What is the treatment for rheumatic heart disease?

Treatment for Rheumatic Heart Disease

All patients with rheumatic heart disease require long-term intramuscular benzathine penicillin G (1.2 million units every 4 weeks) as the cornerstone of treatment to prevent recurrent rheumatic fever and progression of valvular damage. 1, 2

Secondary Antibiotic Prophylaxis

First-Line Regimen

• Intramuscular benzathine penicillin G 1.2 million units every 4 weeks is the gold standard with Class I, Level A evidence and is approximately 10 times more effective than oral antibiotics in preventing recurrence. 1, 2, 3
• For high-risk patients or those with recurrence despite adherence to the 4-week regimen, administer benzathine penicillin G every 3 weeks. 2, 4

Alternative Regimens for Penicillin Allergy

• Oral penicillin V: 250 mg twice daily for children or 500 mg 2-3 times daily for adolescents/adults. 1, 2
• 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 for patients allergic to both penicillin and sulfadiazine, but avoid in patients taking cytochrome P450 3A inhibitors. 2
• For non-severe penicillin hypersensitivity, erythromycin orally twice daily is an option. 5

Duration of Prophylaxis

The duration depends on the severity of cardiac involvement:

• Rheumatic fever with carditis and residual heart disease (persistent valvular disease): Continue for 10 years after the last episode OR until age 40 years, whichever is longer; consider lifelong prophylaxis for high-risk patients (teachers, day-care workers with high streptococcal exposure). 6, 1, 2

• Rheumatic fever with carditis but no residual heart disease: Continue for 10 years after the last episode OR until age 21 years, whichever is longer. 6, 1, 2

• Rheumatic fever without carditis: Continue for 5 years after the last episode OR until age 21 years, whichever is longer. 6, 1, 2

Critical Caveat: Post-Valve Surgery

• Prophylaxis must continue after valve surgery, including prosthetic valve replacement, following the same duration guidelines as non-surgical patients—this is a critical departure from the assumption that valve replacement eliminates the need for secondary prophylaxis. 1

Management of Acute Rheumatic Fever

Initial Treatment

• Administer a full therapeutic course of penicillin to eradicate residual group A Streptococcus, even if throat culture is negative at diagnosis. 2, 4
• 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. 2
• In severe cases with significant cardiac involvement, consider corticosteroids such as prednisone at 1-2 mg/kg/day for 1-2 weeks. 4

Management of Cardiac Complications

Heart Failure Management

• Apply guideline-directed medical therapy including diuretics, ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when left ventricular systolic dysfunction develops. 2
• Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions. 2

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. 2

Infective Endocarditis Prophylaxis

Current guidelines no longer recommend routine endocarditis prophylaxis for patients with rheumatic heart disease, unless they have prosthetic cardiac valves, prosthetic material used for valve repair, or previous infective endocarditis. 6, 2

Important Exception

• For patients receiving penicillin prophylaxis for rheumatic fever who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin, as oral α-hemolytic streptococci are likely to have developed resistance to penicillin. 1, 2
• Patients with rheumatic heart disease receiving benzathine penicillin G prophylaxis should receive amoxicillin prophylaxis before undergoing high-risk dental or surgical procedures; if recently treated with penicillin/amoxicillin or have immediate penicillin hypersensitivity, use clindamycin. 5

Safety Considerations for Intramuscular Benzathine Penicillin G

High-Risk Patients

• Patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, decreased left ventricular systolic dysfunction, or symptomatic heart disease may be at elevated risk of cardiovascular compromise following BPG injections. 7
• For these elevated-risk patients, oral prophylaxis should be strongly considered as the risk of adverse reaction to BPG may outweigh its theoretical benefit. 7

Risk Reduction Strategies

• Implement a multifaceted strategy for vasovagal risk reduction in all patients with rheumatic heart disease receiving BPG. 7

Additional Preventive Measures

• Administer influenza and pneumococcal vaccinations according to standard recommendations. 2
• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 2
• Maintain optimal oral health as an important component of overall healthcare. 1
• Family members of patients with current or previous rheumatic fever should have prompt treatment of streptococcal infections. 4

Common Pitfalls to Avoid

• Never discontinue secondary prophylaxis after valve surgery—patients remain susceptible to group A streptococcus infection and recurrent acute rheumatic fever. 1
• At least one-third of rheumatic fever cases result from asymptomatic group A Streptococcus infections, making prevention challenging and underscoring the importance of continuous prophylaxis. 4
• Even when streptococcal pharyngitis is treated optimally, rheumatic fever can still occur in susceptible individuals. 4