What is the recommended prophylaxis for rheumatic heart disease?

Rheumatic Heart Disease Prophylaxis

Intramuscular benzathine penicillin G 1.2 million units every 4 weeks is the gold standard for secondary prophylaxis of rheumatic heart disease, with approximately 10 times greater efficacy than oral antibiotics in preventing recurrent rheumatic fever. 1, 2

First-Line Prophylactic Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the recommended first-line prophylaxis with Class I, Level A evidence from the American Heart Association. 3, 1, 2

• For high-risk patients (those with severe valvular disease or documented recurrence despite adherence), administer benzathine penicillin G every 3 weeks instead of every 4 weeks to maintain more consistent protective penicillin levels. 3, 1, 2

• Intramuscular benzathine penicillin G reduces rheumatic fever recurrence risk to 0.1% compared to 1% with oral antibiotics (RR 0.07,95% CI 0.02 to 0.26), representing approximately 10-fold superiority. 4

Alternative Regimens for Penicillin Allergy

• For patients with penicillin allergy, use oral penicillin V 250 mg twice daily as the second-line option. 3, 1

• Sulfadiazine 1 gram orally once daily (or 0.5 gram once daily for patients weighing ≤27 kg) is an alternative for penicillin-allergic patients. 3, 1, 2

• Erythromycin 250 mg orally twice daily is recommended by the American Heart Association for patients allergic to both penicillin and sulfadiazine. 5

• Avoid macrolide antibiotics in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, certain SSRIs) due to drug interactions. 3, 2

Duration of Prophylaxis Based on Disease Severity

The duration of prophylaxis depends on the presence and severity of cardiac involvement:

• For patients with rheumatic fever WITH carditis AND persistent valvular disease (documented rheumatic heart disease): Continue prophylaxis for 10 years after the last attack OR until age 40 years, whichever is longer. 3, 1, 2

• For patients with 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

• For patients with rheumatic fever WITHOUT carditis: Continue prophylaxis for 5 years after the last attack OR until age 21 years, whichever is longer. 1, 2

• Consider lifelong prophylaxis for patients at high risk of group A streptococcus exposure (healthcare workers, teachers, military personnel, those living in endemic areas). 3, 1, 2

Critical Management Points

• Prophylaxis must continue even after valve replacement surgery, as valve replacement does not eliminate the risk of recurrent acute rheumatic fever from group A streptococcus infection. 1

• Before initiating long-term prophylaxis, administer a full therapeutic course of penicillin to eradicate any residual group A Streptococcus, even if throat culture is negative. 1, 2

• For patients receiving penicillin prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin (such as clindamycin), as oral α-hemolytic streptococci likely have developed penicillin resistance. 1, 6

Endocarditis Prophylaxis Considerations

• Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone, unless the patient has prosthetic valves, prosthetic material used in valve repair, or previous infective endocarditis. 1, 2

• Maintaining optimal oral health remains the most important preventive measure against infective endocarditis in all patients with rheumatic heart disease. 3, 1

Common Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely, even if the patient feels well or has undergone valve surgery—this is the most common error leading to recurrent rheumatic fever. 1, 7

• Do not assume that oral antibiotics are equivalent to intramuscular benzathine penicillin G—the evidence shows approximately 10-fold difference in efficacy (0.1% vs 1% recurrence rate). 4

• Avoid abrupt discontinuation at arbitrary age cutoffs without considering individual risk factors such as ongoing streptococcal exposure, severity of valvular disease, and time since last attack. 3, 1

• Intramuscular benzathine penicillin G probably increases risk of hypersensitivity reactions (RR 137,95% CI 8.51 to 2210) and local injection site reactions (RR 29,95% CI 1.74 to 485) compared to no antibiotics, but may not significantly increase risk of anaphylaxis or sciatic nerve injury. 4

Additional Preventive Measures

• Administer influenza and pneumococcal vaccinations according to standard recommendations for all patients with rheumatic heart disease. 2, 7

• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease, while avoiding heavy isometric repetitive training that increases LV afterload. 3

• Apply guideline-directed medical therapy (diuretics, ACE inhibitors/ARBs, beta-blockers, aldosterone antagonists, sacubitril/valsartan) if left ventricular systolic dysfunction develops. 3, 2, 7