What are the latest guidelines for managing rheumatic fever?

Latest Guidelines for Rheumatic Fever Management

Secondary Prophylaxis: The Cornerstone of Management

All patients with a history of rheumatic fever or rheumatic heart disease require long-term antibiotic prophylaxis to prevent recurrent episodes and progression of valvular damage. 1

First-Line Antibiotic Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard prophylaxis regimen with Class I, Level A evidence from the American Heart Association. 2, 3

• Intramuscular benzathine penicillin G is approximately 10 times more effective than oral antibiotics in preventing recurrence (0.1% vs 1% recurrence rate). 2, 4

• For high-risk patients or those with recurrence despite adherence to the 4-week regimen, administer benzathine penicillin G every 3 weeks. 2, 5 This shorter interval maintains adequate serum penicillin levels (≥0.02 mcg/mL) in 56% of patients at 21 days versus only 33% at 28 days, and reduces streptococcal infections from 12.6 to 7.5 per 100 patient-years. 6

Alternative Regimens for Penicillin Allergy

• Penicillin V 250 mg orally twice daily (for children) or 500 mg 2-3 times daily (for adolescents/adults). 2, 3

• Sulfadiazine 1 gram orally once daily (for adults) or 0.5 gram once daily for patients weighing ≤27 kg. 1, 2

• Macrolide or azalide antibiotics (dose varies) for patients allergic to both penicillin and sulfadiazine, but avoid in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, certain SSRIs). 1

Duration of Prophylaxis: Risk-Stratified Approach

The duration depends on cardiac involvement and residual disease:

Rheumatic Fever WITH Carditis AND Residual Heart Disease

• Continue prophylaxis for 10 years after the last attack OR until age 40 years, whichever is longer. 1, 2

• Consider lifelong prophylaxis for patients at high risk of group A streptococcus exposure (teachers, healthcare workers, military recruits, parents of young children, economically disadvantaged populations). 1, 2

• Secondary prophylaxis must continue even after valve replacement surgery. 3 This is a critical point—valve surgery does not eliminate the risk of recurrent rheumatic fever. 3

Rheumatic Fever WITH Carditis BUT NO Residual Heart Disease

• Continue prophylaxis for 10 years after the last attack OR until age 21 years, whichever is longer. 1, 2

Rheumatic Fever WITHOUT Carditis

• Continue prophylaxis for 5 years after the last attack OR until age 21 years, whichever is longer. 1, 2

Acute Rheumatic Fever Management

Initial Treatment

• Administer a full therapeutic course of penicillin to eradicate residual group A Streptococcus, even if throat culture is negative at diagnosis. 2, 5 At least one-third of rheumatic fever cases arise from asymptomatic streptococcal infections. 2

• Initiate long-term antimicrobial prophylaxis immediately once acute rheumatic fever is diagnosed. 2

• Provide adjunctive therapy with acetaminophen or NSAIDs for moderate to severe symptoms or high fever, but avoid aspirin in children due to Reye's syndrome risk. 5

Diagnostic Approach

• Perform throat culture or rapid antigen detection test for group A Streptococcus, though sensitivity is low and negative rapid tests require throat culture confirmation. 5

• Echocardiography is significantly more sensitive and specific than auscultation alone for detecting carditis and valvular involvement—use it routinely. 5

Cardiac Complications Management

Medical Therapy for Left Ventricular Dysfunction

• Apply guideline-directed medical therapy including diuretics, ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when left ventricular systolic dysfunction develops. 1, 5

• Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions. 1

Surgical Intervention

• Evaluate all patients with symptomatic severe rheumatic mitral stenosis for percutaneous mitral balloon commissurotomy or mitral valve surgery within 3 months of diagnosis. 5

Surveillance Schedule

• Mild disease: echocardiography every 3-5 years 5
• Moderate disease: echocardiography every 1-2 years 5
• Severe disease or dilating left ventricle: echocardiography every 6-12 months 5

Infective Endocarditis Prophylaxis

The American Heart Association no longer recommends routine endocarditis prophylaxis for patients with rheumatic heart disease, unless they have: 2, 3

• Prosthetic cardiac valves (including transcatheter-implanted prostheses)
• Prosthetic material used for valve repair (annuloplasty rings, chords, clips)
• Previous infective endocarditis

Critical Caveat for Dental Procedures

For patients receiving penicillin prophylaxis for rheumatic fever who require endocarditis prophylaxis for dental procedures, use an agent OTHER than penicillin (such as clindamycin or amoxicillin), as oral α-hemolytic streptococci likely have developed penicillin resistance. 3, 7

Maintaining optimal oral health remains the most important component of preventing infective endocarditis. 1, 3

Additional Preventive Measures

• Administer influenza and pneumococcal vaccinations according to standard recommendations. 1, 5

• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 1

• Implement register/recall systems and dedicated health teams to improve adherence to secondary prophylaxis, as adherence remains a global challenge. 8, 9