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 benzathine penicillin G 1.2 million units IM 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 and must be initiated immediately upon diagnosis. 1, 3 This intramuscular regimen is approximately 10 times more effective than oral antibiotics in preventing recurrent acute rheumatic fever, which accelerates valve damage. 3

Duration of Prophylaxis

The duration depends on disease severity and must continue even after valve surgery—a critical pitfall frequently overlooked: 1, 3

• Patients with persistent valvular disease: Continue for ≥10 years after last attack OR until age 40 (whichever is longer) 1, 3
• Patients with carditis but no residual heart disease: Continue for 10 years after last attack OR until age 21 (whichever is longer) 3
• Patients without carditis: Continue for 5 years after last attack OR until age 21 (whichever is longer) 3
• High-risk patients (frequent streptococcal exposure): Consider lifelong prophylaxis 3

Alternative Regimens for Penicillin Allergy

• Oral penicillin V: 250 mg twice daily (children) or 500 mg 2-3 times daily (adults) 3
• Sulfadiazine: 1 gram once daily (adults) or 0.5 gram once daily (≤27 kg) 3
• Macrolides/azalides: Only if allergic to both penicillin and sulfadiazine; avoid with cytochrome P450 3A inhibitors 3

For high-risk patients with recurrence despite adherence, administer benzathine penicillin G every 3 weeks instead of every 4 weeks. 3

Medical Management

Heart Rate Control

Heart rate control is essential for symptom relief, particularly in atrial fibrillation or during exertion: 1

• First-line agents: Beta-blockers or non-dihydropyridine calcium channel blockers 1
• Digoxin: Specifically recommended for rate control in atrial fibrillation with mitral stenosis 1
• Indication: Symptomatic resting or exertional sinus tachycardia in normal sinus rhythm 4

Volume Management

Diuretics are indicated for symptom relief when pulmonary edema or congestion develops, 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 heart disease. 1 This is a critical distinction from other cardiac conditions.

Indications for anticoagulation: 4, 1

• All patients with atrial fibrillation
• History of systemic thromboembolism
• Presence of left atrial thrombus on imaging
• Dense spontaneous echo contrast in the left atrium
• Severely dilated left atrium

Heart Failure Management

For patients with left ventricular systolic dysfunction, implement guideline-directed medical therapy: 2

• ACE inhibitors or ARBs
• Beta-blockers
• Aldosterone antagonists
• Sacubitril/valsartan when indicated
• Caution: Avoid abrupt blood pressure 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 determine valve morphology for intervention suitability. 4

Transesophageal echocardiography (TEE) should be performed before percutaneous mitral balloon commissurotomy (PMBC) to assess for left atrial thrombus and evaluate mitral regurgitation severity. 4

Exercise Testing

When resting echocardiographic findings and clinical symptoms are discordant, exercise testing with Doppler or invasive hemodynamic assessment is recommended to evaluate symptomatic response, exercise capacity, and the response of mean mitral gradient and pulmonary artery pressure. 4

Interventional Management

Percutaneous Mitral Balloon Commissurotomy (PMBC)

PMBC at a Comprehensive Valve Center is the preferred intervention for symptomatic patients with severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) who have favorable valve morphology. 4, 1, 2

Favorable valve morphology criteria: 2

• Mobile, relatively thin leaflets
• Free of calcium
• Without significant subvalvular fusion
• Less than 2+ mitral regurgitation
• Absence of left atrial thrombus

Class I indications for PMBC: 4

• Symptomatic patients (NYHA class II, III, or IV) with severe mitral stenosis (MVA ≤1.5 cm²) and favorable valve morphology

Class IIa indications for PMBC: 4, 1

• Asymptomatic patients with severe mitral stenosis and pulmonary artery systolic pressure >50 mmHg
• Asymptomatic patients with severe mitral stenosis planning pregnancy
• Asymptomatic patients with severe mitral stenosis and high thromboembolic risk

Long-term outcomes show 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 or IV) with severe mitral stenosis who: 4, 2

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

For women of childbearing age requiring valve replacement, bioprosthetic valves are recommended over mechanical valves to avoid anticoagulation hazards during pregnancy. 1

Monitoring and Surveillance

Regular echocardiographic monitoring is essential to detect disease progression: 1

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

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

During pregnancy: 1

• Beta-blockers for heart rate control
• Diuretics for volume overload
• Cardiac surgery may be necessary if medical and interventional therapies fail

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, 3 However, this is not needed if the patient is already receiving secondary prophylaxis antibiotics. 1, 2

For patients on penicillin prophylaxis who require endocarditis prophylaxis, use an agent other than penicillin (such as clindamycin). 5

Additional Preventive Measures

• Vaccinations: Influenza and pneumococcal vaccines according to standard recommendations 2, 3
• Oral health: Optimal maintenance to reduce endocarditis risk 1, 2
• Exercise: Regular aerobic exercise to improve cardiovascular fitness in asymptomatic patients 3

Critical Pitfalls to Avoid

Common errors that worsen outcomes: 1, 2

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