Management of Acute Rheumatic Fever with Acute Mitral Regurgitation
For a child with acute rheumatic fever and acute mitral regurgitation causing heart failure, immediately initiate a full 10-day course of penicillin to eradicate Group A Streptococcus, begin continuous secondary prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks (or every 3 weeks in high-risk situations), and consider corticosteroids at 1-2 mg/kg/day for severe cardiac inflammation. 1
Acute Phase Antibiotic Treatment
First-Line Regimen
- Administer a complete 10-day course of penicillin to eradicate residual Group A Streptococcus, even if the throat culture is negative at the time of diagnosis 1, 2
- Oral penicillin V 250 mg twice daily for 10 days is the standard regimen for most children 1, 2
- For children weighing >27 kg, adolescents, and adults, increase the dose to penicillin V 500 mg 2-3 times daily for 10 days 1, 2
- Alternatively, intramuscular benzathine penicillin G as a single injection (600,000 units for patients <27 kg; 1,200,000 units for patients ≥27 kg) may be used 2
Penicillin-Allergic Patients
- For penicillin allergy, use erythromycin or first-generation cephalosporins (if no immediate-type hypersensitivity) 1
- Azithromycin 500 mg once daily for 5 days or clarithromycin 250 mg twice daily for 10 days are acceptable alternatives 1
Anti-Inflammatory Therapy for Cardiac Involvement
Corticosteroid Therapy
- In cases with severe inflammation or cardiac involvement causing heart failure, administer prednisone 1-2 mg/kg/day for 1-2 weeks 1
- For severe cases with significant cardiac involvement, intravenous methylprednisolone 1000 mg/day initially may be considered, followed by oral prednisone 1
Aspirin Therapy
- High-dose aspirin (acetylsalicylic acid) 75-100 mg/kg/day for 4-6 weeks is effective for controlling inflammatory manifestations of arthritis and mild carditis 2
- Avoid aspirin in children due to the risk of Reye syndrome 3
- The arthritis of rheumatic fever responds rapidly to acetylsalicylic acid, typically resolving within days 2
Secondary Prophylaxis (Critical for Preventing Recurrence)
Gold Standard Regimen
- Initiate continuous antimicrobial prophylaxis immediately upon diagnosis of acute rheumatic fever 1, 2
- Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard, providing approximately 10 times greater protection than oral antibiotics (0.1% vs 1% recurrence rate) 1, 4, 2
- The relative risk reduction with intramuscular benzathine penicillin G versus oral antibiotics is 0.07 (95% CI 0.02 to 0.26) 1, 4
High-Risk Dosing Adjustment
- For high-risk populations or patients with recurrence despite adherence to the 4-week regimen, administer benzathine penicillin G every 3 weeks 1, 4, 2
- High-risk populations include children, adolescents, parents of young children, teachers, healthcare workers, military recruits, and economically disadvantaged populations 4, 2
Alternative Oral Regimens (Less Effective)
- For patients who cannot tolerate intramuscular injections: penicillin V 250 mg twice daily (children) or 500 mg 2-3 times daily (adolescents/adults) 1, 4
- For penicillin-allergic patients: sulfadiazine 0.5 g once daily for patients ≤27 kg or 1 g once daily for adults 1, 4
- For patients allergic to both penicillin and sulfonamides: macrolides (erythromycin or clarithromycin) or azalides (azithromycin) 1, 4
Duration of Secondary Prophylaxis
Based on Cardiac Involvement
- For rheumatic carditis with residual heart disease (such as mitral regurgitation): continue prophylaxis for at least 10 years after the last attack OR until age 40 years (whichever is longer), often lifelong 1, 4, 2
- For rheumatic carditis without residual heart disease: 10 years after the last attack OR until age 21 years (whichever is longer) 1, 4, 2
- For rheumatic fever without carditis: 5 years after the last attack OR until age 21 years (whichever is longer) 1, 4, 2
Critical Pitfalls and Caveats
Why Continuous Prophylaxis is Essential
- At least one-third of rheumatic fever cases result from asymptomatic Group A Streptococcal infections, making prevention challenging 1, 4
- Even when streptococcal pharyngitis is optimally treated, rheumatic fever can still occur in susceptible individuals 1, 4
- Each recurrence of acute rheumatic fever worsens cardiac damage and valvular disease 4, 2
- The majority of prophylaxis failures occur in non-adherent patients; even with optimal adherence, oral prophylaxis carries higher recurrence risk than intramuscular benzathine penicillin G 3, 4
Adherence Strategies
- Strongly consider intramuscular benzathine penicillin G for patients unlikely to complete oral courses, those with personal or family histories of rheumatic fever, and those with environmental risk factors 2
- Warming benzathine penicillin G to room temperature before administration reduces injection discomfort 2
- Switching to oral prophylaxis should only be considered when patients reach late adolescence or young adulthood AND have remained free of rheumatic attacks for at least 5 years 3, 4
Drug Interactions and Contraindications
- Macrolide antibiotics should be avoided in patients receiving strong cytochrome P450 3A inhibitors (e.g., azole antifungals, HIV protease inhibitors, certain SSRIs) due to QT prolongation risk 4
- Sulfonamides are contraindicated in late pregnancy due to transplacental passage and competition with bilirubin for albumin-binding sites 3, 4
Endocarditis Prophylaxis
- Routine endocarditis prophylaxis is no longer recommended for patients with rheumatic heart disease, unless they have prosthetic valves or prosthetic material used in valve repair 3, 4
- For patients on penicillin prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin because oral α-hemolytic streptococci are likely penicillin-resistant 3, 4