What is the treatment for rheumatic heart disease?

Treatment of Rheumatic Heart Disease

The treatment of rheumatic heart disease requires a comprehensive strategy centered on lifelong secondary antibiotic prophylaxis with intramuscular benzathine penicillin G (1.2 million units every 4 weeks), guideline-directed medical therapy for heart failure and valve complications, anticoagulation for atrial fibrillation, and timely percutaneous or surgical valve intervention for moderate-to-severe symptomatic disease. 1

Secondary Prophylaxis: The Foundation of Treatment

Intramuscular benzathine penicillin G (1.2 million units every 4 weeks) is the first-line prophylaxis regimen with the strongest evidence for preventing rheumatic fever recurrences. 1 This is non-negotiable and represents the most critical intervention to prevent disease progression. 2

Duration of Prophylaxis

The duration must be tailored to disease severity 1:

• Patients with persistent valvular disease: Continue for 10 years after the last attack OR until age 40 (whichever is longer) 1
• Patients with rheumatic fever and carditis but no residual heart disease: Continue for 10 years after the last attack OR until age 21 1
• Patients without carditis: Shorter durations may be considered, but this represents a minority 2

Penicillin-Allergic Patients

For patients with documented penicillin allergy, alternatives include oral penicillin V, sulfadiazine, or macrolide antibiotics. 1 However, these are less effective than intramuscular benzathine penicillin G, so true allergy should be confirmed before switching. 2

Common pitfall: Discontinuing secondary prophylaxis too early is a major cause of recurrent rheumatic fever and progressive valve damage. 1 Compliance with injection schedules is often poor; register-based programs with community health worker involvement significantly improve adherence. 2

Medical Management of Cardiac Complications

Heart Failure Management

For patients with left ventricular systolic dysfunction and heart failure, standard guideline-directed medical therapy should be implemented. 1 This includes:

• Diuretics for volume management 1
• ACE inhibitors or ARBs as first-line agents (preferred due to potential anti-inflammatory properties) 1
• Beta-blockers for rate control and cardiac protection 1
• Aldosterone antagonists (spironolactone or eplerenone) for advanced heart failure 1
• Sacubitril/valsartan when indicated for reduced ejection fraction 1

Blood pressure should be managed carefully, avoiding abrupt lowering in patients with stenotic lesions. 1

Anticoagulation for Atrial Fibrillation

Patients with rheumatic heart disease and atrial fibrillation require anticoagulation with warfarin targeting an INR of 2.0-3.0. 3 This is particularly critical as rheumatic mitral stenosis with atrial fibrillation carries extremely high stroke risk. 3

• Target INR: 2.5 (range 2.0-3.0) for most patients with valvular atrial fibrillation 3
• Monitoring: Community-based anticoagulation monitoring must be available and affordable 2
• Novel oral anticoagulants (NOACs): Currently NOT recommended for valvular atrial fibrillation; warfarin remains the standard 3

Critical pitfall: Inadequate anticoagulation monitoring leads to either thromboembolism (under-anticoagulation) or bleeding (over-anticoagulation). 1 Regular INR monitoring every 2-4 weeks is essential. 3

Acute Rheumatic Carditis Treatment

For patients presenting with acute rheumatic carditis 4:

• Severe carditis with heart failure or pericarditis: Corticosteroids (more potent anti-inflammatory than salicylates) for 12 weeks 4
• Mild carditis without heart failure: Salicylates may be sufficient for 12 weeks 4
• Penicillin: Eradicate streptococci regardless of carditis severity 4

Valvular regurgitation, not myocarditis, is the primary cause of heart failure in acute rheumatic carditis. 4 Therefore, severe mitral regurgitation causing intractable hemodynamics may require urgent surgical intervention even during the acute phase. 4

Interventional and Surgical Management

Indications for Valve Intervention

All patients with symptomatic severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) should be evaluated for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis. 1

Percutaneous Mitral Balloon Commissurotomy (PMBC)

PMBC is the preferred intervention for patients with favorable valve morphology (mobile, relatively thin leaflets free of calcium, without significant subvalvular fusion) and less than 2+ mitral regurgitation in the absence of left atrial thrombus. 1

• Long-term outcomes: 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years 1
• Advantages: Less invasive than surgery, shorter recovery, avoids prosthetic valve complications 2

Surgical Intervention

Surgery is indicated when 1:

