How should a child or adolescent with confirmed rheumatic fever or established rheumatic heart disease be managed, including lifelong secondary penicillin prophylaxis (with alternatives for penicillin allergy), heart‑failure treatment, anticoagulation for atrial fibrillation, and indications for surgical valve intervention?

Management of Rheumatic Fever and Rheumatic Heart Disease in Children and Adolescents

Immediate Treatment of Acute Episode

All children and adolescents with confirmed acute rheumatic fever must receive a complete 10-day course of penicillin to eradicate Group A Streptococcus, even if throat culture is negative at diagnosis, followed immediately by lifelong secondary prophylaxis with intramuscular benzathine penicillin G. 1

Primary Antibiotic Eradication

• Oral Penicillin V: 250 mg twice daily for 10 days for children <27 kg; 500 mg 2-3 times daily for 10 days for children ≥27 kg, adolescents, and adults 1

• Intramuscular Benzathine Penicillin G: Single injection of 600,000 units for patients <27 kg; 1,200,000 units for patients ≥27 kg 1

• Strongly prefer intramuscular route for patients unlikely to complete oral therapy, those with personal/family history of rheumatic fever or rheumatic heart disease, and those with environmental risk factors 1

• For penicillin allergy: Azithromycin 500 mg once daily for 5 days or clarithromycin 250 mg twice daily for 10 days in adults; erythromycin or first-generation cephalosporins (if no immediate-type hypersensitivity) in children 2, 1

Anti-Inflammatory Treatment

• High-dose aspirin (acetylsalicylic acid): 75-100 mg/kg/day for arthritis and mild carditis, continued for 4-6 weeks 1

• Corticosteroids (prednisone 1-2 mg/kg/day for 1-2 weeks): Reserved for severe carditis, congestive heart failure, or pericarditis, as these are more potent anti-inflammatory agents than salicylates 2, 3

• Total duration of anti-inflammatory therapy: 12 weeks 4, 3

Lifelong Secondary Prophylaxis

Benzathine penicillin G 1,200,000 units intramuscularly every 4 weeks is the gold standard for secondary prophylaxis, providing approximately 10 times greater protection than oral antibiotics (0.1% vs 1% recurrence rate; relative risk 0.07,95% CI 0.02-0.26). 1, 5

Prophylaxis Regimen Selection

• Standard regimen: Benzathine penicillin G 1,200,000 units IM every 4 weeks 1, 5

• High-risk populations: Consider every 3 weeks dosing for children, adolescents, parents of young children, teachers, healthcare workers, military recruits, economically disadvantaged populations, or patients with recurrence despite adherence to 4-week regimen 1, 5

• Penicillin allergy: Sulfadiazine as alternative; erythromycin orally twice daily for non-severe or immediate penicillin hypersensitivity 1, 6

Duration of Secondary Prophylaxis Based on Cardiac Involvement

The duration is determined by the presence or absence of carditis during the initial episode, not current cardiac status: 5

Cardiac Status	Duration	Citation
No carditis	5 years after last attack OR until age 21 (whichever is longer)	[1,5]
Carditis without residual valvular disease	10 years after last attack OR until age 21 (whichever is longer)	[1,5]
Carditis with residual valvular disease	10 years after last attack OR until age 40 (whichever is longer); often lifelong	[1,5,2]

Critical Rationale for Continuous Prophylaxis

• At least one-third of rheumatic fever cases arise from asymptomatic Group A Streptococcus infections, making continuous prophylaxis essential even in asymptomatic patients 5, 1

• Each recurrence causes additional cardiac valve damage, and recurrent rheumatic fever can occur even when symptomatic streptococcal infections are treated optimally 1, 3

• Patients with prior acute rheumatic fever have extremely high risk of recurrence when exposed to new Group A Streptococcus pharyngitis 1

Heart Failure Management

Valvular regurgitation (particularly mitral regurgitation), not myocarditis, is the cause of congestive heart failure in active rheumatic carditis. 3

Medical Management

• Standard heart failure therapy: Diuretics, ACE inhibitors, and beta-blockers as indicated by hemodynamic status 4

• Corticosteroids: Indicated for severe carditis with congestive heart failure, as these are more effective than salicylates for severe cardiac involvement 2, 3

Surgical Intervention Indications

• Mitral valve replacement or repair: Indicated for intractable hemodynamics due to severe mitral regurgitation unresponsive to medical therapy during acute phase 3

• Prophylaxis continuation: Secondary antibiotic prophylaxis must continue even after valve surgery, including prosthetic valve replacement 2

Anticoagulation for Atrial Fibrillation

Patients with rheumatic heart disease who develop atrial fibrillation require anticoagulation due to high stroke risk. 7, 4

• Protocol for atrial fibrillation management should follow standard guidelines for anticoagulation in valvular atrial fibrillation, typically with warfarin (target INR 2-3) 4

• Atrial fibrillation is a recognized complication of advanced rheumatic heart disease that can develop in untreated or inadequately prophylaxed patients 7

Surgical Valve Intervention Indications

Surgery is indicated when hemodynamically significant chronic valvular lesions develop, typically from inadequate penicillin prophylaxis leading to recurrent episodes. 3

Timing Considerations

• Acute phase: Mitral valve surgery (replacement or repair) for intractable heart failure due to severe mitral regurgitation 3

• Chronic phase: Valve surgery for hemodynamically significant lesions causing symptoms or ventricular dysfunction 3

• Development of chronic valvular lesions depends on presence/absence of carditis in previous attacks and compliance with secondary prophylaxis 3

Monitoring and Follow-Up

Serial echocardiographic evaluations are essential for monitoring patients with previous rheumatic fever, as they detect subclinical progression of valvular disease not apparent on physical examination. 5

• Echocardiography is more sensitive and specific than auscultation for identifying cardiac involvement and exact cause of heart murmurs 7

• The 2015 modified Jones criteria include echocardiography for assessing cardiac involvement in acute rheumatic fever diagnosis 7

• Do not discontinue prophylaxis prematurely based solely on normal echocardiographic findings, as patients remain susceptible to Group A Streptococcus infection 5

Endocarditis Prophylaxis

Routine endocarditis prophylaxis is NOT recommended for patients with rheumatic heart disease, unless they have prosthetic valves or prosthetic material used in valve repair. 5

• Exception: Patients with rheumatic heart disease receiving benzathine penicillin G prophylaxis should receive amoxicillin prophylaxis before high-risk dental or surgical procedures; if recently treated with penicillin/amoxicillin or have immediate penicillin hypersensitivity, use clindamycin 6

Critical Pitfalls to Avoid

• Never assume normal valvular function eliminates need for prophylaxis—duration is determined by presence/absence of carditis during initial episode, not current cardiac status 5

• Never stop prophylaxis prematurely—recurrent rheumatic fever worsens cardiac damage, and many triggering infections are asymptomatic 5, 1

• Never use broad-spectrum cephalosporins when narrow-spectrum agents suffice—this is more expensive and selects for resistant flora 1

• Never prescribe macrolides with cytochrome P450 3A inhibitors due to QT prolongation risk 2

• Warm benzathine penicillin G to room temperature before administration to reduce injection discomfort 1

• Family members of patients with rheumatic fever should have prompt treatment of streptococcal infections 2