What is the management plan for rheumatic heart disease?

Management of Rheumatic Heart Disease

All patients with rheumatic heart disease require lifelong secondary antibiotic prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks, combined with medical management of cardiac complications and timely valve intervention when severe symptomatic disease develops. 1, 2

Secondary Antibiotic Prophylaxis

Benzathine penicillin G 1.2 million units IM every 4 weeks is the gold standard regimen with the strongest evidence (Class I, Level A) for preventing recurrent acute rheumatic fever. 1, 3

Alternative Regimens for Penicillin Allergy

• Oral penicillin V 250 mg twice daily 3
• Sulfadiazine 1 g orally once daily (adults); 0.5 g once daily for patients ≤27 kg 3
• Macrolide antibiotics (erythromycin) for non-severe or immediate penicillin hypersensitivity 4

Duration of Prophylaxis

• Patients with persistent valvular disease: Continue for ≥10 years after last acute rheumatic fever episode OR until age 40, whichever is longer 1, 3
• Patients with carditis but no residual heart disease: 10 years after last attack OR until age 21, whichever is longer 3
• Patients without carditis: 5 years after last attack OR until age 21, whichever is longer 3
• Critical pitfall: Prophylaxis must continue even after valve surgery—this does not eliminate the risk of recurrent acute rheumatic fever 3

Special Considerations for High-Risk Patients

• Patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, or reduced left ventricular systolic function may be at elevated risk of cardiovascular compromise following benzathine penicillin G injections. 5
• For these elevated-risk patients, oral prophylaxis should be strongly considered instead of intramuscular injections 5

Medical Management

Heart Rate Control

• Beta-blockers or non-dihydropyridine calcium channel blockers are first-line for rate control, particularly in patients with atrial fibrillation or exertional symptoms. 1
• Digoxin is specifically recommended for rate control in atrial fibrillation with mitral stenosis 1

Volume Management

• Diuretics are indicated for symptom relief when pulmonary edema or congestion is present, as mitral stenosis causes elevated left atrial pressure and pulmonary congestion 1

Anticoagulation

Vitamin K antagonists (INR 2-3), NOT NOACs, are mandatory for anticoagulation in rheumatic mitral stenosis. 1

Indications for anticoagulation:

• All patients with atrial fibrillation and moderate-to-severe mitral stenosis 6, 1
• History of systemic thromboembolism 6, 1
• Presence of left atrial thrombus on imaging 6, 1
• Dense spontaneous echo contrast in the left atrium 1
• Severely dilated left atrium 1

Heart Failure Management

• For patients with left ventricular systolic dysfunction, implement guideline-directed medical therapy including ACE inhibitors/ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when indicated 2
• Careful blood pressure management is essential—avoid abrupt BP lowering in stenotic lesions 2

Diagnostic Evaluation

Initial Assessment

• Transthoracic echocardiography (TTE) is indicated to establish diagnosis, quantify hemodynamic severity, assess concomitant valvular lesions, and demonstrate valve morphology for determining suitability for intervention. 6
• Transesophageal echocardiography (TEE) should be performed before percutaneous mitral balloon commissurotomy (PMBC) to assess for left atrial thrombus and evaluate severity of mitral regurgitation 6

Exercise Testing

• In patients with rheumatic mitral stenosis and discrepancy between resting echocardiographic findings and clinical symptoms, exercise testing with Doppler or invasive hemodynamic assessment is recommended to evaluate symptomatic response, exercise capacity, and response of mean mitral gradient and pulmonary artery pressure 6

Interventional Management

Percutaneous Mitral Balloon Commissurotomy (PMBC)

PMBC at a Comprehensive Valve Center is the preferred intervention for symptomatic patients (NYHA class II-IV) with severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) and favorable valve morphology. 6, 1, 2

Requirements for PMBC:

• Favorable valve morphology (mobile, relatively thin leaflets free of calcium, without significant subvalvular fusion) 2
• Less than moderate (2+) mitral regurgitation 6, 1
• Absence of left atrial thrombus 6, 1

PMBC for Asymptomatic Patients:

• Pulmonary artery systolic pressure >50 mmHg at rest: PMBC is reasonable 6, 1
• New-onset atrial fibrillation: PMBC may be considered 6, 1
• Planning pregnancy: Pre-pregnancy evaluation and intervention should be performed 1

Long-term Outcomes:

• 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years 2

Surgical Intervention

Mitral valve surgery (repair, commissurotomy, or valve replacement) is indicated for severely symptomatic patients (NYHA class III-IV) with severe rheumatic mitral stenosis who:

• Are not candidates for PMBC due to unfavorable valve anatomy 6, 2
• Have moderate-to-severe mitral regurgitation 2
• Have left atrial thrombus 2
• Have failed previous PMBC 6, 2
• Require other cardiac procedures 6
• Do not have access to PMBC 6

Special Consideration:

• Bioprosthetic valve rather than mechanical is recommended for women of childbearing age requiring valve replacement to avoid anticoagulation hazards during pregnancy 1

Monitoring and Surveillance

Regular echocardiographic monitoring is essential to detect disease progression, with frequency depending on severity: 1, 2

• Severe mitral stenosis: Every 6-12 months 1, 2
• Moderate mitral stenosis: Every 1-2 years 1, 2
• Mild mitral stenosis: Every 3-5 years 1, 2

Infective Endocarditis Prophylaxis

• Antibiotic prophylaxis before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa is recommended 2
• Critical exception: Not needed if patient is already on secondary prophylaxis antibiotics 1
• For patients receiving penicillin prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin, as oral α-hemolytic streptococci are likely to have developed resistance 3
• 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 4

Special Populations

Pregnancy

• Women with severe mitral stenosis should avoid pregnancy until the valve disease is treated. 1
• Pre-pregnancy evaluation and intervention should be performed in asymptomatic patients with severe stenosis 1, 2
• Beta-blockers and diuretics are recommended during pregnancy for heart rate control and volume overload 1
• Cardiac surgery may be necessary if medical and interventional therapies fail 1

Additional Preventive Measures

• Influenza and pneumococcal vaccinations are recommended 1, 2
• Optimal oral health maintenance is essential to reduce endocarditis risk 1, 3

Common Pitfalls to Avoid

• Discontinuing secondary antibiotic prophylaxis too early or after valve surgery 1, 3
• Using NOACs instead of warfarin for anticoagulation in rheumatic mitral stenosis 1
• Inadequate anticoagulation monitoring 1, 2
• Failure to recognize pregnancy as a high-risk period requiring pre-pregnancy intervention 1, 2
• Assuming medical therapy alone is sufficient for severe symptomatic disease—delaying valve intervention worsens outcomes 2
• Neglecting regular follow-up echocardiography 1, 2
• Overlooking the need for infective endocarditis prophylaxis during high-risk procedures 2
• Assuming valve replacement eliminates rheumatic fever risk—patients remain susceptible to group A streptococcus infection 3