• Valve anatomy is unfavorable for PMBC (heavy calcification, severe subvalvular fusion)
• PMBC has failed or resulted in significant mitral regurgitation
• Moderate-to-severe tricuspid regurgitation requires concomitant repair
• Left atrial thrombus is present (contraindication to PMBC)

Valve repair is preferred over replacement when technically feasible, as it avoids prosthetic valve complications and may not require lifelong anticoagulation. 2 However, rheumatic valve pathology often necessitates replacement. 2

Asymptomatic Patients

For truly asymptomatic patients, intervention decisions are more nuanced 2:

• Severe mitral stenosis before planned pregnancy: Intervention should be considered 1
• Progressive left ventricular dysfunction: May warrant intervention even without symptoms 2
• Very severe stenosis with pulmonary hypertension: Consider intervention to prevent irreversible complications 2

Critical pitfall: Delaying valve intervention in symptomatic patients with severe disease is harmful, as medical therapy alone cannot substitute for definitive treatment. 1 Waiting for symptoms to worsen may allow irreversible left ventricular dysfunction or sudden death. 2

Special Considerations in Pregnancy

Women with moderate-to-severe rheumatic heart disease should be evaluated before pregnancy, and interventional therapy should be considered prior to conception. 1 Pregnancy dramatically increases hemodynamic stress and can unmask previously undiagnosed disease. 5

Management During Pregnancy

• Beta-blockers: For rate control in mitral stenosis 1
• Diuretics: Cautiously for volume management 1
• Anticoagulation: Required for mechanical valves or atrial fibrillation, but warfarin is teratogenic in first trimester; transition to low-molecular-weight heparin or unfractionated heparin during organogenesis (weeks 6-12), then may resume warfarin in second/third trimester 5
• Delivery planning: High-risk patients require multidisciplinary care with cardiology and maternal-fetal medicine 5

Pregnancy represents a high-risk period that is frequently overlooked. 1 Maternal and fetal mortality remain elevated even with optimal care. 5

Infective Endocarditis Prophylaxis

Antibiotic prophylaxis is recommended before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa in patients with rheumatic heart disease. 1 However, patients already receiving continuous secondary prophylaxis with benzathine penicillin G do not require additional prophylaxis. 2

Standard regimen: Amoxicillin 2g orally 30-60 minutes before procedure (or clindamycin 600mg for penicillin-allergic patients). 1

Monitoring and Follow-Up

Echocardiographic Surveillance

Regular echocardiography is essential to detect disease progression 1:

• Mild disease: Every 3-5 years 1
• Moderate disease: Every 1-2 years 1
• Severe disease or dilating left ventricle: Every 6-12 months 1

Pitfall: Failure to perform regular follow-up echocardiography can miss the optimal window for intervention before irreversible complications develop. 1

Additional Preventive Measures

• Optimal oral health maintenance: Reduces endocarditis risk 1
• Influenza and pneumococcal vaccinations: Recommended for all patients 1
• Patient and family education: Critical for adherence to prophylaxis and recognition of warning symptoms 2

Health System Approaches

Register-based comprehensive rheumatic heart disease control programs significantly improve outcomes. 2 These programs should include:

• Community health worker involvement for prophylaxis delivery 2
• Integration into primary health care systems 2
• Reliable supply of high-quality benzathine penicillin G 2
• Access to portable echocardiography for screening and diagnosis 2
• Linkages to surgical centers for definitive intervention 2

Access to cardiac surgery remains severely limited in rheumatic heart disease-endemic regions, representing a major barrier to optimal care. 5, 6 Efforts to increase surgical capacity and establish international training partnerships are ongoing. 2

Summary of Treatment Algorithm

1. Establish diagnosis with clinical criteria (Jones criteria) and echocardiography 7, 6
2. Initiate secondary prophylaxis with benzathine penicillin G 1.2 million units IM every 4 weeks 1
3. Treat acute carditis if present (corticosteroids for severe, salicylates for mild) 4
4. Implement medical therapy for heart failure (ACE inhibitors, beta-blockers, diuretics, aldosterone antagonists) 1
5. Anticoagulate if atrial fibrillation present (warfarin, INR 2.0-3.0) 3
6. Evaluate for intervention if symptomatic moderate-to-severe valve disease (PMBC preferred for favorable anatomy) 1
7. Monitor regularly with echocardiography based on disease severity 1
8. Provide endocarditis prophylaxis for high-risk dental procedures 1
9. Counsel regarding pregnancy and optimize before conception if moderate-to-severe disease 1, 5