How should rheumatic heart disease be medically managed, including secondary penicillin prophylaxis, heart‑failure therapy, anticoagulation for atrial fibrillation, and criteria for surgical referral?

Medical Management of Rheumatic Heart Disease

All patients with rheumatic heart disease require long-term benzathine penicillin G 1.2 million units intramuscularly every 4 weeks as the cornerstone of management, combined with guideline-directed heart failure therapy when left ventricular dysfunction develops, anticoagulation for atrial fibrillation, and timely surgical referral for severe symptomatic valve disease. 1, 2

Secondary Penicillin Prophylaxis

First-Line Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard with Class I, Level A evidence and is approximately 10 times more effective than oral antibiotics. 2, 3
• For high-risk patients (those with recurrence despite adherence or ongoing high streptococcal exposure), administer benzathine penicillin G every 3 weeks instead of every 4 weeks to maintain more consistent protective levels. 2, 4
• Good adherence reduces the risk of acute rheumatic fever recurrence or rheumatic heart disease progression by 71%. 5

Critical Safety Consideration for Severe Disease

• Patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, or reduced left ventricular systolic function are at elevated risk of cardiovascular compromise following benzathine penicillin G injections. 6
• For these elevated-risk patients, oral prophylaxis should be strongly considered as the risk of adverse reaction may outweigh the theoretical benefit of intramuscular administration. 6

Alternative Regimens for Penicillin Allergy

• Oral penicillin V 250 mg twice daily is the second-line option. 2, 3
• Sulfadiazine 1 gram orally once daily (or 0.5 gram once daily for patients weighing ≤27 kg) for penicillin-allergic patients. 2, 3
• Macrolide or azalide antibiotics only if allergic to both penicillin and sulfadiazine, but avoid in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, some SSRIs) due to drug interactions. 1, 3

Duration of Prophylaxis Based on Disease Severity

For rheumatic fever with carditis and residual heart disease (persistent valvular 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, day-care workers, healthcare workers in endemic areas). 1, 2

For 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, 3

For rheumatic fever without carditis:

• Continue prophylaxis for 5 years after the last attack OR until age 21 years, whichever is longer. 1, 3

Essential Prophylaxis Principles

• Prophylaxis must continue even after valve replacement surgery, as surgery does not eliminate the risk of recurrent acute rheumatic fever. 1, 2
• 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. 3, 4

Heart Failure Therapy

Guideline-Directed Medical Therapy

• When left ventricular systolic dysfunction develops, apply standard heart failure therapy including diuretics, ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan. 1, 3, 4
• Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions (mitral stenosis, aortic stenosis) as this can precipitate hemodynamic collapse. 1, 3
• Use diuretics for volume overload in symptomatic patients. 1

Heart Rate Control

• Beta-blockers are recommended for heart rate control, particularly important in patients with mitral stenosis where diastolic filling time is critical. 1
• Digoxin may be added for additional rate control in atrial fibrillation when beta-blockers alone are insufficient. 1

Anticoagulation for Atrial Fibrillation

Indications for Anticoagulation

• Anticoagulation should be considered for all women with rheumatic heart disease and atrial fibrillation. 1
• Also consider anticoagulation for patients in sinus rhythm with very severe left atrial dilatation, spontaneous echo contrast on echocardiography, or heart failure. 1
• The choice of anticoagulation agent should be discussed with the patient, weighing bleeding risk against thromboembolism risk. 1

Criteria for Surgical Referral

Timing of Intervention

• Evaluate all patients with symptomatic severe rheumatic mitral stenosis for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis. 3, 4
• For asymptomatic patients with severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²), PMBC at a comprehensive valve center is reasonable. 1

Special Considerations for Pregnancy

• Women with moderate-severe mitral stenosis considering pregnancy should undergo PMBC prior to pregnancy if symptomatic or if asymptomatic but with clinically significant stenosis. 1
• If intervention is needed during pregnancy, PMBC after the 20th week should only be performed in experienced centers with multidisciplinary team consultation. 1

Infective Endocarditis Prophylaxis

Current Recommendations

• Routine endocarditis prophylaxis is NOT recommended for rheumatic heart disease alone. 3, 4
• Antibiotic prophylaxis IS reasonable before dental procedures (involving manipulation of gingival tissue, periapical region, or perforation of oral mucosa) ONLY for patients with: 1
  • Prosthetic cardiac valves (including transcatheter-implanted prostheses)
  • Prosthetic material used for valve repair (annuloplasty rings, chords, clips)
  • Previous infective endocarditis
• Antibiotic prophylaxis is NOT recommended for nondental procedures (TEE, esophagogastroduodenoscopy, colonoscopy, cystoscopy) in the absence of active infection. 1

Most Important Preventive Measure

• Maintaining optimal oral health remains the most important component of preventing infective endocarditis in all patients with rheumatic heart disease. 1, 2

Additional Preventive Measures

Vaccinations

• Administer influenza and pneumococcal vaccinations according to standard recommendations for all patients with rheumatic heart disease. 1, 2, 3

Exercise

• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 2, 3

Common Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely, even if the patient feels well, has normal echocardiography, or has undergone valve surgery. 2, 3
• Do not stop prophylaxis at arbitrary age cutoffs without considering individual risk factors such as ongoing streptococcal exposure, severity of valvular disease, and time since last attack. 2
• For patients receiving benzathine penicillin G prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin to provide broader coverage. 3
• In patients with severe valvular disease or reduced ventricular function, strongly consider switching from intramuscular to oral prophylaxis to avoid cardiovascular compromise from injection reactions. 6