What is the treatment for rheumatic heart disease?

Treatment of Rheumatic Heart Disease

The treatment of rheumatic heart disease requires mandatory lifelong secondary prophylaxis with benzathine penicillin G 1.2 million units intramuscularly every 4 weeks to prevent recurrent acute rheumatic fever, combined with guideline-directed medical therapy for heart failure when present, and surgical or interventional valve repair/replacement for severe valvular disease. 1, 2

Secondary Prevention: The Foundation of Treatment

Lifelong antibiotic prophylaxis is non-negotiable for all patients with rheumatic heart disease. 1, 2

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the preferred regimen 1
• In high-risk situations, administration every 3 weeks is recommended 1
• Alternative regimens for penicillin-allergic patients include:
  • Penicillin V potassium 250 mg orally twice daily 1
  • Sulfadiazine 1 g orally once daily 1
  • Macrolide or azalide antibiotics (avoid with medications inhibiting cytochrome P450 3A) 1

Duration of prophylaxis depends on disease severity:

• With persistent valvular disease: ≥10 years or until age 40 (whichever is longer), with consideration for lifelong prophylaxis in high-risk exposure settings 1, 2
• With carditis but no residual valve disease: 10 years or until age 21 (whichever is longer) 1
• Without carditis: 5 years or until age 21 (whichever is longer) 1

Critical pitfall: Secondary prophylaxis is required even after valve replacement surgery 1

Medical Management of Heart Failure

When congestive heart failure develops from rheumatic valvular disease, implement standard guideline-directed medical therapy immediately 2:

Diuretics for Volume Overload

• Start loop diuretics immediately if pulmonary congestion or peripheral edema is present for rapid symptomatic relief 2
• For insufficient response: increase diuretic dose, combine loop diuretics with thiazides, or administer loop diuretics twice daily 2
• Avoid thiazides if GFR <30 mL/min except when used synergistically with loop diuretics 2

ACE Inhibitors for Systolic Dysfunction

• Initiate ACE inhibitors as first-line therapy for left ventricular systolic dysfunction (ejection fraction <40-45%), even in asymptomatic patients 2
• Start with low dose and uptitrate to target doses proven in trials, not based on symptoms alone 2
• Review and potentially reduce diuretics for 24 hours before starting ACE inhibitors to avoid excessive hypotension 2
• Monitor blood pressure, renal function, and electrolytes at 1-2 weeks after each dose increment, at 3 months, then every 6 months 2
• Stop ACE inhibitors if renal function deteriorates substantially 2
• Substitute angiotensin receptor blockers (ARBs) if ACE inhibitors cause intolerable cough or angioedema 2

Beta-Blockers

• Add beta-blockers (bisoprolol, carvedilol, or metoprolol XL/CR) to ACE inhibitors and diuretics for all patients with stable heart failure and reduced ejection fraction in NYHA class II-IV 2

Aldosterone Antagonists

• Add spironolactone for advanced heart failure (NYHA class III-IV) in addition to ACE inhibitors and diuretics to improve survival and morbidity 2

Additional Pharmacologic Considerations

• Sacubitril/valsartan may be used per standard heart failure guidelines 2
• Digoxin for symptom control, particularly beneficial if atrial fibrillation is present 2
• Hydralazine/isosorbide dinitrate as alternative vasodilator therapy if ACE inhibitors and ARBs are contraindicated 2

Medications to Avoid

• Avoid NSAIDs as they interfere with ACE inhibitor efficacy 2
• Avoid potassium-sparing diuretics during ACE inhibitor initiation 2
• Avoid calcium channel blockers with negative inotropic effects (non-dihydropyridines) if ejection fraction <50% 2

Management of Atrial Fibrillation

Atrial fibrillation is common in rheumatic heart disease, occurring in approximately 49% of patients 3:

• Rate control using beta-blockers or digoxin 2
• Anticoagulation with warfarin is mandatory in patients with rheumatic heart disease and atrial fibrillation 2
• Anticoagulation was utilized in only 35-38% of eligible patients in observational studies, representing significant undertreatment 3, 4

Surgical and Interventional Treatment

Definitive catheter-based or surgical intervention is the only treatment that can improve outcomes for patients with moderate or severe rheumatic valvular disease. 5

• Revascularization is reasonable in patients with severe cardiac allograft vasculopathy and suitable anatomy 1
• Surgical procedures were performed in only 8.5% of patients in one tertiary center study, indicating significant underutilization 4
• Access to intervention remains very limited in RHD endemic regions 5

Valve-Specific Patterns

The most common valvular lesions requiring intervention include 3, 4:

• Mitral stenosis (85-85%) - most common abnormality
• Mitral regurgitation (70-87%)
• Aortic regurgitation (53%)
• Combinations of 3-4 valve lesions (MR+MS+TR or MR+MS+TR+AR) in 28-30% of patients

Additional Preventive Measures

• Infective endocarditis prophylaxis for appropriate procedures 1, 2
• Optimal oral health maintenance - the most important component of preventing infective endocarditis 1, 2
• Influenza and pneumococcal vaccinations per standard recommendations 1, 2

Monitoring and Surveillance

Regular echocardiographic surveillance is essential 2:

• Every 6-12 months for severe disease
• Every 1-2 years for moderate disease
• Every 3-5 years for mild disease

Routine echocardiography should be performed for all patients with RHD, focusing on younger adults, to facilitate diagnosis and treatment before complications develop. 3

Key Clinical Pearls

• Rheumatic heart disease predominantly affects younger adults with median age 28-44 years 3, 4
• Pansystolic murmur is present in approximately 92% of patients 3
• Congestive heart failure occurs in 89% of patients at presentation 3
• The majority of patients (82%) are treated with medical therapies alone, though many would benefit from surgical intervention 4
• Mitral valve leaflet thickening, calcification, and restriction of motion are present in 92% of patients 3