whichever is longer. [42] 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.

A study that investigated the difference in clinical manifestations and outcomes between first episode and recurrent rheumatic fever 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. [49]  However, an interesting study by Hand et al demonstrated few patients achieved serum concentrations above 0.02 mg/L of benzylpenicillin G for the majority of the time between injections given as secondary prophylaxis. [50] This suggests there is a gap in our current understanding of the true effect of benzylpenicillin G and its associated clinical outcomes seen in rheumatic heart disease.

As noted earlier, although patients with rheumatic heart disease and valve damage previously required empiric antibiotics prior to surgical and dental procedures to help prevent bacterial endocarditis, this is no longer the recommendation. [42, 43] Based upon updated guidelines from the American Heart Association, only a limited number of cardiac conditions now require antibiotic prophlyaxis prior to dental procedures. [42, 43] Patients with rheumatic heart disease and valve damage do not require prophylaxis at this time.

Consultations

In addition to cardiology consultation, complications may require cardiothoracic surgery consultation (heart failure and progressive valve insufficiency) and neurology consultation (chorea, PANDAS [pediatric autoimmune neuropsychiatric disorders associated with streptococcal throat infections]).

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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.

About 40% 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. The rheumatic heart valve surgery score (RheSCORE) model has been demonstrated to outperform other scoring systems in predicting hospital mortality in patients referred for surgical management of rheumatic heart disease. [51]

Percutaneous balloon mitral valvuloplasty using the Inoue balloon, initially described in 1984, [52] appears to produce good results and has been extensively used in countries with a high incidence of rheumatic fever. The more recently described percutaneously implantable mitral clip may be useful in selected cases of mitral insufficiency; further studies are needed to confirm the utility in rheumatic mitral insufficiency. [53, 54, 55, 56]  

In the past, due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve replacement appeared to be the preferred surgical option for patients with high rates of recurrent symptoms after annuloplasty or other repair procedures. However, modifications of standard repair techniques, adherence to the importance of good leaflet coaptation, and strict quality control with stringent use of intraoperative transesophageal echocardiography have all contributed to improved long-term results. [57]

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Diet and Activity

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 levels of acute phase reactants have returned to normal. Patients with chorea may require a wheelchair and should be on homebound instruction until the abnormal movements resolve.

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Long-Term Monitoring

Patients with rheumatic fever usually show significant improvement after the initiation of anti-inflammatory therapy. However, they should not be allowed to resume full activities until all clinical symptoms have abated and laboratory values have returned to normal levels.

Emphasize the importance of prophylaxis against recurrent streptococcal pharyngitis and rheumatic fever with each patient. Each recurrent episode of rheumatic carditis produces further valve damage and increases the likelihood that valve replacement will be required. Patients should remain on antibiotic prophylaxis at least until their early twenties. Many physicians believe that lifelong prophylaxis is appropriate.

Patients should be examined regularly to detect signs of mitral stenosis, pulmonary hypertension, arrhythmias, and congestive heart failure.

 

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