What is the management of Rheumatic Heart Disease (RHD) with mitral stenosis?

Management of Rheumatic Heart Disease with Mitral Stenosis

For rheumatic heart disease with mitral stenosis, initiate lifelong secondary antibiotic prophylaxis with benzathine penicillin G 1.2 million units IM every 4 weeks, provide symptom control with diuretics and heart rate control agents, anticoagulate if atrial fibrillation or thromboembolic risk factors are present, and perform percutaneous mitral balloon commissurotomy (PMBC) for symptomatic severe stenosis (MVA ≤1.5 cm²) with favorable valve morphology. 1, 2, 3

Secondary Prophylaxis - The Foundation of Management

All patients with rheumatic heart disease require long-term antibiotic prophylaxis to prevent recurrent acute rheumatic fever, which accelerates valve damage. 2

• Benzathine penicillin G 1.2 million units IM every 4 weeks is the gold standard regimen with the strongest evidence for preventing recurrences. 2
• Duration should be ≥10 years after the last attack OR until age 40 (whichever is longer) for patients with persistent valvular disease. 1, 2
• For penicillin-allergic patients, alternatives include oral penicillin V, sulfadiazine, or macrolide antibiotics. 2
• Critical pitfall: Prophylaxis must continue even after valve surgery - this is frequently overlooked but explicitly recommended. 1

Medical Management - Symptom Control and Complication Prevention

Heart Rate Control

Heart rate control is essential for symptom relief, particularly in patients with atrial fibrillation or during exertion. 1, 3

• Beta-blockers (e.g., metoprolol) or non-dihydropyridine calcium channel blockers are first-line for rate control. 1, 3
• Ivabradine can be used for heart rate control in sinus rhythm and may provide superior exercise capacity compared to beta-blockers. 1, 4
• Digoxin is specifically recommended for rate control in atrial fibrillation with mitral stenosis. 3

Volume Management

• Diuretics are indicated for symptom relief when pulmonary edema or congestion is present. 1, 3
• This is particularly important as mitral stenosis causes elevated left atrial pressure and pulmonary congestion. 5

Anticoagulation - Critical for Stroke Prevention

Anticoagulation with vitamin K antagonists (INR 2-3) is mandatory in specific high-risk scenarios: 1, 3

• All patients with atrial fibrillation and moderate-to-severe mitral stenosis 1
• History of systemic thromboembolism 1, 3
• Presence of left atrial thrombus on imaging 1, 3
• Dense spontaneous echo contrast in the left atrium 3
• Severely dilated left atrium (diameter >55 mm or volume index ≥60 mL/m²) 1, 3

Critical caveat: Use vitamin K antagonists (warfarin), NOT NOACs, as NOACs are not validated in rheumatic mitral stenosis. 3

Interventional Management - Timing and Patient Selection

Indications for PMBC (First-Line Intervention)

PMBC at a comprehensive valve center is the preferred intervention for rheumatic mitral stenosis when: 1, 2, 3

Symptomatic patients (NYHA class II-IV) with:

• Severe mitral stenosis (MVA ≤1.5 cm²) 1
• Favorable valve morphology (echocardiographic score ≤8) 1
• Less than moderate mitral regurgitation 1
• Absence of left atrial thrombus 1

Asymptomatic patients with severe stenosis (MVA ≤1.5 cm²) and any of:

• New-onset atrial fibrillation 1, 3
• Pulmonary artery systolic pressure >50 mmHg at rest 1, 3
• High thromboembolic risk (history of embolism, dense spontaneous contrast) 1, 3
• Planning pregnancy 2

PMBC may be considered even with suboptimal anatomy if the patient is high surgical risk or not a surgical candidate. 1

Surgical Intervention

Mitral valve surgery (repair preferred over replacement when feasible) is indicated when: 1

• Severely symptomatic patients (NYHA III-IV) with MVA ≤1.5 cm² who are not suitable for PMBC due to:
  • Unfavorable valve anatomy (heavy calcification, echocardiographic score >8) 1
  • Moderate-to-severe mitral regurgitation 1
  • Left atrial thrombus (contraindication to PMBC) 1
• Patient is undergoing other cardiac surgery 1
• Previous failed PMBC 1

For women of childbearing age requiring valve replacement, consider bioprosthetic valve rather than mechanical to avoid anticoagulation hazards during pregnancy. 1

Management of Restenosis After PMBC

If restenosis occurs after initial PMBC: 1

• Repeat PMBC can be performed if the predominant mechanism is commissural refusion, especially in elderly patients or those at high surgical risk. 1
• Valve replacement is needed in most cases when restenosis is due to progressive valve degeneration rather than commissural refusion. 1

Monitoring and Surveillance

Regular echocardiographic monitoring is essential to detect disease progression: 2, 3

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

More frequent monitoring is warranted if the left ventricle is dilating or if the patient is approaching intervention thresholds. 2

Special Populations

Pregnancy

Women with severe mitral stenosis should avoid pregnancy until the valve disease is treated. 1

• Pre-pregnancy evaluation and intervention (PMBC if suitable) should be performed in asymptomatic patients with severe stenosis. 1, 2
• During pregnancy, use beta-blockers for heart rate control and diuretics for volume overload. 1
• Cardiac surgery during pregnancy may be necessary if medical and interventional therapies fail and there is risk to the mother's life. 1

Heart Failure with LV Dysfunction

If left ventricular systolic dysfunction develops (uncommon in isolated mitral stenosis but can occur): 2

• Implement guideline-directed medical therapy: ACE inhibitors/ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when indicated. 2
• Careful blood pressure management is essential, avoiding abrupt BP lowering in stenotic lesions. 2

Additional Preventive Measures

Infective endocarditis prophylaxis: 2

• Antibiotic prophylaxis before dental procedures involving gingival manipulation, periapical region, or oral mucosa perforation 2
• Not needed if patient is already on secondary prophylaxis antibiotics 2

Vaccinations: 2

• Influenza and pneumococcal vaccinations are recommended 2

Optimal oral health maintenance to reduce endocarditis risk 2

Common Pitfalls to Avoid

• Discontinuing secondary antibiotic prophylaxis too early - this must continue for ≥10 years or until age 40, and even after valve surgery. 1, 2
• Using NOACs instead of warfarin - only vitamin K antagonists are validated in rheumatic mitral stenosis. 3
• Inadequate anticoagulation monitoring in patients with atrial fibrillation or other thromboembolic risk factors. 2
• Failure to recognize pregnancy as a high-risk period requiring pre-pregnancy intervention. 1, 2
• Assuming medical therapy alone is sufficient - it is palliative and does not prevent disease progression; regular monitoring for intervention indications is essential. 3
• Neglecting regular follow-up echocardiography to detect progression to intervention thresholds. 2