What is the management approach for rheumatic heart disease?

Management of Rheumatic Heart Disease

Core Management Strategy

All patients with rheumatic heart disease require lifelong secondary antibiotic prophylaxis with intramuscular benzathine penicillin G (1.2 million units every 4 weeks) as the cornerstone of management, combined with guideline-directed medical therapy for heart failure when present, and timely valve intervention for severe symptomatic disease. 1, 2, 3

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Secondary Antibiotic Prophylaxis (Mandatory for All Patients)

First-Line Regimen

• Intramuscular benzathine penicillin G 1.2 million units every 4 weeks is the gold standard with the strongest evidence for preventing recurrent rheumatic fever and reducing progression of valve disease. 1, 3, 4
• This regimen is approximately 10 times more effective than oral antibiotics at preventing recurrence. 3, 5

Penicillin-Allergic Patients

• Oral penicillin V 250 mg twice daily for non-severe penicillin hypersensitivity. 1, 3
• Sulfadiazine 1 g once daily (0.5 g for patients ≤27 kg) as an alternative. 3
• Erythromycin orally twice daily for immediate penicillin hypersensitivity. 6

Duration of Prophylaxis

The duration depends on disease severity and must be strictly followed: 7, 3

• Rheumatic fever WITH carditis AND residual valve disease: 10 years after last episode OR until age 40, whichever is longer; consider lifelong for high-risk patients (teachers, daycare workers with high streptococcal exposure). 7, 3
• Rheumatic fever WITH carditis but NO residual valve disease: 10 years after last episode OR until age 21, whichever is longer. 7, 3
• Rheumatic fever WITHOUT carditis: 5 years after last episode OR until age 21, whichever is longer. 7, 3

Critical Consideration

• Secondary prophylaxis must continue even after valve surgery, as valve replacement does not eliminate the risk of recurrent acute rheumatic fever. 3

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Medical Management of Heart Failure

Acute Decompensation

• Initiate loop diuretics immediately for pulmonary congestion or peripheral edema. 2
• Assess volume status, functional capacity, and perform laboratory evaluation including BNP/NT-proBNP. 2

Guideline-Directed Medical Therapy for Reduced Ejection Fraction

Implement the following in sequence: 1, 2

• ACE inhibitors or ARBs as first-line vasodilator therapy
• Beta-blockers for mortality benefit (carvedilol, metoprolol succinate, or bisoprolol)
• Aldosterone antagonists (spironolactone or eplerenone) for NYHA class II-IV
• Sacubitril/valsartan when indicated for advanced heart failure
• Hydralazine/isosorbide dinitrate if ACE inhibitors/ARBs contraindicated 2

Atrial Fibrillation Management

• Rate control with beta-blockers or digoxin as first-line therapy. 2
• Anticoagulation with warfarin is mandatory for stroke prevention in rheumatic heart disease with atrial fibrillation (higher thrombotic risk than non-rheumatic AF). 1, 2
• Digoxin provides additional benefit for symptom control in heart failure with atrial fibrillation. 2

Blood Pressure Management

• Avoid abrupt blood pressure lowering in patients with stenotic valve lesions (mitral or aortic stenosis), as they are preload-dependent. 1

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Valve Intervention

Indications for Intervention

Evaluate all patients with the following for percutaneous or surgical intervention within 3 months: 1

• Symptomatic severe mitral stenosis (mitral valve area ≤1.5 cm²)
• Asymptomatic severe mitral stenosis in women planning pregnancy
• Moderate-severe mitral stenosis with symptoms (NYHA class II-IV)

Percutaneous Mitral Balloon Commissurotomy (PMBC)

• Preferred first-line intervention for favorable valve anatomy: mobile leaflets, minimal calcification, <2+ mitral regurgitation, no left atrial thrombus. 1
• 70-80% of patients remain symptom-free at 10 years after successful PMBC. 1

Surgical Valve Intervention

Indicated when: 1

• Unfavorable valve morphology (heavily calcified, immobile leaflets, significant subvalvular fusion)
• PMBC has failed or is contraindicated
• Moderate-to-severe tricuspid regurgitation requiring concomitant repair
• Left atrial thrombus present

Critical Pitfall

• Never delay valve intervention in symptomatic patients with severe disease—medical therapy alone is not a substitute for definitive treatment and leads to progressive irreversible cardiac damage. 1

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Infective Endocarditis Prophylaxis

When to Provide Prophylaxis

• All patients with rheumatic heart disease undergoing dental procedures involving manipulation of gingival tissue, periapical region, or oral mucosa perforation require antibiotic prophylaxis. 1, 2
• Exception: Patients already receiving benzathine penicillin G prophylaxis who require endocarditis prophylaxis should receive an alternative agent (not penicillin), as oral streptococci may have developed penicillin resistance. 3, 6

Recommended Regimen

• Amoxicillin prophylaxis before high-risk dental or surgical procedures for patients on benzathine penicillin G. 6
• Clindamycin for patients with immediate penicillin hypersensitivity or recent penicillin/amoxicillin exposure. 6

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Pregnancy Considerations

Pre-Pregnancy Evaluation

• All women with moderate-severe rheumatic heart disease must be evaluated before pregnancy and valve intervention considered if indicated. 1
• Pregnancy is a high-risk period due to increased hemodynamic demands.

Management During Pregnancy

• Continue secondary prophylaxis with benzathine penicillin G throughout pregnancy. 1
• Beta-blockers and diuretics are safe and effective for symptom control. 1
• Anticoagulation management requires careful monitoring—warfarin in second/third trimester, transition to heparin near delivery. 1

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Monitoring and Surveillance

Echocardiographic Follow-Up

Regular surveillance is essential to detect disease progression: 1, 2

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

Additional Preventive Measures

• Optimal oral health maintenance to reduce endocarditis risk. 1, 2
• Influenza and pneumococcal vaccinations to prevent respiratory infections. 1, 2

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Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely—this is the most common error leading to recurrent rheumatic fever and progressive valve damage. 1, 2, 3
• Never assume valve surgery eliminates the need for secondary prophylaxis—patients remain susceptible to group A streptococcus infection. 3
• Never delay valve intervention in symptomatic severe disease—medical therapy alone leads to irreversible cardiac damage. 1
• Never provide inadequate anticoagulation monitoring in patients with atrial fibrillation—rheumatic mitral stenosis carries exceptionally high stroke risk. 1, 2
• Never overlook pregnancy as a high-risk period—hemodynamic changes can precipitate acute decompensation. 1
• Never use penicillin for endocarditis prophylaxis in patients already on benzathine penicillin G—resistance is likely. 3, 6

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Health System Approaches

• Register-based comprehensive control programs with community health worker involvement improve adherence to secondary prophylaxis. 1
• Integration into primary health care systems with patient and family education enhances long-term outcomes. 1
• School-based delivery programs for benzathine penicillin G improve adherence in pediatric populations. 8